Experiences of employment among young people with juvenile idiopathic arthritis: a qualitative study.

PubMed

Hanson, Helen; Hart, Ruth I; Thompson, Ben; McDonagh, Janet E; Tattersall, Rachel; Jordan, Alison; Foster, Helen E

2018-08-01

This study explored expectations and experiences of employment among young people with juvenile idiopathic arthritis and the role of health professionals in promoting positive employment outcomes. Semistructured interviews (n = 13) and three focus groups (n = 9, n = 4, n = 3) were conducted with young people (16-25 y) and adults (26-31 y) with juvenile idiopathic arthritis and semistructured interviews (n = 9) were conducted with health professionals. Transcripts were analyzed thematically. Young people with juvenile idiopathic arthritis have concerns about employers' attitudes toward employees with long-term health conditions and lack knowledge of antidiscrimination legislation. Young people not in education, employment or training identify arthritis as a key barrier. Challenges associated with arthritis (e.g., pain, psychological distress) may not be visible to employers. Decisions about disclosing arthritis are challenging and cause anxiety. Young people associate good disease management and access to flexible and convenient care with their capacity to succeed in employment. Psychosocial and vocational interventions have benefited some young people but are not routinely available. Low expectations of employers may affect young people's decisions about disclosure and seeking appropriate support in the work place. Health professionals can equip young people with knowledge and skills to negotiate appropriate support, through signposting to antidiscrimination information and offering practice of transferable skills such as disclosure in consultations. Implications for rehabilitation Young people with juvenile idiopathic arthritis encounter challenges with regard to employment; many lack the knowledge and skills to negotiate appropriate support from employers. Rehabilitation professionals could play a more substantial role in equipping them with relevant knowledge and skills by signposting to antidiscrimination information and nurturing

Evaluation of Fitness and the Balance Levels of Children with a Diagnosis of Juvenile Idiopathic Arthritis: A Pilot Study

PubMed Central

Maggio, Maria Cristina; Corsello, Giovanni; Messina, Giuseppe; Iovane, Angelo; Palma, Antonio

2017-01-01

Background: Juvenile idiopathic arthritis is a main cause of physical disability and has high economic costs for society. The purpose of this study was to assess the fitness levels and the postural and balance deficits with a specific test battery. Methods: Fifty-six subjects were enrolled in this study. Thirty-nine healthy subjects were included in the control group and seventeen in the juvenile idiopathic arthritis group. All subjects were evaluated using a posturography system. The fitness level was evaluated with a battery of tests (Abalakov test, sit-up test, hand grip test, backsaver sit and reach, the toe touch test). An unpaired t-test was used to determine differences. Pearson’s correlation coefficient was used to evaluate the correlation between the tests. Results: The battery of tests demonstrated that subjects in the juvenile idiopathic arthritis group have lower fitness levels compared to the control group. The juvenile idiopathic arthritis group showed low postural control with respect to the control group. Pearson analysis of the juvenile idiopathic arthritis group data showed significant correlations between variables. Pearson’s results from the control group data showed a similar trend. Conclusions: The results suggest that the battery of tests used could be an appropriate tool. However, we highlight that these conclusions need to be supported by other studies with a larger population scale. PMID:28753965

Evaluation of Fitness and the Balance Levels of Children with a Diagnosis of Juvenile Idiopathic Arthritis: A Pilot Study.

PubMed

Patti, Antonino; Maggio, Maria Cristina; Corsello, Giovanni; Messina, Giuseppe; Iovane, Angelo; Palma, Antonio

2017-07-19

Background: Juvenile idiopathic arthritis is a main cause of physical disability and has high economic costs for society. The purpose of this study was to assess the fitness levels and the postural and balance deficits with a specific test battery. Methods: Fifty-six subjects were enrolled in this study. Thirty-nine healthy subjects were included in the control group and seventeen in the juvenile idiopathic arthritis group. All subjects were evaluated using a posturography system. The fitness level was evaluated with a battery of tests (Abalakov test, sit-up test, hand grip test, backsaver sit and reach, the toe touch test). An unpaired t -test was used to determine differences. Pearson's correlation coefficient was used to evaluate the correlation between the tests. Results: The battery of tests demonstrated that subjects in the juvenile idiopathic arthritis group have lower fitness levels compared to the control group. The juvenile idiopathic arthritis group showed low postural control with respect to the control group. Pearson analysis of the juvenile idiopathic arthritis group data showed significant correlations between variables. Pearson's results from the control group data showed a similar trend. Conclusions: The results suggest that the battery of tests used could be an appropriate tool. However, we highlight that these conclusions need to be supported by other studies with a larger population scale.

Specialty Practice and Cost Considerations in the Management of Uveitis Associated With Juvenile Idiopathic Arthritis.

PubMed

Palestine, Alan G; Singh, Jasleen K; Kolfenbach, Jason R; Ozzello, Daniel J

2016-07-01

To evaluate whether cost, prior insurance authorization concerns, and subspecialty practice influence therapeutic decisions in the treatment of uveitis associated with juvenile idiopathic arthritis. A total of 2,965 pediatric ophthalmologists, uveitis specialists, retina specialists, and rheumatologists across the United States were surveyed via e-mail regarding their choice in long-term therapy for a hypothetical patient with uveitis associated with juvenile idiopathic arthritis. Outcomes of interest were differences in therapy choice based on cost/prior authorization and specialty practice. There were significant differences in the use of methotrexate and biologics among specialists, both with and without consideration for cost and prior authorization. Physicians in four different specialties who treat uveitis associated with juvenile idiopathic arthritis agree on methotrexate as a first-line treatment choice and a biologic immunosuppressive medication as a second choice, but there are significant differences between the specialties in their use of these medications. Cost and insurance considerations did not affect therapy selection. [J Pediatr Ophthalmol Strabismus. 2016;53(4):246-251.]. Copyright 2016, SLACK Incorporated.

Treatment of juvenile idiopathic arthritis-associated uveitis: challenges and update.

PubMed

Rabinovich, C Egla

2011-09-01

To update the current understanding of the risk factors for poor outcomes in juvenile idiopathic arthritis-related uveitis. In addition, current therapies, both traditional and biological, are reviewed. Male sex, independent of age or antinuclear antibody status, is associated with increased ocular morbidity. Having anterior chamber inflammation on first exam increases the risk of developing vision-threatening eye complications. Presence of one complication increases the risk of developing another. Risk of cataract development associated with topical glucocorticoid use is better defined. Longer duration of remission on therapy has been found to decrease the risk of disease flare after discontinuation of methotrexate. Recent studies of both nonbiological and biological therapies for arthritis-related uveitis are discussed. With a better understanding of risk factors associated with the ocular morbidity of uveitis associated with juvenile idiopathic arthritis, aggressive therapies can be targeted for improved visual outcomes. Alternative treatments to avoid long-term corticosteroid use include the use of antimetabolites and biological therapies. More prospective comparator studies and/or use of multicenter databases are needed to better understand best treatments.

The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

PubMed

Rider, Lisa G; Shah, Mona; Mamyrova, Gulnara; Huber, Adam M; Rice, Madeline Murguia; Targoff, Ira N; Miller, Frederick W

2013-07-01

The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high

The Myositis Autoantibody Phenotypes of the Juvenile Idiopathic Inflammatory Myopathies

PubMed Central

Shah, Mona; Mamyrova, Gulnara; Huber, Adam M.; Rice, Madeline Murguia; Targoff, Ira N.; Miller, Frederick W.

2013-01-01

Abstract The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, “shawl-sign” rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and “mechanic’s hands,” and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was

[Importance of the new biologicals and cytokine antagonists in the treatment of juvenile idiopathic arthritis (JIA)].

PubMed

Horneff, G

2005-06-01

Juvenile idiopathic arthritis is group of diseases of unknown aetiology characterised by the occurrence of chronic arthritis during childhood. Compared to adult onset rheumatoid arthritis, its course is more variable. Increasing knowledge of the inflammatory process as well as in molecular genetics and biotechnology has enable the production of new drugs, the biologicals. These are able to specifically block mechanisms of immune activation and thereby interfere with the inflammatory process. An increasing number of biologicals have been tried in clinical studies in adults suffering from rheumatoid arthritis, psoriasis or psoriasis arthritis and a couple of them were already licensed for treatment. Treatment of juvenile idiopathic arthritis by blockade of tumournecrosis-factor (TNF) using the soluble receptor Etanercept or the monoclonal antibodies Infliximab and Adalimumab showed comparable clinical efficacy. Blockade of TNF therefore already reached a certain place in the therapeutic algorythm for treatment of juvenile idiopathic arthritis. Currently, only Etanercept is licensed for treatment of active juvenile polyarthritis refractory to methotrexate. Studies using Infliximab and Adalimumab will be completed in the near future. However, antibodies blocking TNF may already be used in patients suffering from active uncontrolled chronic uveitis in whom visual impairment is threatening. TNF blockers may also be indicated in juvenile ankylosing spondylitis. The use of further biologicals, the interleukin-1 receptor antagonist Anakinra, Atlizumab (MRA) blocking the receptor for interleukin-6 or Abatacept, an inhibitory ligand of the co-stimulatory T cell membrane molecule CD28, remain experimental and should be preserved for clinical studies.

Update on the Treatment of Uveitis in Patients with Juvenile Idiopathic Arthritis: A Review.

PubMed

Asproudis, Ioannis; Katsanos, Andreas; Kozeis, Nikolaos; Tantou, Alexandra; Konstas, Anastasios G

2017-12-01

Chronic uveitis is a common extra-articular manifestation of juvenile idiopathic arthritis. The classic clinical picture is one of chronic anterior uveitis, which usually remains asymptomatic until ocular complications arise. The risk of uveitis is increased in girls with an early onset of oligoarthritis and positive antinuclear antibodies. Even though the inflammation in patients with juvenile idiopathic arthritis is initially limited in the anterior part of the eye, chronic active inflammation may eventually cause significant damage to the posterior pole. Complications may include band keratopathy, cataract, secondary glaucoma, posterior synechiae, cystoid macular edema, and hypotony. The cooperation of ophthalmologists with rheumatologists may help define the best treatment plan. The ophthalmic therapeutic regimen includes topical corticosteroids and mydriatics, while in severe cases immunosuppressive and biological agents are introduced. Surgical management of complications might be needed.

Updates on the risk markers and outcomes of severe juvenile idiopathic arthritis-associated uveitis

PubMed Central

Angeles-Han, Sheila T; Yeh, Steven; Vogler, Larry B

2013-01-01

Uveitis is the most common extra-articular manifestation of juvenile idiopathic arthritis, which is the most common systemic cause of uveitis in children. Known risk factors for uveitis include antinuclear antibody seropositivity, young age of arthritis onset, specific juvenile idiopathic arthritis subtype and short duration of disease. Risk markers for severe ocular disease include gender, age and complications at initial visit. Due to the risk for vision-compromising sequelae such as cataracts, band keratopathy, glaucoma, vision loss and blindness, an understanding of the risk factors for uveitis development and severe ocular disease is crucial to help prevent serious visual disability and complications. This paper reviews the pathogenesis of uveitis, known risk factors for uveitis development and severe visual outcome, and addresses the need for additional biomarkers of uveitis risk, prognosis and remission. PMID:24187594

[Physiotherapy for juvenile idiopathic arthritis].

PubMed

Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

2012-07-01

Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

Feasibility and efficacy of intraarticular steroids (IAS) in juvenile idiopathic arthritis (JIA).

PubMed

Verma, Sumit; Gupta, Rajiva; Lodha, Rakesh; Kabra, S K

2009-03-01

Thirteen children with juvenile idiopathic arthritis (JIA) were treated with intraarticular steroid injection of triamcilone acetonide as a day care procedure. More than half (53.4%) the children were free of pain, limp and NSAID's use, with improvement in functional score at 12 weeks. No side effects were reported during the period of the study.

[Osteoporosis in premenopausal women].

PubMed

Mitringer, Antje; Pietschmann, P

2002-01-01

Osteoporosis is a systemic disease of bone, which is characterized by decreased bone mass and changes in the microarchitecture of bone tissue followed by brittleness of bones and increased risk of fractures. Osteoporosis frequently is a disease of postmenopausal women, nevertheless, in rare cases, osteoporosis can also occur in young adults. There are only few studies on the pathophysiology of "premenopausal osteoporosis"; in addition to idiopathic forms, osteoporosis in young women can be caused by glucocorticoid treatment, by eating disorders or can be associated with pregnancy.

Facioskeletal changes in children with juvenile idiopathic arthritis

PubMed Central

Twilt, M; Schulten, A J M; Nicolaas, P; Dülger, A; van Suijlekom‐Smit, L W A

2006-01-01

Objective To investigate the facioskeletal morphology in patients with juvenile idiopathic arthritis (JIA) with and without temporomandibular joint (TMJ) involvement. Methods Eighty five patients were included. TMJ involvement was defined by orthopantomogram alterations. Lateral cephalograms were used to determine linear and angular measurements and occlusion. Results Patients regardless of their TMJ status had a 67% chance for retrognathia and a 52% chance for posterior rotation of the mandible and, respectively, 82% and 58% if TMJ involvement were present. Changes were not uniformly distributed among the different subtypes. Conclusion Patients with JIA have an altered facial morphology, especially in the presence of TMJ involvement. PMID:16699052

Physiotherapy in pauciarticular juvenile idiopathic arthritis. Case study.

PubMed

Zuk, Beata; Kaczor, Zofia; Zuk-Drążyk, Berenika; Księżopolska-Orłowska, Krystyna

2014-01-01

Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood and adolescence. This term encompasses a group of chronic systemic inflammatory diseases of the connective tissue which cause arthritis in patients under 16 years of age lasting at least 6 weeks. The authors presented the characteristic features of physiotherapy based on functional examination results on the basis of two cases of girls with pauciarticular JIA treated according to an established pharmacological regimen. Physiotherapy should be introduced at an early stage of the disease. Kinesiotherapy preceded by history-taking and a functional examination of the patient, has to focus on both primary and secondary joint lesions.

Management of male osteoporosis.

PubMed

Cortet, B; Vasseur, J; Grardel, B; Catanzariti, L; Marchandise, X; Delcambre, B

2001-05-01

The objective of this study was to evaluate the efficacy of treatments for male osteoporosis selected based on the cause of the disease. Sixty-three men with osteoporosis (T-score at the lumbar spine and/or femoral neck lower than -2.5) with a mean age of 53+/-11 years were studied. Forty-three (68.3%) had a history of fracturing without trauma (vertebral fractures, 37 patients, 57%). Treatments were as follows: idiopathic osteoporosis: calcium and vitamin D supplements (N = 10) or cyclical etidronate for 2 weeks followed by calcium and vitamin D supplements for 76 days (N = 29); moderate idiopathic phosphate diabetes: calcitriol and phosphate (N = 15); idiopathic hypercalciuria: hydrochlorothiazide (N = 6); and hypogonadism: testosterone (N = 3). Percentage change in bone mineral density (mean +/- standard error of the mean) after 18 months: calcium and vitamin D (lumbar spine: 0.6+/-2; femoral neck: 2.2+/-2.2); etidronate (lumbar spine: 3.6+/-1.4*; femoral neck: 0.5+/-1); calcitriol (lumbar spine: 7.0+/-3.5*; femoral neck: 0.0+/-1.4); thiazide diuretic (lumbar spine: 1+/-3.2; femoral neck: -2.3+/-3.7); and testosterone (lumbar spine: 6.8+/-6.4; femoral neck: 2.5+/-2.7), where *P < 0.05 versus baseline. Gastrointestinal side effects occurred in three patients (4.8%), including two on calcitriol-phosphate therapy and one on etidronate therapy. Of the six (9.5%) patients who experienced incident fractures, four were on etidronate, one on calcitriol-phosphate, and one on calcium-vitamin D. No patients discontinued their treatment because of side effects. Etidronate and the combination of calcitriol-phosphate produce a significant increase in lumbar spine bone mass in men with idiopathic osteoporosis or moderate idiopathic phosphate diabetes.

Leptin concentration in children with juvenile idiopathic arthritis.

PubMed

Maciejewska-Paszek, Izabela; Grochowska-Niedworok, Elżbieta; Siwiec, Andrzej; Dul, Lechosław; Gruenpeter, Anna; Szczerba, Henryk; Irzyniec, Tomasz

2015-01-01

Leptin regulates the organism's immune response. Juvenile idiopathic arthritis (JIA) is a chronic joint disease in children, leading to chronic changes in motor organs. In children with JIA (n = 42) and healthy subjects (n = 28), leptin concentration (LEP), body mass index (BMI), haematocrit (HTC), haemoglobin (HB), morphotic elements (WBC,LYMPH), erythrocyte sedimentation rate (ESR), and ANA Hep-2 antibodies were analysed. JIA group was divided into: children with a longer (51-148 months) (IA) n = 22 and a shorter disease period (2-18 months) (IB) n = 20. Only 58.3% of the IA and 50% of the IB group had ANA Hep-2 confirmed. The ill children had higher and more diversified LYMPH and ESR levels compared to the healthy children. The highest LEP for the IA group was 37.5 ng/cm3, (Me 5.85), for IB - 40.10 ng/cm3, (Me 2.46) as compared to the IC - 3.74 ng/cm3 (Me 2.85), respectively. The average BMI value for the IA group was 16.61 kg/m2, for IC it was 18.91 kg/m2, and the median for IB was 15.89 kg/m2. Children with BMI values < 23 kg/m2 from the IA and IB group had a reduction in LEP as compared to control group (p = 0.04). The relationship between the illness and LEP diversification per BMI unit was found in both groups. Children with a shorter illness period had higher LEP differentiation per BMI unit compared to the healthy children. Children with juvenile idiopathic arthritis with BMI < 23 kg/m2 had lower leptin concentrations than healthy subjects. Ill children with a shorter-term disease had a higher diversification of leptin concentration per BMI unit as compared to healthy controls.

Long-term results after Boston brace treatment in late-onset juvenile and adolescent idiopathic scoliosis.

PubMed

Lange, Johan Emil; Steen, Harald; Gunderson, Ragnhild; Brox, Jens Ivar

2011-08-31

It is recommended that research in patients with idiopathic scoliosis should focus on short- and long-term patient-centred outcome. The aim of the present study was to evaluate outcome in patients with late-onset juvenile or adolescent idiopathic scoliosis 16 years or more after Boston brace treatment. 272 (78%) of 360 patients, 251 (92%) women, responded to follow-up examination at a mean of 24.7 (range 16 - 32) years after Boston brace treatment. Fifty-eight (21%) patients had late-onset juvenile and 214 had adolescent idiopathic scoliosis. All patients had clinical and radiological examination and answered a standardised questionnaire including work status, demographics, General Function Score (GFS) (100 - worst possible) and Oswestry Disability Index (ODI) (100 - worst possible), EuroQol (EQ-5D) (1 - best possible), EQ-VAS (100 - best possible), and Scoliosis Research Society - 22 (SRS - 22) (5 - best possible). The mean age at follow-up was 40.4 (31-48) years. The prebrace major curve was in average 33.2 (20 - 57)°. At weaning and at the last follow-up the corresponding values were 28.3 (1 - 58)° and 32.5 (7 - 80)°, respectively. Curve development was similar in patients with late-onset juvenile and adolescent start. The prebrace curve increased > 5° in 31% and decreased > 5° in 26%. Twenty-five patients had surgery. Those who did not attend follow-up (n = 88) had a lower mean curve at weaning: 25.4 (6-53)°. Work status was 76% full-time and 10% part-time. Eighty-seven percent had delivered a baby, 50% had pain in pregnancy. The mean (SD) GFS was 7.4 (10.8), ODI 9.3 (11.0), EQ-5D 0.82 (0.2), EQ-VAS 77.6 (17.8), SRS-22: pain 4.1 (0.8), mental health 4.1 (0.6), self-image 3.7 (0.7), function 4.0 (0.6), satisfaction with treatment 3.7 (1.0). Surgical patients had significantly reduced scores for SRS-physical function and self-image, and patients with curves ≥ 45° had reduced self-image. Long-term results were satisfactory in most braced patients and

[Medical treatment of juvenile idiopathic arthritis].

PubMed

Horneff, Gerd; Augustin, Sankt

2008-09-01

Juvenile idiopathic arthritis is the most common chronic autoimmune disease. The outcome of this inflammatory disease is uncertain. Patients may suffer from severe joint damage leading to mutilations as well as from extraarticular manifestations. The prognosis is variable and depends in part on the number of affected joints and the occurrence of extraarticular manifestations. Pharmacomedical treatment has changed markedly in the last decade. It consists of a combination therapy including nonsteroidal antirheumatics, glucocorticoids either systemic or intraarticular, classical disease modifying drugs like sulfasalazine and methotrexate as well as leflunomide and biologicals. These new therapeutic strategies have effected dramatic improvements also in patients with severe, so far intractable disease. The TNF inhibitors etanercept and adalimumab have succeeded in double blind controlled trials, while infliximab failed to show significant superiority over placebo. Further treatment options include inhibitors of interleukin 1 (anakinra and rilonacept), interleukin 6 (tocilizumab) and inhibitors of T-cell activation (abatacept). This review will summarize the pharmacotherapeutic options based on studies published in the literature.

The prevalence of intraspinal anomalies in infantile and juvenile patients with "presumed idiopathic" scoliosis: a MRI-based analysis of 504 patients.

PubMed

Zhang, Wen; Sha, Shifu; Xu, Leilei; Liu, Zhen; Qiu, Yong; Zhu, Zezhang

2016-04-27

Though several studies have reported the incidence of intraspinal neural axis abnormalities in infantile and juvenile "presumed idiopathic" scoliosis, there has been a varying prevalence ranging from 11.1 to 26.0% based on a limited sample size. Therefore, such inconclusive findings have resulted in some questions on the MRI-associated role in the management of these patients. We aimed to investigate the prevalence and distribution of intraspinal anomalies in the infantile and juvenile patients with "presumed idiopathic" scoliosis and to explore the radiographic and clinical indicators with large sample size. A total of 504 infantile and juvenile patients diagnosed with "presumed idiopathic" scoliosis were examined for potentially-existing neural axis abnormalities by MRI. Patients were grouped into two cohorts according to the presence of neural axis abnormalities. Radiographic parameters including curve magnitude, curve pattern, location of apex, degree of thoracic kyphosis, and span of curve were recorded and compared between the two groups. The prevalence of the neural abnormalities between the infantile-age group and juvenile-age group was also compared. The student t test was used to evaluate the differences of continuous variables and the chi-square test was used to evaluate the difference of categorical variables. Fisher exact test was applied to detect the difference of the rate of intraspinal anomalies between the "infantile idiopathic scoliosis" and "juvenile idiopathic scoliosis" group. Involving the spinal cord, 94 patients (18.7%) were found to have a neural abnormality: Arnold-Chiari malformation alone in 43 patients, Arnold-Chiari malformation combined with syringomyelia in 18 patients, isolated syringomyelia in 13 patients, diastematomyelia in six patients, tethered cord combined with diastematomyelia in six patients, tethered cord alone in four patients, and other uncommon intraspinal abnormalities in the remaining four patients. Totally Arnold

Developments in the Classification and Treatment of the Juvenile Idiopathic Inflammatory Myopathies

PubMed Central

Rider, Lisa G.; Katz, James D.; Jones, Olcay Y.

2013-01-01

The juvenile idiopathic inflammatory myopathies (JIIM) are rare, heterogeneous autoimmune diseases that share chronic muscle inflammation and weakness. JIIM broadly includes three major clinicopathologic groups: juvenile dermatomyositis, juvenile polymyositis, and overlap myositis. A growing spectrum of clinicopathologic groups and serologic phenotypes defined by the presence of myositis-specific or myositis-associated autoantibodies are now recognized, each with differing demographics, clinical manifestations, laboratory findings, and prognoses. With the first multi-center collaborative studies and controlled trials using standardized preliminarily validated outcome measures, the therapy of juvenile myositis has advanced. Although daily oral corticosteroids remain the backbone of treatment, disease-modifying anti-rheumatic drugs (DMARDs) are almost always used as adjunctive therapy. Methotrexate is the conventional DMARD for the initial therapy, either alone or combined with intravenous pulse methylprednisolone, and/or intravenous immunoglobulin for patients with moderate to severe disease. Cyclosporine may be added to these or serve as an alternative to methotrexate. Other drugs and biologic therapies, including mycophenolate mofetil, tacrolimus, cyclophosphamide, rituximab, and infliximab, might benefit selected patients with recalcitrant disease, unacceptable steroid toxicity, or patients with risk factors for poor prognosis. The treatment of cutaneous disease, calcinosis, and the role for rehabilitation are also discussed. PMID:24182859

SECONDARY OSTEOPOROSIS: PATHOPHYSIOLOGY AND MANAGEMENT

PubMed Central

Mirza, Faryal; Canalis, Ernesto

2015-01-01

Osteoporosis is a skeletal disorder characterized by decreased bone mineral density and compromised bone strength predisposing to an increased risk of fractures. Although idiopathic osteoporosis is the most common form of osteoporosis, secondary factors may contribute to the bone loss and increased fracture risk in patients presenting with fragility fractures or osteoporosis. Several medical conditions and medications significantly increase the risk for bone loss and skeletal fragility. This review focuses on some of the common causes of osteoporosis, addressing the underlying mechanisms, diagnostic approach and treatment of low bone mass in the presence of these conditions. PMID:25971649

25-hydroxyvitamin D levels and juvenile idiopathic arthritis: is there an association with disease activity?

USDA-ARS?s Scientific Manuscript database

To examine the association between serum levels of 25-hydroxyvitamin D [25(OH)D] and disease activity in juvenile idiopathic arthritis (JIA), to determine the prevalence of vitamin D (VD) deficiency [25(OH)D=19 ng/ml] and insufficiency [25(OH)D 20-29 ng/ml], and to determine factors associated with ...

[Interdisciplinary and evidence-based treatment guideline for juvenile idiopathic arthritis].

PubMed

Guellac, N; Niehues, T

2008-01-01

Treatment of Juvenile Idiopathic Arthritis (JIA) has improved quality of life in children and adolescents suffering from JIA. However, it varies considerably from caregiver to caregiver. Therefore a standardisation of care on the basis of consensus treatment recommendations offers the chance to further improve the quality of care for children and adolescents with JIA. We aimed to establish an interdisciplinary, evidence-based treatment guideline for JIA based on the existing guideline from 2005. We did a systematic literature analysis in PUBMED with the key words "juvenile idiopathic (rheumatoid) arthritis" and "therapy". As limits in PUBMED we used: humans, published in the last 3 years, all child 0-18 years, clinical trial. Studies relating to diagnosis of JIA, Uveitis, vaccination, transition and rofexocibe were excluded. Authors of the 2005 guideline and representatives nominated by different societies were invited to attend the consensus conferences which were hosted by a professional moderator. Following societies were invited: Berufsverband der Kinder- und Jugendärzte (BVKJ), Deutsche Gesellschaft für Kinder- und Jugendmedizin (DGKJ), Deutsche Gesellschaft für Rheumatologie (DGRh), Deutsche Ophthalmologische Gesellschaft (DOG), Deutsche Rheuma-Liga Bundesverband, Verein zur Förderung und Unterstützung rheumatologisch erkrankter Kinder und deren Eltern, Vereinigung für Kinderorthopädie, Zentraler Verband der Physiotherapeuten und Krankengymnasten (ZVK). Consensus conferences took place in Düsseldorf on May 9th and August 1st 2007 and were each attended by more than 95% of the nominated representatives Finally, statements were confirmed in a Delphi method. Consensus statements regarding drug therapy, symptomatic and surgical management of JIA were compiled and judged strictly by the criteria of Evidence-Based Medicine (EBM).

Access to pediatric rheumatology care for Juvenile Idiopathic Arthritis in the United Arab Emirates.

PubMed

Khawaja, Khulood; Al-Maini, Mustafa

2017-05-16

This study looks at access to care for Juvenile Idiopathic Arthritis through pediatric rheumatology in the UAE, as an example of multi-ethnic society. Patients with a diagnosis of Juvenile idiopathic arthritis were identified through the hospital electronic medical records system from January 1st 2011 to December 31st 2014. All residents of the United Arab Emirates hold an Emirates identity card. We divided our patients into two groups: Emirati-Emirates, who are native Emirati children and hold the Emirati nationality, as stated on their Emirates identity card, and who therefore have full, comprehensive access to free medical care; and non-Emirati-Emirates, who represent other nationalities, as stated on their Emirates identity card. The primary objective of this study is to look at access to care for Juvenile idiopathic arthritis through pediatric rheumatology in the two groups. The secondary objective is to look at the effect of having multiple types of healthcare insurance coverage on access to biologics. A retrospective review was carried out. Sixty-six patients with JIA identified: 33 Emirates and 33 non-Emirates. For Emirates, the mean time from onset to first appointment with pediatric rheumatologist and diagnosis is 9 months (range: 1-48), and for non-Emirates is 12.4 months (range: 1-96). Among the Emirates, 10 patients are currently on biologic with methotrexate. Among the non-Emirates, 15 are on biologic with methotrexate. Among the Emirates, 12 are currently in remission while on treatment, as are 10 non-Emirates. Regarding disability, one Emirati patient has blindness secondary to noncompliance while under previous treatment. One Non-Emirati developed joint deformities due to periods of noncompliance and no follow up. Delay in presentation to pediatric rheumatology has been identified as an important factor in our population, which is multi-cultural and multi-ethnic. Type of health care insurance cover did not affect number of patients getting

Abatacept in children with juvenile idiopathic arthritis: a randomised, double-blind, placebo-controlled withdrawal trial.

PubMed

Ruperto, Nicolino; Lovell, Daniel J; Quartier, Pierre; Paz, Eliana; Rubio-Pérez, Nadina; Silva, Clovis A; Abud-Mendoza, Carlos; Burgos-Vargas, Ruben; Gerloni, Valeria; Melo-Gomes, Jose A; Saad-Magalhães, Claudia; Sztajnbok, Flavio; Goldenstein-Schainberg, Claudia; Scheinberg, Morton; Penades, Immaculada Calvo; Fischbach, Michael; Orozco, Javier; Hashkes, Philip J; Hom, Christine; Jung, Lawrence; Lepore, Loredana; Oliveira, Sheila; Wallace, Carol A; Sigal, Leonard H; Block, Alan J; Covucci, Allison; Martini, Alberto; Giannini, Edward H

2008-08-02

Some children with juvenile idiopathic arthritis either do not respond, or are intolerant to, treatment with disease-modifying antirheumatic drugs, including anti-tumour necrosis factor (TNF) drugs. We aimed to assess the safety and efficacy of abatacept, a selective T-cell costimulation modulator, in children with juvenile idiopathic arthritis who had failed previous treatments. We did a double-blind, randomised controlled withdrawal trial between February, 2004, and June, 2006. We enrolled 190 patients aged 6-17 years, from 45 centres, who had a history of active juvenile idiopathic arthritis; at least five active joints; and an inadequate response to, or intolerance to, at least one disease-modifying antirheumatic drug. All 190 patients were given 10 mg/kg of abatacept intravenously in the open-label period of 4 months. Of the 170 patients who completed this lead-in course, 47 did not respond to the treatment according to predefined American College of Rheumatology (ACR) paediatric criteria and were excluded. Of the patients who did respond to abatacept, 60 were randomly assigned to receive 10 mg/kg of abatacept at 28-day intervals for 6 months, or until a flare of the arthritis, and 62 were randomly assigned to receive placebo at the same dose and timing. The primary endpoint was time to flare of arthritis. Flare was defined as worsening of 30% or more in at least three of six core variables, with at least 30% improvement in no more than one variable. We analysed all patients who were treated as per protocol. This trial is registered, number NCT00095173. Flares of arthritis occurred in 33 of 62 (53%) patients who were given placebo and 12 of 60 (20%) abatacept patients during the double-blind treatment (p=0.0003). Median time to flare of arthritis was 6 months for patients given placebo (insufficient events to calculate IQR); insufficient events had occurred in the abatacept group for median time to flare to be assessed (p=0.0002). The risk of flare in patients

[The temporomandibular joint in juvenile idiopathic arthritis: what radiologists need to look for on magnetic resonance imaging].

PubMed

De La Hoz Polo, M; Navallas, M

2014-01-01

The term "juvenile idiopathic arthritis" (JIA) encompasses a group of arthritis of unknown cause with onset before the age of 16 years that last for at least 6 weeks. The prevalence of temporomandibular joint involvement in published series ranges from 17% to 87%. Temporomandibular joint involvement is difficult to detect clinically, so imaging plays a key role in diagnosis and monitoring treatment. MRI is the technique of choice for the study of arthritis of the temporomandibular joint because it is the most sensitive technique for detecting acute synovitis and bone edema. Power Doppler ultrasonography can also detect active synovitis by showing the hypervascularization of the inflamed synovial membrane, but it cannot identify bone edema. This article describes the MRI technique for evaluating the temporomandibular joint in patients with juvenile idiopathic arthritis, defines the parameters to look for, and illustrates the main findings. Copyright © 2013 SERAM. Published by Elsevier Espana. All rights reserved.

[Juvenile idiopathic arthritis and oral health].

PubMed

Kobus, Agnieszka; Kierklo, Anna; Sielicka, Danuta; Szajda, Sławomir Dariusz

2016-05-04

Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health.

The role of heme oxygenase-1 in systemic-onset juvenile idiopathic arthritis.

PubMed

Takahashi, Akitaka; Mori, Masaaki; Naruto, Takuya; Nakajima, Shoko; Miyamae, Takako; Imagawa, Tomoyuki; Yokota, Shumpei

2009-01-01

We have determined the serum levels of heme oxygenase-1 (HO-1) in 56 patients with systemic-onset juvenile idiopathic arthritis (s-JIA) and compared these with serum HO-1 levels in healthy controls and patients with other pediatric rheumatic diseases. Serum HO-1 levels were measured by the sandwich enzyme-linked immunosorbent assay. The mean serum HO-1 level in s-JIA patients during the active phase was 123.6 +/- 13.83 ng/ml, which was significantly higher than that in patients with polyarticular juvenile idiopathic arthritis (p-JIA), Kawasaki disease, systemic lupus erythematosus or mixed connective tissue disease (P < 0.0005). The serum levels of HO-1, cytokines and cytokine receptors in patients with s-JIA were also assessed at both the active and inactive phases. The serum HO-1 level in patients with s-JIA in the active phase was found to be significantly greater than that in patients with the disease in the inactive phase (P < 0.0001). An assessment of the relationships between serum HO-1 levels and other laboratory parameters or cytokines in patients with s-JIA did not reveal any strong correlations. These results suggest that the serum level of HO-1 may be a useful marker for the differential diagnosis of s-JIA. Further study will be necessary to elucidate the mechanism of HO-1 production and to clarify the role of HO-1 in the disease process.

Juvenile idiopathic arthritis-associated uveitis.

PubMed

Clarke, Sarah L N; Sen, Ethan S; Ramanan, Athimalaipet V

2016-04-27

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.

Transforming Growth Factor-Beta (TGF-β) Signaling in Paravertebral Muscles in Juvenile and Adolescent Idiopathic Scoliosis

PubMed Central

Kwiecien, Magdalena

2014-01-01

Most researchers agree that idiopathic scoliosis (IS) is a multifactorial disease influenced by complex genetic and environmental factors. The onset of the spinal deformity that determines the natural course of the disease, usually occurs in the juvenile or adolescent period. Transforming growth factors β (TGF-βs) and their receptors, TGFBRs, may be considered as candidate genes related to IS susceptibility and natural history. This study explores the transcriptional profile of TGF-βs, TGFBRs, and TGF-β responsive genes in the paravertebral muscles of patients with juvenile and adolescent idiopathic scoliosis (JIS and AIS, resp.). Muscle specimens were harvested intraoperatively and grouped according to the side of the curve and the age of scoliosis onset. The results of microarray and qRT-PCR analysis confirmed significantly higher transcript abundances of TGF-β2, TGF-β3, and TGFBR2 in samples from the curve concavity of AIS patients, suggesting a difference in TGF-β signaling in the pathogenesis of juvenile and adolescent curves. Analysis of TGF-β responsive genes in the transcriptomes of patients with AIS suggested overrepresentation of the genes localized in the extracellular region of curve concavity: LTBP3, LTBP4, ITGB4, and ITGB5. This finding suggests the extracellular region of paravertebral muscles as an interesting target for future molecular research into AIS pathogenesis. PMID:25313366

Advances in the treatment of polyarticular juvenile idiopathic arthritis

PubMed Central

Webb, Kate; Wedderburn, Lucy R.

2015-01-01

Purpose of review To review recent advances in the management strategies of polyarticular course juvenile idiopathic arthritis (JIA) and identify unanswered questions and avenues for further research. Recent findings There is evidence for an early, aggressive, treat-to-target approach for polyarticular JIA. Clinical disease activity criteria have been recently defined and validated, including criteria for inactive disease and the juvenile arthritis disease activity score (JADAS). There is a need for evidence-based, defined disease targets and biomarkers for prediction of response, including targets for remission induction, and guidelines on drug withdrawal. Recent treatment consensus plans and guidelines are discussed and compared, including the 2015 NHS England clinical policy statement, the 2014 Childhood Arthritis and Rheumatology Research Alliance (CARRA) treatment plans and the 2011 American College of Rheumatology (ACR) guidelines. Evidence for new agents such as tocilizumab, rituximab, golimumab, ustekinumab, certolizumab and tofacitinib is promising: the recent clinical trials are summarized here. Stratification of individual patient treatment remains a goal, and predictive biomarkers have been shown to predict success in the withdrawal of methotrexate therapy. Summary There are promising advances in the treatment approaches, disease activity criteria, clinical guidelines, pharmaceutical choices and individually stratified therapy choices for polyarticular JIA. PMID:26147756

Scoliosis elasticity assessed by manual traction: 49 juvenile and adolescent idiopathic cases.

PubMed

Soucacos, P K; Soucacos, P N; Beris, A E

1996-04-01

We assessed preoperative curve elasticity in 49 consecutive patients with juvenile or adolescent idiopathic scoliosis who were operated on with Harrington distraction rods. Preoperatively, the curve was determined from posteroanterior radiographs taken in the standing position and in the supine position, with traction. In the latter, the radiographs were taken at the moment of maximal traction when one technician applied traction to the ankles and another to the wrists. The scoliotic curve in the 10 patients with juvenile scoliosis averaged 59 degrees and 32 degrees in the standing and supine positions with traction, respectively. Immediately postoperatively, the curve averaged 19 degrees. 39 patients with adolescent scoliosis had a scoliotic curve which averaged 58 degrees in the standing position and 32 degrees in the supine position with traction. The mean postoperative measurement was 21 degrees. These findings suggest that manual traction is a simple and reliable means of predicting the minimal correction of the scoliotic curve to be expected, using Harrington distraction rods.

Judicious use of biologicals in juvenile idiopathic arthritis.

PubMed

Zhao, Yongdong; Wallace, Carol

2014-11-01

Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disorder that may cause joint destruction. Biological treatments targeting specific cytokines and cell interactions have transformed the outcomes of JIA. This review focuses on the selection of patients for and the timing and selection of biological treatment in JIA. Tumor necrosis factor (TNF) inhibitors remain the first choice for polyarticular JIA, followed by abatacept and tocilizumab. Monoclonal-antibody TNF inhibitors and abatacept are usually chosen for methotrexate-resistant uveitis. Recent clinical trials of canakinumab, rilonacept, and tocilizumab have obtained great improvement in both systemic and arthritic features in chronic systemic JIA patients. Current guidelines support the early use of a short-acting IL-1 antagonist for macrophage activation syndrome, a life-threatening complication. TREAT and ACUTE studies suggest that a therapeutic window of opportunity during early disease may exist in JIA. Early initiation of biological therapy may be associated with slower progression of joint damage and longer remission.

Methotrexate-induced nausea in the treatment of juvenile idiopathic arthritis.

PubMed

Falvey, Sonja; Shipman, Lauren; Ilowite, Norman; Beukelman, Timothy

2017-06-19

Methotrexate is the most commonly used disease modifying antirheumatic drug in the treatment of juvenile idiopathic arthritis and can be effective in controlling disease in many patients. A significant proportion of patients experience nausea and vomiting induced by methotrexate therapy, which can lead to decreased quality of life and discontinuation of treatment with methotrexate. Many strategies have been employed in attempts to reduce methotrexate-induced nausea, including folate supplementation, switching from oral to subcutaneous methotrexate, anti-emetic therapy, behavioral therapy, and others. Anticipatory nausea can be difficult to treat, making primary prevention of nausea with anti-emetics an attractive approach. Understanding the prevalence and impact of methotrexate-induced nausea, as well as potentially effective interventions, may help maximize the therapeutic benefits of methotrexate.

Australian Paediatric Rheumatology Group standards of care for the management of juvenile idiopathic arthritis.

PubMed

Munro, Jane; Murray, Kevin; Boros, Christina; Chaitow, Jeffrey; Allen, Roger C; Akikusa, Jonathan; Adib, Navid; Piper, Susan E; Singh-Grewal, Davinder

2014-09-01

This standards document outlines accepted standards of management for children, adolescents and young adults with juvenile idiopathic arthritis (JIA) in Australia. This document acknowledges that the chronic inflammatory arthritis conditions (JIA) in childhood are different diseases from inflammatory arthritis in adults and that specific expertise is required in the care of children with arthritis. © 2014 The Authors. Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Uveitis associated with juvenile idiopathic arthritis.

PubMed

Sen, Ethan S; Dick, Andrew D; Ramanan, Athimalaipet V

2015-06-01

Uveitis is a potentially sight-threatening complication of juvenile idiopathic arthritis (JIA). JIA-associated uveitis is recognized to have an autoimmune aetiology characterized by activation of CD4(+) T cells, but the underlying mechanisms might overlap with those of autoinflammatory conditions involving activation of innate immunity. As no animal model recapitulates all the features of JIA-associated uveitis, questions remain regarding its pathogenesis. The most common form of JIA-associated uveitis is chronic anterior uveitis, which is usually asymptomatic initially. Effective screening is, therefore, essential to detect early disease and commence treatment before the development of visually disabling complications, such as cataracts, glaucoma, band keratopathy and cystoid macular oedema. Complications can result from uncontrolled intraocular inflammation as well as from its treatment, particularly prolonged use of high-dose topical corticosteroids. Accumulating evidence supports the early introduction of systemic immunosuppressive drugs, such as methotrexate, as steroid-sparing agents. Prospective randomized controlled trials of TNF inhibitors and other biologic therapies are underway or planned. Future research should aim to identify biomarkers to predict which children are at high risk of developing JIA-associated uveitis or have a poor prognosis. Such biomarkers could help to ensure that patients receive earlier interventions and more-potent therapy, with the ultimate aim of reducing loss of vision and ocular morbidity.

Identification of dendritic cells in the blood and synovial fluid of children with Juvenile Idiopathic Arthritis.

PubMed

Tabarkiewicz, Jacek; Postępski, Jacek; Olesińska, Edyta; Roliński, Jacek; Tuszkiewicz-Misztal, Ewa

2011-01-01

Childhood chronic arthritis of unknown etiology is known collectively as juvenile idiopathic arthritis (JIA) and consists of heterogeneous subtypes with unique clinical patterns of disease. JIA is the commonest rheumatic disease in children and may still result in significant disability, with joint deformity, growth impairment, and persistence of active arthritis into adulthood. Basic research is rather focused on rheumatoid arthritis, and this lead to small number of publications considering JIA. In this study we examine, by flow cytometry, the expression of dendritic cells (DCs) in the peripheral blood and synovial fluid of children with active JIA in a group of 220 patients. We reveal a significant decrease in the percentage of immature DCs in the blood of patients compared to control children. Surprisingly, we found higher percentages of mature circulating dendritic cells. Both populations of DCs, immature and mature, were accumulated in patients' synovial fluid. We also confirmed the presence of CD206+/CD209+ in JIA samples, which can represent a population of macrophages with dendritic cells morphology. Our results support the thesis that dendritic cells are crucial in the induction and maintenance of autoimmune response and local inflammation during juvenile idiopathic arthritis.

The Natural History of Idiopathic Scoliosis During Growth: A Meta-Analysis.

PubMed

Di Felice, Francesca; Zaina, Fabio; Donzelli, Sabrina; Negrini, Stefano

2018-05-01

The aim of the study was to provide a meta-analysis of current literature concerning the natural history of idiopathic scoliosis during growth. A comprehensive search of Medline, Embase, And Scopus databases was conducted up to November 2016. Eligible works were prospective or retrospective studies that enrolled patients with infantile idiopathic scoliosis, juvenile idiopathic scoliosis, or adolescent idiopathic scoliosis, followed up without any treatment from the time of detection. A meta-analysis for proportion was performed. The following studies were grouped per diagnosis: infantile idiopathic scoliosis, juvenile idiopathic scoliosis, and adolescent idiopathic scoliosis. Of the 1797 citations screened, we assessed 61 full-text articles and included 13 of these (2301 participants). Three studies included infantile idiopathic scoliosis patients (347 participants), five studies included a mixed population of juvenile idiopathic scoliosis and adolescent idiopathic scoliosis (1330 participants), and five studies included adolescent idiopathic scoliosis patients only (624 participants). The random pooled estimated progression rate was 49% (95% confidence interval = 1%-97%) for infantile idiopathic scoliosis, 49% in a mixed group of patients affected by juvenile idiopathic scoliosis or adolescent idiopathic scoliosis (95% confidence interval = 19%-79%), and 42% in adolescent idiopathic scoliosis (95% confidence interval = 11%-73%). During growth, idiopathic scoliosis tends to progress in a high percentage of cases. The progression rate varies according to the age at diagnosis, with infantile scoliosis being the most unpredictable. There are many confounders, such as age, Risser sign and baseline Cobb angles that were not consistent among studies, making the data very heterogeneous.

Juvenile Arthritis

MedlinePlus

Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

Methotrexate therapy may prevent the onset of uveitis in juvenile idiopathic arthritis.

PubMed

Papadopoulou, Charalampia; Kostik, Mikhail; Böhm, Marek; Nieto-Gonzalez, Juan Carlos; Gonzalez-Fernandez, Maria Isabel; Pistorio, Angela; Martini, Alberto; Ravelli, Angelo

2013-09-01

To evaluate whether early treatment with methotrexate (MTX) prevents the onset of uveitis in children with juvenile idiopathic arthritis. The clinical charts of all consecutive patients seen between January 2002 and February 2011 who had a disease duration <1 year at first visit and had received a stable management for at least 2 years with or without MTX were reviewed. Patients who were given systemic medications other than MTX (except nonsteroidal anti-inflammatory drugs) were excluded. Patients with systemic arthritis, rheumatoid factor-positive arthritis, or enthesitis-related arthritis were also excluded. In each patient, the 2-year follow-up period after first visit was examined to establish whether uveitis had occurred. A total of 254 patients with a median disease duration of 0.3 year were included. Eighty-six patients (33.9%) were treated with MTX, whereas 168 patients (66.1%) did not receive MTX. During the 2-year follow-up, 211 patients (83.1%) did not develop uveitis, whereas 43 patients (16.9%) had uveitis a median of 1.0 year after the first visit. The frequency of uveitis was lower in MTX-treated than in MTX-untreated patients (10.5% vs 20.2%, respectively, P = .049). Survival analysis confirmed that patients treated with MTX had a lower probability of developing uveitis. Early MTX therapy may prevent the onset of uveitis in children with juvenile idiopathic arthritis. Because our study may be affected by confounding by indication, the potential of MTX to reduce the incidence of ocular disease should be investigated in a randomized controlled trial. Copyright © 2013 Mosby, Inc. All rights reserved.

Differential diagnosis and secondary causes of osteoporosis.

PubMed

Taxel, P; Kenny, A

2000-01-01

Secondary osteoporosis refers to osteoporosis in which an underlying cause or factor other than those attributable to the postmenopausal state or aging can be identified. Primary, or idiopathic, osteoporosis implies that a secondary cause cannot be found. Secondary osteoporosis occurs not only in postmenopausal women but also in men and premenopausal women. In series reported from specialized centers, as many as 30% of postmenopausal women and 50% to 80% of men have an identifiable secondary cause of osteoporosis, although the frequency of secondary osteoporosis is probably much lower in the general population. In assessing the patient with osteoporosis, it is important to look for secondary causes and aggravating factors that are reversible and amenable to therapy. In addition to secondary forms, 2 metabolic bone diseases, osteomalacia and primary hyperparathyroidism, can mimic or aggravate osteoporosis. This paper will summarize the differential diagnosis and management of osteoporosis, osteomalacia, and hyperparathyroidism and review the most common causes of secondary osteoporosis.

Effects on growth and body composition of growth hormone treatment in children with juvenile idiopathic arthritis requiring steroid therapy.

PubMed

Simon, Dominique; Lucidarme, Nadine; Prieur, Anne-Marie; Ruiz, Jean Charles; Czernichow, Paul

2003-11-01

Decreased growth velocity and abnormal body composition including severe osteoporosis are common in glucocorticoid-treated patients with juvenile idiopathic arthritis (JIA). We evaluated the effects of recombinant human growth hormone (GH) given for 3 years on growth velocity, height standard deviation score (SDS), and body composition, together with potential adverse effects on glucose tolerance. Thirteen patients received GH (0.46 mg/kg/week) for 3 years. Body composition was assessed by dual-energy x-ray absorptiometry and glucose tolerance by annual oral glucose tolerance tests. Median growth velocity increased from 2.1 to 6.0 cm/year (p = 0.002) in the first year and remained higher than baseline in the second year of treatment. Height SDS did not change significantly (-4.6 SDS at baseline vs -4.3 SDS at study completion), but the growth response varied markedly across patients. Compared with baseline, lean mass increased by 33%, fat mass remained stable, and lumbar bone mineral density increased by 36.6%. Transient glucose intolerance developed in 6 patients, but glycosylated hemoglobin concentrations did not change significantly and diabetes mellitus did not occur. Treatment with GH restored linear growth without inducing catch-up growth, significantly improved body composition, and prevented further bone loss. Prolonged followup is needed to assess the benefits of GH and longterm consequences of hyperinsulinism.

Update on the medical treatment of juvenile idiopathic arthritis.

PubMed

Hashkes, Philip J; Laxer, Ronald M

2006-12-01

Many exciting developments in the treatment of juvenile idiopathic arthritis (JIA) have emerged recently, including new tools to assess the results of clinical trials (eg, the definition of remission and a radiologic scoring tool). New controlled studies examined the equivalence of meloxicam to naproxen, the efficacy of leflunomide but the superiority of methotrexate, and the use of infliximab in polyarthritis JIA. Initial studies have shown the potential of anti-interleukin (IL)-1 and anti-IL-6 receptor antibody therapy for systemic JIA. Corticosteroid-sparing medications including the use of "biologic modifiers" for JIA-associated uveitis have been described. Evidence-based guidelines for the main subtypes of JIA have been published. However, good evidence on the treatment of several disease subtypes is still lacking. Studies of new medications and the use of combination therapy, including aggressive induction therapy early in the disease course, are necessary to continue improving the outcome of JIA patients.

Retrospective Study Evaluating Treatment Decisions and Outcomes of Childhood Uveitis Not Associated with Juvenile Idiopathic Arthritis.

PubMed

Sardar, Elham; Dusser, Perrine; Rousseau, Antoine; Bodaghi, Bahram; Labetoulle, Marc; Koné-Paut, Isabelle

2017-07-01

To evaluate treatment, ocular complications and outcomes of children with pediatric uveitis not associated with juvenile idiopathic arthritis. This was a retrospective chart review of pediatric uveitis in children under 16 years of age, recruited from the pediatric rheumatology department at Bicêtre Hospital from 2005 to 2015. Patients with juvenile idiopathic arthritis-associated and infectious uveitis were excluded. We used the Standardization of Uveitis Nomenclature Working Group to classify uveitis, disease activity, and treatment end points. We enrolled 56 patients and 102 affected eyes. The mean age at diagnosis was 10 ± 3.5 years (range 3-15), and the mean follow-up 4.2 ± 3.3 years (1-15). The main diagnoses were idiopathic (55%), Behçet disease (15%), and sarcoidosis (5%). The main localization was panuveitis in 44 of 102 eyes (43%). Corticosteroid sparing treatment was needed in 62 of 102 eyes (60%). Second-line therapies included methotrexate and azathioprine, and the third-line therapy was a biologic agent, mainly infliximab, in 33 of 102 eyes (32%). Infliximab achieved uveitis inactivity in 14 of 18 eyes (80%), in all etiologies. Severe complications were present in 68 of 102 eyes (67%). The most common were synechiae 33% of eyes, cataract (20%), and macular edema (25%). Of these, 37% were present at diagnosis. Remission was achieved in 22 of 102 eyes (21%). Conventional therapies were insufficient to treat many of the cases of posterior or panuveitis. This study underlines the need for earlier and more aggressive treatment and antitumor necrosis factor-α therapy was rapidly efficient in most cases of refractory uveitis. Copyright © 2017 Elsevier Inc. All rights reserved.

[Magnetic resonance imaging in juvenile idiopathic arthritis: peculiarities of imaging children].

PubMed

Navallas, M; Rebollo Polo, M; Riaza, L; Muchart López, J; Maristany, T

2013-09-01

The term juvenile idiopathic arthritis (JIA) encompasses a heterogeneous group of arthritides with no known cause that begin before the age of 16 years and persist for at least 6 weeks. In recent decades, imaging techniques have acquired a fundamental role in the diagnosis and follow-up of JIA, owing to the unification of the different criteria for classification, which has strengthened the research in this field, and to the development of disease-modifying antirheumatic drugs. In this article, we briefly explain what JIA is. Moreover, we describe the role and limitations of plain-film radiography, ultrasonography, and magnetic resonance imaging (MRI). Finally, we review the MRI protocol and findings, and we comment on the differential diagnosis. Copyright © 2012 SERAM. Published by Elsevier Espana. All rights reserved.

Juvenile Idiopathic Arthritis in Olmsted County, Minnesota, 1960-2013.

PubMed

Krause, Megan L; Crowson, Cynthia S; Michet, C John; Mason, Thomas; Muskardin, Theresa Wampler; Matteson, Eric L

2016-01-01

To evaluate the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Olmsted County, Minnesota in 1994-2013 and trends in juvenile rheumatoid arthritis (JRA) in 1960-2013. Cases of arthritis in 1994-2013 were identified by diagnosis code with medical chart review to confirm diagnosis separately for JIA and JRA. Overall incidence rates with 95% confidence intervals (95% CIs) were age and sex adjusted to the 2010 US white population. Comparisons were made with an earlier (1960-1993) cohort from this same population. Seventy-one incident cases of JIA in 1994-2013 were identified, with an overall age- and sex-adjusted incidence rate of 10.3 per 100,000 (95% CI 7.9-12.7). Forty-two (59%) were female, with an incidence of 12.4 per 100,000 (95% CI 8.6-16.2), as compared to 8.3 per 100,000 (95% CI 5.2-11.3) in males. The most common subtype was oligoarthritis (63%). The mean ± SD age at diagnosis was 8.2 ± 5.3 years. The prevalence of JIA on January 1, 2000 and January 1, 2010 was 51.0 per 100,000 (95% CI 25.2-76.8) and 57.6 per 100,000 (95% CI 31.0-94.5), respectively. When the annual incidence of JRA was compared over time from 1960 to 2013, there was no significant change in incidence overall; however, the incidence decreased among females (P = 0.003). A cyclic pattern of incidence was observed, with peaks approximately every 10 years. Similar to the findings with regard to incidence, prevalence did not change overall, but decreased among females (P = 0.048). There were 4 deaths in the cohort of JRA patients diagnosed in 1960-2013; the standardized mortality ratio was 1.50 (95% CI 0.41-3.83). Incidence of juvenile arthritis overall in Olmsted County, Minnesota has not changed significantly in the past 53 years. A consistent cyclic pattern was noted. © 2016, American College of Rheumatology.

[Sodium fluoride in the therapy of osteoporosis].

PubMed

Krokowski, E

1979-10-04

Every form of osteoporosis--except the physiological involution osteoporosis--requires treatment. Osteoporosis is no disease by itself but a result or a concomitant phenomenon of various diseases. Therefore the primary disease has to be treated also. Only for the so-called idiopathic osteoporosis hitherto no cause is known. Today sodium fluoride is the only substance with an influence upon the bone which may induce re-ossification and re-stabilization. In the described series of investigations this compound, with which treatment by gradual dosage is possible, has proved its effectiveness. Osteoporosis is more than bone porosity, therefore additionally afflicted organic or functional system like the muscular apparatus, bone marrow, or the intraosseous blood supply, must be treated too. Psychological guidance of the patient is necessary as he must be motivated for activity.

Biomarkers in systemic juvenile idiopathic arthritis: a comparison with biomarkers in cryopyrin-associated periodic syndromes.

PubMed

Nirmala, Nanguneri; Grom, Alexei; Gram, Hermann

2014-09-01

This review summarizes biomarkers in systemic juvenile idiopathic arthritis (sJIA). Broadly, the markers are classified under protein, cellular, gene expression and genetic markers. We also compare the biomarkers in sJIA to biomarkers in cryopyrin-associated periodic syndrome (CAPS). Recent publications showing the similarity of clinical response of sJIA and CAPS to anti-interleukin 1 therapies prompted a comparison at the biomarker level. sJIA traditionally is classified under the umbrella of juvenile idiopathic arthritis. At the clinical phenotypic level, sJIA has several features that are more similar to those seen in CAPS. In this review, we summarize biomarkers in sJIA and CAPS and draw upon the various similarities and differences between the two families of diseases. The main differences between sJIA and CAPS biomarkers are genetic markers, with CAPS being a family of monogenic diseases with mutations in NLRP3. There have been a small number of publications describing cellular biomarkers in sJIA with no such studies described for CAPS. Many of the protein marker's characteristics of sJIA are also seen to characterize CAPS. The gene expression data in both sJIA and CAPS show a strong upregulation of innate immunity pathways. In addition, we describe a strong similarity between sJIA and CAPS at the gene expression level in which several genes that form a part of the erythropoiesis signature are upregulated in both sJIA and CAPS.

Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis.

PubMed

Oray, Merih; Tuğal-Tutkun, İlknur

2016-04-01

Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA)-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual prognosis. The goal of treatment is to achieve enduring drug-free remission. The choice of therapeutic regimen needs to be tailored to each individual case. One must keep in mind that patients under immunomodulatory treatment should be monitored closely due to possible side effects. Local and systemic corticosteroids have long been the mainstay of therapy; however, long-term corticosteroid therapy should be avoided due to serious side effects. Steroid-sparing agents in the treatment of JIA-associated uveitis include antimetabolites and biologic agents in refractory cases. Among the various immunomodulatory agents, methotrexate is generally the first choice, as it has a well-established safety and efficacy profile in pediatric cases and does not appear to increase the risk of cancer. Other classic immunomodulators that may also be used in combination with methotrexate include azathioprine, mycophenolate mofetil, and cyclosporin A. Biologic agents, primarily tumor necrosis factor alpha inhibitors including infliximab or adalimumab, should be considered in cases of treatment failure with classic immunomodulatory agents.

Validity of juvenile idiopathic arthritis diagnoses using administrative health data.

PubMed

Stringer, Elizabeth; Bernatsky, Sasha

2015-03-01

Administrative health databases are valuable sources of data for conducting research including disease surveillance, outcomes research, and processes of health care at the population level. There has been limited use of administrative data to conduct studies of pediatric rheumatic conditions and no studies validating case definitions in Canada. We report a validation study of incident cases of juvenile idiopathic arthritis in the Canadian province of Nova Scotia. Cases identified through administrative data algorithms were compared to diagnoses in a clinical database. The sensitivity of algorithms that included pediatric rheumatology specialist claims was 81-86%. However, 35-48% of cases that were identified could not be verified in the clinical database depending on the algorithm used. Our case definitions would likely lead to overestimates of disease burden. Our findings may be related to issues pertaining to the non-fee-for-service remuneration model in Nova Scotia, in particular, systematic issues related to the process of submitting claims.

Chronic Non-infectious Uveitis in Patients with Juvenile Idiopathic Arthritis.

PubMed

Kolomeyer, Anton M; Tu, Yufei; Miserocchi, Elisabetta; Ranjan, Mangala; Davidow, Amy; Chu, David S

2016-08-01

To describe clinical findings and analyze treatment evolution of chronic, non-infectious uveitis in patients with juvenile idiopathic arthritis (JIA). A total of 82 patients (147 eyes) with JIA-related uveitis treated for ≥2 months were included (78% females; 79% bilateral uveitis; 74% anterior uveitis). Outcome measures were visual acuity (VA), inflammation control, side-effects, and surgical procedures. Mean ± SD age at diagnosis was 4.9 ± 3.8 years; mean ± SD follow-up time was 8.7 ± 7.8 years. Mean VA did not significantly change throughout the study. Three (2%) eyes resulted in no light perception (NLP) vision. Thirty (37%) patients underwent 69 procedures. In total, 41 (50%) patients achieved inflammation control. TNF-α inhibitors were significantly associated with inflammation control. Seven (8.5%) patients stopped treatment due to side-effects. JIA is a cause of significant ocular morbidity. TNF-α inhibitor use was associated with inflammation control. Prospective, randomized, double blind clinical trials in this regard are warranted.

Infection-Related Death among Persons with Refractory Juvenile Idiopathic Arthritis

PubMed Central

Lane, Jonathan P.; Wood, Mark; Friswell, Mark; Flood, Terence J.; Foster, Helen E.

2016-01-01

Severe infections are emerging as major risk factors for death among children with juvenile idiopathic arthritis (JIA). In particular, children with refractory JIA treated with long-term, multiple, and often combined immunosuppressive and antiinflammatory agents, including the new biological disease-modifying antirheumatic drugs (DMARDs), are at increased risk for severe infections and death. We investigated 4 persons with JIA who died during 1994–2013, three of overwhelming central venous catheter–related bacterial sepsis caused by coagulase-negative Staphylococus or α-hemolytic Streptococcus infection and 1 of disseminated adenovirus and Epstein-Barr virus infection). All 4 had active JIA refractory to long-term therapy with multiple and combined conventional and biological DMARDs. Two died while receiving high-dose systemic corticosteroids, methotrexate, and after recent exposure to anti–tumor necrosis factor-α biological DMARDs, and 2 during hematopoietic stem cell transplantation procedure. Reporting all cases of severe infections and especially deaths in these children is of paramount importance for accurate surveillance. PMID:27648582

Biomarkers in Systemic Juvenile Idiopathic Arthritis: A comparison with biomarkers in Cryopyrin Associated Periodic Syndromes

PubMed Central

Nirmala, Nanguneri; Grom, Alexei; Gram, Hermann

2015-01-01

Purpose of review This review summarizes biomarkers in Systemic Juvenile Idiopathic Arthritis (sJIA). Broadly, the markers are classified under protein, cellular, gene expression and genetic markers. We also compare the biomarkers in sJIA to biomarkers in cryopyrin associated periodic syndromes (CAPS). Recent findings Recent publications showing the similarity of clinical response of sJIA and CAPS to anti IL1 therapies prompted a comparison at the biomarker level. Summary sJIA traditionally is classified under the umbrella of juvenile idiopathic arthritis. At the clinical phenotypic level, sJIA has several features that are more similar to those seen in Cryopyrin Associated Periodic Syndromes (CAPS). In this review, we summarize biomarkers in sJIA and CAPS and draw upon the various similarities and differences between the two families of diseases. The main difference between sJIA and CAPS biomarkers are genetic markers with CAPS being a family of monogenic diseases with mutations in NLRP3. There have been a small number of publications describing cellular biomarkers in sJIA with no such studies described for CAPS. Many of the protein markers characteristic of sJIA are also seen to characterize CAPS. The gene expression data in both sJIA and CAPS show a strong upregulation of innate immunity pathways. In addition, we describe a strong similarity between sJIA and CAPS at the gene expression level where several genes that form a part of the erythropoiesis signature are upregulated in both sJIA and CAPS. PMID:25050926

Management of juvenile idiopathic arthritis: hitting the target.

PubMed

Hinze, Claas; Gohar, Faekah; Foell, Dirk

2015-05-01

The treatment of juvenile idiopathic arthritis (JIA) is evolving. The growing number of effective drugs has led to successful treatment and prevention of long-term sequelae in most patients. Although patients with JIA frequently achieve lasting clinical remission, sustained remission off medication is still elusive for most. Treatment approaches vary substantially among paediatric rheumatologists owing to the inherent heterogeneity of JIA and, until recently, to the lack of accepted and well-evidenced guidelines. Furthermore, many pertinent questions related to patient management remain unanswered, in particular regarding treatment targets, and selection, intensity and sequence of initiation or withdrawal of therapy. Existing JIA guidelines and recommendations do not specify treat-to-target or tight control strategies, in contrast to adult rheumatology in which these approaches have been successful. The concepts of window of opportunity (early treatment to improve long-term outcomes) and immunological remission (abrogation of subclinical disease activity) are also fundamental when defining treatment methodologies. This Review explores the application of these concepts to JIA and their possible contribution to the development of future clinical guidelines or consensus treatment protocols. The article also discusses how diverse forms of standardized, guideline-led care and personalized treatment can be combined into a targeted, patient-centred approach to optimize management strategies for patients with JIA.

The juvenile idiopathic inflammatory myopathies: pathogenesis, clinical and autoantibody phenotypes, and outcomes

PubMed Central

Rider, Lisa G.; Nistala, Kiran

2015-01-01

The aim of this review is to summarize recent advances in the understanding of the clinical and autoantibody phenotypes, their associated outcomes, and the pathogenesis of the juvenile idiopathic inflammatory myopathies (JIIMs). The major clinical and autoantibody phenotypes in children have many features similar to those in adults, and each has distinct demographic and clinical features and associated outcomes. The most common myositis autoantibodies in JIIM patients are anti-p155/140, anti-MJ, and anti-MDA5. Higher mortality has been associated with overlap myositis as well as with the presence of anti-synthetase and anti-MDA5 autoantibodies; a chronic illness course and lipodystrophy have been associated with anti-p155/140 autoantibodies; and calcinosis has been associated with anti-MJ autoantibodies. Histologic abnormalities of JIIMs detectable on muscle biopsy have also been correlated with myositis-specific autoantibodies; for example, patients with anti-MDA5 show low levels of inflammatory infiltrate and muscle damage on biopsy. The first genome-wide association study of adult and juvenile dermatomyositis revealed three novel genetic associations, BLK, PLCL1, and CCL21, and confirmed that the human leukocyte antigen region is the primary risk region for juvenile dermatomyositis. Here we review the well-established pathogenic processes in JIIMs, including the type 1 interferon and endoplasmic reticulum stress pathways. Several novel JIIM-associated inflammatory mediators, such as the innate immune system proteins, myeloid-related peptide 8/14, galectin 9, and eotaxin, have emerged as promising biomarkers of disease. Advances in our understanding of the phenotypes and pathophysiology of the JIIMs are leading to better tools to help clinicians stratify and treat these heterogeneous disorders. PMID:27028907

A survey of foot problems in juvenile idiopathic arthritis.

PubMed

Hendry, G; Gardner-Medwin, J; Watt, G F; Woodburn, J

2008-12-01

Evidence suggests that foot problems are common in juvenile idiopathic arthritis (JIA), with prevalence estimates over 90%. The aim of this survey was to describe foot-related impairment and disability associated with JIA and foot-care provision in patients managed under modern treatment paradigms, including disease-modifying anti-rheumatic drugs (DMARDs) and biologic therapies. The Juvenile Arthritis Foot Disability Index (JAFI), Child Health Assessment Questionnaire (CHAQ), and pain visual analogue scale (VAS) were recorded in 30 consecutive established JIA patients attending routine outpatient clinics. Foot deformity score, active/limited joint counts, walking speed, double-support time (s) (DS) and step length symmetry index % (SI) were also measured. Foot-care provision in the preceding 12 months was determined from medical records. Sixty-three per cent of children reported some foot impairment, with a median (range) JAFI subscale score of 1 (0-3); 53% reported foot-related activity limitation, with a JAFI subscale score of 1 (0-4); and 60% reported participation restriction, with a JAFI subscale score of 1 (0-3). Other reported variables were CHAQ 0.38 (0-2), VAS pain 22 (0-79), foot deformity 6 (0-20), active joints 0 (0-7), limited joints 0 (0-31), walking speed 1.09 m/s (0.84-1.38 m/s), DS 0.22 s (0.08-0.26 s) and SI +/-4.0% (+/-0.2-+/-31.0%). A total of 23/30 medical records were reviewed and 15/23 children had received DMARDS, 8/23 biologic agents and 20/23 multiple intra-articular corticosteroid injections. Ten children received specialist podiatry care comprising footwear advice, orthotic therapy and silicone digital splints together with intrinsic muscle strengthening exercises. Despite frequent use of DMARD/biologic therapy and specialist podiatry-led foot care, foot-related impairment and disability persists in some children with JIA.

Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis.

PubMed

Dewoolkar, Mansi; Cimaz, Rolando; Chickermane, Pranav Raman; Khubchandani, Raju P

2017-04-01

To assess the course, outcome and complications in a mono-centric cohort of 53 patients with systemic onset juvenile idiopathic arthritis (s-JIA). In an observational study, 53 consecutive patients diagnosed with s-JIA on or before October 2009 were enrolled and followed up between October 2009 and September 2012. At each 6-12 weekly visit, clinical examination, laboratory investigations and details of on-going treatment were recorded. Disease course was classified as monocyclic, intermittent and persistent. At last visit, outcome was studied with respect to remission (Wallace criteria) and Steinbrocker functional classification. Juvenile Arthritis Damage Index (JADI) was measured on a subset. In 53 patients analysed, the mean follow-up period was 5.5 ± 1.85 y, with a cumulative follow-up period of 291.5 patient-years. The mean age at diagnosis was 6.3 ± 3.4 y. Thirty-three patients suffered from disease and/or drug related complications. Infections were observed in 16 (30%) and macrophage activation syndrome in 5 (9.4%). Nine (17%) had a monocyclic course, 31 (58.5%) had an intermittent course and 13 (24.5%), a persistent course. At last visit, 9/9 patients of the monocyclic group, 17/31 in the intermittent group and 3/13 in the persistent group were in remission. At the end of the study, 96.2% of the index patients were Steinbrocker functional class I and II with the monocyclic group having the best functional outcome. JADI was performed on 20/53 patients. Nine had significant articular damage. The range of Juvenile arthritis damage index-articular (JADI-A) was 0-25/72 (median-6) and of Juvenile arthritis damage index-extra articular (JADI-EA) was 0-4/17 (median-1). The outcome of patients with s-JIA in a resource limited setting where early diagnosis, multidisciplinary care and availability of biologics are hurdles, is further altered by complications related to longstanding disease and over use of steroids.

Osteoporosis in premenopausal women.

PubMed

Langdahl, Bente L

2017-07-01

The scope of this review was to review the newest developments in the context of the existing knowledge on premenopausal bone fragility. Fragility fractures are common in postmenopausal women and men and diagnostic criteria for osteoporosis have been agreed and multiple pharmacological treatments have been developed over the last 25 years. In premenopausal women, fragility fractures and very low bone mass are uncommon and osteoporosis in premenopausal women has therefore attracted much less interest. Recent studies have highlighted that lifestyle and dietary habits affect premenopausal bone mass. Bone mass may be improved by sufficient intake of calcium and vitamin D together with increased physical activity in premenopausal women with idiopathic osteoporosis. If pharmacological treatment is needed, teriparatide has been demonstrated to efficiently increase bone mass; however, no fracture studies and no comparative studies against antiresorptive therapies have been conducted. Pregnancy affects bone turnover and mass significantly, but pregnancy-associated osteoporosis is a rare and heterogeneous condition. The diagnosis of osteoporosis should only be considered in premenopausal women with existing fragility fractures, diseases or treatments known to cause bone loss or fractures. Secondary causes of osteoporosis should be corrected or treated if possible. The women should be recommended sufficient intake of calcium and vitamin and physical activity. In women with recurrent fractures or secondary causes that cannot be eliminated, for example glucocorticoid or cancer treatment, pharmacological intervention with bisphosphonates or teriparatide (not in the case of cancer) may be considered.

Juvenile idiopathic arthritis profile in Turkish children.

PubMed

Yilmaz, Mustafa; Kendirli, Seval G; Altintas, Derya U; Karakoc, Gulbin B; Inal, Ayfer; Kilic, Mehmet

2008-04-01

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders. Publications from different countries point to differences in the disease manifestation of JIA among different populations. The aim of the present paper was to evaluate the clinical and laboratory features of JIA in Turkish children. A total of 196 JIA patients who fulfilled International League of Associations for Rheumatology (ILAR) diagnostic criteria were included in this retrospective study. The data collected were age, gender, age at disease onset and at diagnosis, and follow-up duration. Antinuclear antibody (ANA), rheumatoid factor (RF), and human leukocyte antigen B-27 were evaluated for each patient. There were 102 boys and 94 girls with a mean duration of disease of 4.1 years. The mean age at the first visit was 8.8 years, and the mean age at onset of disease was 6.8 years (range, 8 months-15 years). Polyarticular JIA was the most frequent onset type (37.2%). Other subtypes included oligoarthritis (34.2%), systemic arthritis (15.3%), psoriatic arthritis (1%), enthesitis-related arthritis (9.7%), and other arthritis (2.2%). ANA was positive in 28 patients (14.2%). Chronic uveitis occurred in two patients with oligoarthritis; and two patients with enthesitis-related arthritis had acute uveitis. Three patients (1.4%) developed amyloidosis. Compared to reports from Western countries, remarkably different features of JIA were found in Turkish children, which included higher frequency of polyarticular JIA, higher prevalence among boys, lower rate of ANA positivity and uveitis. Further studies are required to understand how genetic and environmental differences affect JIA expression.

Controversies in juvenile idiopathic arthritis-associated uveitis.

PubMed

Zierhut, Manfred; Heiligenhaus, Arnd; deBoer, Joke; Cunningham, Emmett T; Tugal-Tutkun, Ilknur

2013-06-01

Abstract Juvenile idiopathic arthritis-associated uveitis (JIAU) accounts for a sizable proportion of uveitis cases in children and is an important cause of ocular morbidity in uveitis patients in this age group. The authors present the results of a survey conducted to obtain a better understanding of the current views and practices of ophthalmologists involved in the care of children with JIAU. A detailed questionnaire consisting of 54 questions addressing epidemiology, diagnosis, and therapy of JIAU was distributed to 67 uveitis specialists. The responses from 37 completed questionnaires were tabulated for this report. While the experts often agreed on aspects of the epidemiologic and clinical features of JIAU and its complications, considerable diversity of responses was noted-particularly with regard to practice patterns. Regarding diagnostics and disease monitoring, all experts favored ANA testing, whereas two-thirds also suggested HLA-B27 typing. Laser flare photometry was available to and routinely used by almost one-third of the experts. Optical coherence tomography (OCT) was used by more than half. The survey revealed an overall consensus on therapeutic strategies, including the use of both conventional immunosuppressive and biologic agents. Methotrexate was the initial choice for immunosuppression by most respondents. Most would add an anti-TNF-alpha agent following failure of traditional immunosuppressive therapy, and adalimumab was favored by almost half of the experts. Questions addressing the management of individual situations, such as the treatment of macular edema and perioperative management, revealed considerable differences in therapeutic approaches. The results of this survey support the development of international guidelines for the management of JIAU.

[Evidence and consensus based treatment guidelines 2010 for juvenile idiopathic arthritis by the German Society of Paediatric Rheumatology].

PubMed

Dueckers, G; Guellac, N; Arbogast, M; Dannecker, G; Foeldvari, I; Frosch, M; Ganser, G; Heiligenhaus, A; Horneff, G; Illhardt, A; Krauspe, R; Markus, B; Michels, H; Schneider, M; Singendonk, W; Sitter, H; Spamer, M; Wagner, N; Niehues, T

2011-11-01

Treatment of Juvenile Idiopathic Arthritis (JIA) has improved quality of life in children and adolescents with JIA. Standardisation of care offers the chance to improve the quality of care of those patients. New studies have been published after completion of our last treatment guideline (2007). An updated consensus process is mandatory. A systematic literature analysis in PUBMED (key words: juvenile idiopathic (rheumatoid) arthritis, therapy; limits: humans, published in the last 3 years, all child 0-18 years, clinical trial) revealed 17 relevant studies. Studies relating to diagnosis of JIA, Uveitis, vaccination, transition were excluded. Representatives nominated by scientific societies and organisations were invited to consensus conferences which were hosted by a professional moderator. The following societies were invited: Berufsverband der Kinder- und Jugendärzte (BVKJ), Deutsche Gesellschaft für Kinder- und Jugendmedizin (DGKJ), Deutsche Gesellschaft für Rheumatologie (DGRh), Deutsche Ophthalmologische Gesellschaft (DOG), Deutsche Rheuma-Liga Bundesverband, Verein zur Förderung und Unterstützung rheumatologisch erkrankter Kinder und deren Eltern, Vereinigung für Kinderorthopädie, Zentraler Verband der Physiotherapeuten und Krankengymnasten (ZVK). Consensus conferences were each attended by more than 95% of the nominated representatives. Consensus statements were confirmed by nominal group technique and Delphi method. Updated consensus statements regarding drug therapy, symptomatic and surgical management of JIA were compiled and judged strictly by the criteria of Evidence-Based Medicine (EBM). © Georg Thieme Verlag KG Stuttgart · New York.

Correlation between compliance and brace treatment in juvenile and adolescent idiopathic scoliosis: SOSORT 2014 award winner

PubMed Central

2014-01-01

Background Over the last years, evidence has accumulated in support of bracing as an effective treatment option in patients with idiopathic scoliosis. Yet, little information is available on the impact of compliance on the outcome of conservative treatment in scoliotic subjects. The aim of the present study was to prospectively evaluate the association between compliance to brace treatment and the progression of scoliotic curve in patients with idiopathic adolescent (AIS) or juvenile scoliosis (JIS). Methods Among 1.424 patients treated for idiopathic scoliosis, 645 were eligible for inclusion criteria. Three outcomes were distinguished in agreement with the SRS criteria: curve correction, curve stabilization and curve progression. Brace wearing was assessed by one orthopaedic surgeon (LA) and scored on a standardized form. Compliance to treatment was categorized as complete (brace worn as prescribed), incomplete A (brace removed for 1 month), incomplete B (brace removed for 2 months), incomplete C (brace removed during school hours), and incomplete D (brace worn overnight only). Chi square test, T test or ANOVA and ANOVA for repeated measures tests were used as statistical tests. Results The results from our study showed that at follow-up the compliance was: Complete 61.1%; Incomplete A 5.2%; Incomplete B 10.7%; Incomplete C 14.2%; Incomplete D 8.8%. Curve correction was accomplished in 301/319 of Complete, 19/27 Incomplete A, 25/56 Incomplete B, 52/74 Incomplete C, 27/46 Incomplete D. Cobb mean value was 29.8 ± 7.5 SD at beginning and 17.1 ± 10.9 SD at follow-up. Both Cobb and Perdriolle degree amelioration was significantly higher in patients with complete compliance over all other groups, both in juvenile, both in adolescent scoliosis. In the intention-to-treat analysis, the rate of surgical treatment was 2.1% among patients with definite outcome and 12.1% among those with drop-out. Treatment compliance showed significant interactions with time

CXCR6-CXCL16 interaction in the pathogenesis of Juvenile Idiopathic Arthritis.

PubMed

Martini, Giorgia; Cabrelle, Anna; Calabrese, Fiorella; Carraro, Samuela; Scquizzato, Elisa; Teramo, Antonella; Facco, Monica; Zulian, Francesco; Agostini, Carlo

2008-11-01

In order to evaluate the role of CXCR6/CXCL16 in driving lymphocyte migration into inflamed joints of children with oligoarticular Juvenile Idiopathic Arthritis (JIA) we analysed CXCR6 expression and functional capability in lymphocytes from synovial fluid (SF) by flow cytometry, by real-time polymerase chain reaction (RT-PCR) and migration assays. Furthermore, CXCR6 and CXCL16 expression in synovial tissue (ST) was analysed by immunohistochemistry. T cells isolated from SF of patients with JIA expressed CXCR6 which was functionally active as shown by chemotactic assays. The same cells expressed CXCR3 and it exerted a migratory activity in response to CXCL10. CXCL16 and CXCR6 were intensively expressed on the synovium cells, respectively on macrophages, synoviocytes and endothelial cells and on lymphocytes, synoviocytes and endothelial cells. Taken together, these data suggest that CXCR6 and CXCR3 act coordinately with respective ligands and are involved in the pathophysiology of JIA-associated inflammatory processes.

Prevalence of vitamin D deficiency and insufficiency in children with osteopenia or osteoporosis referred to a pediatric metabolic bone clinic.

PubMed

Bowden, Sasigarn A; Robinson, Renee F; Carr, Roxane; Mahan, John D

2008-06-01

The purpose of this work was to determine the prevalence of vitamin D deficiency and insufficiency in children with osteopenia or osteoporosis and to evaluate the relationship between serum 25-hydroxyvitamin D levels and bone parameters, including bone mineral density. Serum 25-hydroxyvitamin D, 1,25 dihydroxyvitamin D, parathyroid hormone, and other bone markers, as well as bone mineral density, were obtained for 85 pediatric patients with primary osteoporosis (caused by osteogenesis imperfecta or juvenile idiopathic osteoporosis) and secondary osteopenia or osteoporosis caused by various underlying chronic illnesses. Pearson's correlation was used to assess the relationship between vitamin D levels and different bone parameters. Vitamin D insufficiency (defined as serum 25-hydroxyvitamin D <30 ng/mL) was observed in 80.0% of patients. Overt vitamin D deficiency (defined as serum 25-hydroxyvitamin D <10 ng/mL) was present in 3.5% of patients. Using a more recent definition for vitamin D deficiency in adults (defined as serum 25-hydroxyvitamin D <20 ng/mL), 21.1% of the patients had vitamin D deficiency. There was a significant inverse correlation between 25-hydroxyvitamin D and parathyroid hormone levels. There was a positive correlation between 1,25 dihydroxyvitamin D and parathyroid hormone, alkaline phosphatase, and urine markers for bone turnover. Vitamin D insufficiency was remarkably common in pediatric patients with primary and secondary osteopenia or osteoporosis. The inverse relationship between 25-hydroxyvitamin D and parathyroid hormone levels suggests a physiologic impact of insufficient vitamin D levels that may contribute to low bone mass or worsen the primary bone disease. We suggest that monitoring and supplementation of vitamin D should be a priority in the management of pediatric patients with osteopenia or osteoporosis.

Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans for juvenile idiopathic arthritis-associated and idiopathic chronic anterior uveitis.

PubMed

Angeles-Han, Sheila T; Lo, Mindy S; Henderson, Lauren A; Lerman, Melissa A; Abramson, Leslie; Cooper, Ashley M; Parsa, Miriam F; Zemel, Lawrence S; Ronis, Tova; Beukelman, Timothy; Cox, Erika; Sen, H Nida; Holland, Gary N; Brunner, Hermine I; Lasky, Andrew; Rabinovich, C Egla

2018-05-28

Systemic immunosuppressive treatment of pediatric chronic anterior uveitis (CAU), both juvenile idiopathic arthritis (JIA)-associated and idiopathic varies, making it difficult to identify best treatments. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans (CTPs) for CAU for the purpose of reducing practice variability and allowing future comparison of treatments by comparative effectiveness analysis techniques. A core group of pediatric rheumatologists, ophthalmologists with uveitis expertise, and a lay advisor comprised the CARRA uveitis workgroup who performed literature review on pharmacologic treatments, held teleconferences, and developed a case-based survey administered to the CARRA membership to delineate treatment practices. We utilized 3 face-to-face consensus meetings using nominal group technique to develop CTPs. The survey identified areas of treatment practice variability. We developed 2 CTPs for the treatment of CAU, case definitions, and monitoring parameters. The first CTP is directed at children naïve to steroid-sparing medication, and the second at children initiating biologic therapy with options for methotrexate, adalimumab and infliximab. We defined a core dataset and outcome measures with data collection at 3 and 6 months after therapy initiation. The CARRA membership voted acceptance of the CTPs with a >95% (N = 233) approval. Using consensus methodology, two standardized CTPs were developed for systemic immunosuppressive treatment of CAU. These CTPs are not meant as treatment guidelines, but are designed for further pragmatic research within the CARRA research network. Use of these CTPs in a prospective comparison effectiveness study should improve outcomes by identifying best practice options. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

[Uveitis associated with juvenile idiopathic arthritis : Optimization of immunomodulatory therapy].

PubMed

Heiligenhaus, A; Tappeiner, C; Walscheid, K; Heinz, C

2016-05-01

Uveitis associated with juvenile idiopathic arthritis (JIA-associated uveitis) is a vision-threatening disorder with a high complication rate. Besides early diagnosis within screening programs an adequate therapy is essential for improvement of the long-term prognosis. Corticosteroid therapy is often insufficient. In addition to conventional immunosuppression, immunomodulatory drugs, so-called biologicals, are novel highly effective treatment modalities. A systematic search of the literature was carried out for biologicals currently used in the treatment of JIA-associated uveitis. Review of current publications, summary of treatment guidelines and discussion of treatment options for therapy refractive patients. In accordance with the current recommendations tumor necrosis factor (TNF) inhibitors are administered if uveitis inactivity cannot be achieved with topical corticosteroids and in the next stage with immunosuppressants (methotrexate preferred). According to the currently available data adalimumab is then preferred. When the effectiveness of TNF inhibitors ceases during long-term administration and/or recurrences, other biological response modifiers are attractive treatment options (e. g. lymphocyte inhibitors or specific receptor antagonists). The TNF inhibitors are of major importance for the treatment of JIA-associated uveitis. Prospective studies and registries would be desirable in order to be able to compare the value of TNF inhibitors and other biologicals and for optimization of treatment recommendations.

A Patient-Specific Foot Model for the Estimate of Ankle Joint Forces in Patients with Juvenile Idiopathic Arthritis.

PubMed

Prinold, Joe A I; Mazzà, Claudia; Di Marco, Roberto; Hannah, Iain; Malattia, Clara; Magni-Manzoni, Silvia; Petrarca, Maurizio; Ronchetti, Anna B; Tanturri de Horatio, Laura; van Dijkhuizen, E H Pieter; Wesarg, Stefan; Viceconti, Marco

2016-01-01

Juvenile idiopathic arthritis (JIA) is the leading cause of childhood disability from a musculoskeletal disorder. It generally affects large joints such as the knee and the ankle, often causing structural damage. Different factors contribute to the damage onset, including altered joint loading and other mechanical factors, associated with pain and inflammation. The prediction of patients' joint loading can hence be a valuable tool in understanding the disease mechanisms involved in structural damage progression. A number of lower-limb musculoskeletal models have been proposed to analyse the hip and knee joints, but juvenile models of the foot are still lacking. This paper presents a modelling pipeline that allows the creation of juvenile patient-specific models starting from lower limb kinematics and foot and ankle MRI data. This pipeline has been applied to data from three children with JIA and the importance of patient-specific parameters and modelling assumptions has been tested in a sensitivity analysis focused on the variation of the joint reaction forces. This analysis highlighted the criticality of patient-specific definition of the ankle joint axes and location of the Achilles tendon insertions. Patient-specific detection of the Tibialis Anterior, Tibialis Posterior, and Peroneus Longus origins and insertions were also shown to be important.

Anthropometric and skeletal phenotype in men with idiopathic osteoporosis and their sons is consistent with deficient estrogen action during maturation.

PubMed

Lapauw, Bruno; Taes, Youri; Goemaere, Stefan; Toye, Kaatje; Zmierczak, Hans-Georg; Kaufman, Jean-Marc

2009-11-01

Pathophysiology of deficient bone mass acquisition in male idiopathic osteoporosis (IO) remains poorly understood. Our objective was to investigate volumetric and geometric parameters of the appendicular skeleton, biochemical markers, and anthropometrics in men with IO. Our cross-sectional study included 107 men diagnosed with idiopathic low bone mass, 23 of their adult sons, and 130 age-matched controls. Body composition and areal bone parameters (dual-energy x-ray absorptiometry) and volumetric and geometric parameters of radius and tibia (peripheral quantitative computed tomography) were assessed. Serum levels of testosterone, estradiol (E(2)), and SHBG, and bone turnover markers were measured using immunoassays. Free hormone fractions were calculated. Men with idiopathic low bone mass had lower weight (-9.6%), truncal height (-3.3%), and upper/lower body segment ratio (-2.7%; all P < 0.001) and presented at the radius and tibia lower trabecular (-19.0 and -23.6%, respectively; both P < 0.001) and cortical volumetric bone mineral density (vBMD) (-2.4 and -1.7%; both P < 0.001) and smaller cortical areas (-9.7 and -13.6%; both P < 0.001) and thicknesses (-13.5 and -14.5%, both P < 0.001) due to larger endosteal circumferences (+11.8 and +7.4%, both P < 0.001) than controls. Furthermore, (free) E(2) was lower and SHBG higher (both P < 0.01). Their sons had lower trabecular vBMD (-10.3%, P = 0.036) and a thinner cortex (-8.3%, P = 0.024) at the radius. Bone mass deficits in men with idiopathic low bone mass involve trabecular and cortical bone, resulting from lower vBMD and smaller cortical bone cross-sectional areas and thicknesses. A similar bone phenotype is present in at least part of their sons. The lower E(2), together with characteristics as lower upper/lower body segment ratio, larger endosteal circumferences and lower vBMD, may indicate an estrogen-related factor in the pathogenesis of male IO.

Clinical management algorithm of uveitis associated with juvenile idiopathic arthritis: interdisciplinary panel consensus.

PubMed

Bou, Rosa; Adán, Alfredo; Borrás, Fátima; Bravo, Beatriz; Calvo, Inmaculada; De Inocencio, Jaime; Díaz, Jesús; Escudero, Julia; Fonollosa, Alex; de Vicuña, Carmen García; Hernández, Victoria; Merino, Rosa; Peralta, Jesús; Rúa, María-Jesús; Tejada, Pilar; Antón, Jordi

2015-05-01

Uveitis associated with juvenile idiopathic arthritis (JIA) typically involves the anterior chamber segment, follows an indolent chronic course, and presents a high rate of uveitic complications and a worse outcome as compared to other aetiologies of uveitis. Disease assessment, treatment, and outcome measures have not been standardized. Collaboration between pediatric rheumatologists and ophthalmologists is critical for effective management and prevention of morbidity, impaired vision, and irreparable visual loss. Although the Standardization of Uveitis Nomenclature Working Group recommendations have been a great advance to help clinicians to improve consistency in grading and reporting data, difficulties arise at the time of deciding the best treatment approach in the individual patient in routine daily practice. For this reason, recommendations for a systematized control and treatment strategies according to clinical characteristics and disease severity in children with JIA-related uveitis were developed by a panel of experts with special interest in uveitis associated with JIA. A clinical management algorithm organized in a stepwise regimen is here presented.

VibroCV: a computer vision-based vibroarthrography platform with possible application to Juvenile Idiopathic Arthritis.

PubMed

Wiens, Andrew D; Prahalad, Sampath; Inan, Omer T

2016-08-01

Vibroarthrography, a method for interpreting the sounds emitted by a knee during movement, has been studied for several joint disorders since 1902. However, to our knowledge, the usefulness of this method for management of Juvenile Idiopathic Arthritis (JIA) has not been investigated. To study joint sounds as a possible new biomarker for pediatric cases of JIA we designed and built VibroCV, a platform to capture vibroarthrograms from four accelerometers; electromyograms (EMG) and inertial measurements from four wireless EMG modules; and joint angles from two Sony Eye cameras and six light-emitting diodes with commercially-available off-the-shelf parts and computer vision via OpenCV. This article explains the design of this turn-key platform in detail, and provides a sample recording captured from a pediatric subject.

The importance of an ophthalmologic examination in patients with juvenile idiopathic arthritis.

PubMed

Rodríguez-García, Alejandro

2015-01-01

Uveitis occurs within the first year of arthritis onset in 73% of patients with juvenile idiopathic arthritis (JIA) considered at risk. The intraocular inflammation is characterized by an insidious onset and a silent and chronic clinical course capable of producing significant visual loss due to complications such as: cataract formation, secondary glaucoma, maculopathy and optic neuropathy. The absence of initial signs and symptoms, along with a deficient ophthalmic monitoring produce a delay in diagnosis with serious consequences. It has been estimated that 47% of JIA patients at risk for developing uveitis are legally blind (20/200 or worse) at least in one eye at the time of their first visit to the ophthalmologist. To reduce ocular complications and improve their visual outcome, it is necessary that rheumatologists refer all patients recently diagnosed (within the first month) with JIA for an ophthalmic evaluation, and maintain periodical follow-up visits based on classification and risk category of the disease. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

Prior to extension, Transcriptomes of fibroblast-like Synoviocytes from extended and Polyarticular juvenile idiopathic arthritis are indistinguishable.

PubMed

Brescia, AnneMarie C; Simonds, Megan M; McCahan, Suzanne M; Sullivan, Kathleen E; Rose, Carlos D

2018-01-08

Our intent was to identify differences between the transcriptome of fibroblast-like synoviocytes (FLS) in oligoarticular juvenile idiopathic arthritis (JIA) before extension when compared to persistent subtype of JIA, when the two are clinically indistinguishable. Additionally, we sought to determine if differences between the transcriptomes of FLS from extended-to-be and polyarticular course JIA could be detected. Our hypothesis was that intrinsic differences in the transcriptome of the FLS from extended-to-be JIA would distinguish them from persistent oligoarticular JIA, before the course is clinically apparent. Global gene expression was defined in cultured FLS from 6 controls, 12 JIA with persistent course, 7 JIA prior to extension (extended-to-be), 4 JIA with extended course and 6 polyarticular onset, using Affymetrix Human GeneChips 133plus2.0. Bioconductor Linear Models for Microarray Analysis revealed 22 probesets with differential expression between persistent and extended-to-be FLS at 15% FDR, however only 2 probesets distinguished extended-to-be from extended and none distinguished extended-to-be and polyarticular at 15% FDR. Differences in extended and polyarticular gene expression profiles were not detected. Confirmation of select genes was done on the RNA level by RT-qPCR and on the protein level in synovial fluid by ELISA. The transcriptome of FLS from extended-to-be juvenile idiopathic arthritis is distinct from persistent course before a clinical distinction can be made. Additionally, the transcriptome of extended-to-be and polyarticular course, including those who have already extended, are indistinguishable. These gene expression data suggest that FLS already reflect a polyarticular behavior early in disease course, suggesting that extended-to-be may be "latent polyarticular" at onset. These differences can be used to develop early biomarkers of disease course, allowing for better-informed treatment decisions.

Contrast-enhanced MRI compared with the physical examination in the evaluation of disease activity in juvenile idiopathic arthritis.

PubMed

Hemke, Robert; Maas, Mario; van Veenendaal, Mira; Dolman, Koert M; van Rossum, Marion A J; van den Berg, J Merlijn; Kuijpers, Taco W

2014-02-01

To assess the value of magnetic resonance imaging (MRI) in discriminating between active and inactive juvenile idiopathic arthritis (JIA) patients and to compare physical examination outcomes with MRI outcomes in the assessment of disease status in JIA patients. Consecutive JIA patients with knee involvement were prospectively studied using an open-bore MRI. Imaging findings from 146 JIA patients were analysed (59.6% female; mean age, 12.9 years). Patients were classified as clinically active or inactive. MRI features were evaluated using the JAMRIS system, comprising validated scores for synovial hypertrophy, bone marrow oedema, cartilage lesions and bone erosions. Inter-reader reliability was good for all MRI features (intra-class correlation coefficient [ICC] = 0.87-0.94). No differences were found between the two groups regarding MRI scores of bone marrow oedema, cartilage lesions or bone erosions. Synovial hypertrophy scores differed significantly between groups (P = 0.016). Nonetheless, synovial hypertrophy was also present in 14 JIA patients (35.9%) with clinically inactive disease. Of JIA patients considered clinically active, 48.6% showed no signs of MRI-based synovitis. MRI can discriminate between clinically active and inactive JIA patients. However, physical examination is neither very sensitive nor specific in evaluating JIA disease activity compared with MRI. Subclinical synovitis was present in >35% of presumed clinically inactive patients. • MRI is sensitive for evaluating juvenile idiopathic arthritis (JIA) disease activity. • Contrast-enhanced MRI can distinguish clinically active and inactive JIA patients. • Subclinical synovitis is present in 35.9 % of presumed clinically inactive patients. • Physical examination is neither sensitive nor specific in evaluating JIA disease activity.

Glucocorticoids in the management of systemic juvenile idiopathic arthritis.

PubMed

Vannucci, Gaia; Cantarini, Luca; Giani, Teresa; Marrani, Edoardo; Moretti, Davide; Pagnini, Ilaria; Simonini, Gabriele; Cimaz, Rolando

2013-10-01

Glucocorticoids have been the mainstay of treatment for many years in systemic-onset juvenile idiopathic arthritis (sJIA), causing important side effects and some difficulties in the management of this disease. Until the introduction of biologic agents, oral glucocorticoids were used to control fever and other systemic features for several months or even years if systemic manifestations persisted. Nowadays, clinicians have valid alternatives that have revolutionized the natural history of sJIA. Biologic agents, such as the interleukin-1 inhibitors anakinra and the more recent canakinumab, or the interleukin-6 inhibitor tocilizumab, have improved the prognosis of this debilitating disease. Glucocorticoids still have to be considered at the onset of disease when a non-steroidal anti-inflammatory drug therapy fails or when there are life-threatening complications such as severe anemia or pericarditis, or macrophage activation syndrome. Local (intra-articular) triamcinolone hexacetonide is the treatment of choice for arthritis limited to one joint or a few joints in patients without systemic activity. To date, there is still great heterogeneity in the management of sJIA patients, but in recent years there have been attempts to design algorithms and treatment protocols for glucocorticoids, disease-modifying anti-rheumatic drugs, and biologic agents. This review provides an overview of the current knowledge of glucocorticoid therapy in sJIA, comments on recently published recommendations, and gives practical support to the clinician for management of this disease.

The impact of substance abuse on osteoporosis screening and risk of osteoporosis in women with psychotic disorders.

PubMed

Kelly, D L; Myers, C S; Abrams, M T; Feldman, S; Park, J; McMahon, R P; Shim, J-C

2011-04-01

Review of the 1-year prevalence of screening for osteoporosis and of osteoporosis or idiopathic fracture in Maryland Medicaid administrative records found that screening rates did not differ among women in the control population, women with psychosis, and women with major mood disorders, but were reduced compared to controls in women with substance use disorder, with or without psychosis. Prevalence of osteoporosis was increased compared to controls in women with major mood disorders or women over 55 dually diagnosed with psychosis and substance use disorder. Osteoporosis is a major public health concern. Substance abuse and psychosis may be risk factors, however, frequency of screening and disease risk in women with psychotic disorders and substance use disorder (SUD) remains unknown. This study examined rates (FY 2005) of osteoporosis screening and disease risk in Medicaid enrolled women aged 50 to 64 (N = 18,953). Four diagnostic groups were characterized: (1) psychosis, (2) SUD, (3) major mood disorder, and (4) controls. The interaction of psychosis and SUD on screening and disease prevalence of osteoporosis was tested. The prevalence of osteoporosis across the entire population was 6.7%. Four percent of those without an osteoporosis diagnosis received osteoporosis screening with no notable differences between psychosis and controls. Those with SUD, however, had a significant reduction in screening compared to controls (OR = 0.61, 95% CI = 0.40-0.91, p = 0.016). Women with a major mood disorder were more likely to have osteoporosis in their administrative record (OR = 1.32, 95% CI = 1.03-1.70, p = 0.028) compared to controls. Those who were dually diagnosed (SUD and psychosis) in the oldest ages (55-64 years) had a markedly higher prevalence of osteoporosis compared to controls (OR = 6.4 CI = 1.51-27.6, p = 0.012), whereas this interaction (SUD and psychosis) was not significant in the entire population over age 49. Osteoporosis screening in the Medicaid

Predictors of emotional functioning in youth after surgical correction of idiopathic scoliosis.

PubMed

Zebracki, Kathy; Thawrani, Dinesh; Oswald, Timothy S; Anadio, Jennifer M; Sturm, Peter F

2013-09-01

Patients with idiopathic scoliosis, although otherwise healthy, often have significant concerns about their self-image and appearance. In a group of juveniles and adolescents, this can impact adjustment in school, functioning in peer groups, and general sense of well-being. There are limited data to help physicians reliably and precisely identify those who are at higher risk of poor emotional adjustment even after spine deformity correction. The purpose of this study was to determine the predictors of emotional maladjustment in juvenile and adolescent patients after surgical correction of idiopathic scoliosis. A total of 233 juveniles, mean age 11.26 ± 1.02 (range, 8 to 12) years and 909 adolescents, mean age 14.91 ± 1.61 (range, 13 to 21) years, who underwent surgical correction for idiopathic scoliosis and who were participating in a prospective longitudinal multicenter database, were enrolled in the study. Participants completed the Scoliosis Research Society-22 (SRS-22) questionnaire before surgery and 2 years postoperatively. Radiographs were used to measure Cobb angle and surface measurements were used to determine decompensation and trunk shift. Adolescents reported poorer mental health preoperatively (P<0.05) and 2 years postoperatively (P<0.001) than juveniles; however, both groups reported improved mental health (P<0.001) and self-image (P<0.01) postoperatively. Mental health 2 years postoperatively was predicted by preoperative self-image (P<0.05), mental health (P<0.001), and main thoracic Cobb angle (P<0.05) in the juvenile group. Within the adolescent group, mental health 2 years postoperatively was predicted by preoperative mental health (P<0.001); self-image 2 years postoperatively was predicted by preoperative mental health (P<0.01) and self-image (P<0.001). Self-image and mental health are significantly improved after spine deformity correction in juveniles and adolescents with idiopathic scoliosis. However, consistent with normative development

[Treatment of osteoporosis with a delayed-action sodium fluoride preparation].

PubMed

Chlud, K

1977-01-01

40 patients with various types of osteoporosis, aged 17 to 76 years, were treated for 12 months with a new NaF-drug (Ossiplex-Retard, film tbl. 25 mg NaF plus 200 mg vitamine C). Daily dosage was 50-100 mg. Clinical symptoms, X-ray-status of the axial skeleton, alkaline serum phosphatase and the consumption of analgetics were used for assessment. 2/3 of the patients with presenile and steroid induced osteoporosis responded well to treatment, while those with osteogenesis imperfecta, idiopathic osteoporosis and plasmocytosis did not show clinical or radiological improvement. In senile osteoporosis, a physiological process of aging, should only be given NaF-treatment, if fractures without callus formation are present. Harmless side effects, (gastrointestinal intolerance in 15% and a painful tendoperiostosis syndrome in 12.5%), necessitated discontinuation of treatment in 3 cases. The NaF-drug with added vitamine C has less side effects than other NaF-containing compounds and should therefore be included as part of the therapeutical regimen of certain types of osteoporosis.

A national registry for juvenile dermatomyositis and other paediatric idiopathic inflammatory myopathies: 10 years' experience; the Juvenile Dermatomyositis National (UK and Ireland) Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies

PubMed Central

Martin, Neil; Krol, Petra; Smith, Sally; Murray, Kevin; Pilkington, Clarissa A.; Davidson, Joyce E.

2011-01-01

Objectives. The paediatric idiopathic inflammatory myopathies (IIMs) are a group of rare chronic inflammatory disorders of childhood, affecting muscle, skin and other organs. There is a severe lack of evidence base for current treatment protocols in juvenile myositis. The rarity of these conditions means that multicentre collaboration is vital to facilitate studies of pathogenesis, treatment and disease outcomes. We have established a national registry and repository for childhood IIM, which aims to improve knowledge, facilitate research and clinical trials, and ultimately to improve outcomes for these patients. Methods. A UK-wide network of centres and research group was established to contribute to the study. Standardized patient assessment, data collection forms and sample protocols were agreed. The Biobank includes collection of peripheral blood mononuclear cells, serum, genomic DNA and biopsy material. An independent steering committee was established to oversee the use of data/samples. Centre training was provided for patient assessment, data collection and entry. Results. Ten years after inception, the study has recruited 285 children, of which 258 have JDM or juvenile PM; 86% of the cases have contributed the biological samples. Serial sampling linked directly to the clinical database makes this a highly valuable resource. The study has been a platform for 20 sub-studies and attracted considerable funding support. Assessment of children with myositis in contributing centres has changed through participation in this study. Conclusions. This establishment of a multicentre registry and Biobank has facilitated research and contributed to progress in the management of a complex group of rare muscloskeletal conditions. PMID:20823094

Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.

PubMed

Lefèvre-Utile, Alain; Galeotti, Caroline; Koné-Paut, Isabelle

2014-05-01

Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Ghrelin levels in patients with juvenile idiopathic arthritis: relation to anti-tumor necrosis factor treatment and disease activity.

PubMed

Karagiozoglou-Lampoudi, Thomais; Trachana, Maria; Agakidis, Charalampos; Pratsidou-Gertsi, Polyxeni; Taparkou, Anna; Lampoudi, Sotiria; Kanakoudi-Tsakalidou, Florentia

2011-10-01

Studies in adults with rheumatoid arthritis reported low serum ghrelin that increased following anti-tumor necrosis factor (TNF) infusion. Data on juvenile idiopathic arthritis (JIA) are lacking. The aim of this pilot study was to explore serum ghrelin levels in patients with JIA and the possible association with anti-TNF treatment, disease activity, and nutritional status. Fifty-two patients with JIA (14/52 on anti-TNF treatment) were studied. Juvenile idiopathic arthritis was inactive in 3 of 14 anti-TNF-treated patients and in 11 of 38 non-anti-TNF-treated patients. The nutritional status, energy intake/requirements, appetite, and fasting serum ghrelin levels were assessed. Ghrelin control values were obtained from 50 individuals with minor illness matched for age, sex, and body mass index. Ghrelin levels in patients with JIA were significantly lower than in controls (P < .001, confidence interval [CI] = -101 to -331). Analysis according to anti-TNF treatment and disease activity showed that ghrelin levels were comparable to control values only in 3 patients with anti-TNF-induced remission. Ghrelin in non-anti-TNF-treated patients in remission was low. Multiple regression analysis showed that disease activity (P = .002, CI = -84.16 to -20.01) and anti-TNF treatment (P = .003, CI = -82.51 to -18.33) were significant independent predictors of ghrelin after adjusting for other potential confounders. Ghrelin did not correlate with nutritional status, energy balance, and appetite. Serum ghrelin is low in patients with JIA and is restored to values similar to those in controls following anti-TNF-induced remission. Our study provides evidence that TNF blockade is independently associated with serum ghrelin, which possibly contributes to anti-TNF-induced remission. These preliminary results could form the basis for future research. Copyright © 2011 Elsevier Inc. All rights reserved.

Handwriting difficulties in juvenile idiopathic arthritis: a pilot study.

PubMed

Haberfehlner, Helga; Visser, Bart; Daffertshofer, Andreas; van Rossum, Marion Aj; Roorda, Leo D; van der Leeden, Marike; Dekker, Joost; Hoeksma, Agnes F

2011-01-01

The aim of the present study was to describe handwriting difficulties of primary school children with juvenile idiopathic arthritis (JIA), and to investigate possible correlations with hand function and writing performance. In a cross-sectional approach, 15 children with JIA and reported handwriting difficulties were included together with 15 healthy matched controls. Impairments (signs of arthritis or tenosynovitis, reduced grip force and limited range of motion of the wrist (wrist-ROM)), activity limitations (reduced quality and speed of handwriting, pain during handwriting), and participation restrictions (perceived handwriting difficulties at school) were assessed and analysed. Although selected by the presence of handwriting difficulties, the majority of the JIA children (73%) had no active arthritis of the writing hand, and only minor hand impairments were found. Overall, the JIA children performed well during the short handwriting test, but the number of letters they wrote per minute decreased significantly during the 5-minute test, compared to the healthy controls. JIA patients had significantly higher pain scores on a 100 mm Visual Analogue Scale, compared to the healthy controls. The actual presence of arthritis, and limitation in grip force and wrist-ROM did not correlate with reported participation restrictions with regard to handwriting at school. The JIA children reported pain during handwriting, and inability to sustain handwriting for a longer period of time. The results of this pilot study show that JIA children with handwriting difficulties, experience their restrictions mainly through pain and the inability to sustain handwriting for a longer period of time. No correlations could be found with impairments.

Caregiver recall of treatment recommendations in juvenile idiopathic arthritis.

PubMed

De Civita, Mirella; Feldman, Debbie Ehrmann; Meshefedjian, Garbis A; Dobkin, Patricia L; Malleson, Pete; Duffy, Ciarán M

2007-03-15

Health care providers in juvenile idiopathic arthritis (JIA) might refer to caregivers' self-report of children's treatment-related behaviors to assist in clinical decisions. However, caregivers may believe that they are adhering to treatment even though they have a different understanding of recommendations than that intended by the medical team. We examined whether caregiver recall of children's JIA treatment matched actual recommendations at baseline and 3, 6, 9, and 12 months. A total of 235 primary caregivers were recruited from rheumatology clinics at 2 pediatric university-based teaching hospitals in Canada. Using the Parent Adherence Report Questionnaire, caregivers indicated whether their child was prescribed medications and/or exercises. Medical charts were reviewed to determine the prescribed treatment. Level of agreement between both sets of data was then examined. A total of 175 caregivers provided complete data. Mean age of the children was 10.2 years (68.6% girls); 44.6% were diagnosed with oligoarthritis. Kappa coefficients for medication represented substantial to almost perfect agreement beyond chance, with better levels of agreement at 12 months (kappa = 0.81, 95% confidence interval [95% CI] 0.68, 0.94) than at baseline (kappa = 0.61, 95% CI 0.47, 0.76). Kappa coefficients for exercise represented slight to moderate agreement beyond chance, with better agreement at 12 months (kappa = 0.44, 95% CI 0.24, 0.63) than at baseline (kappa = 0.27, 95% CI 0.08, 0.47). Weaker agreement for the exercise regimen raises concern that caregivers may pay less attention to exercise recommendations or that these recommendations may not be easily understood.

Effects of growth hormone treatment on growth in children with juvenile idiopathic arthritis.

PubMed

Simon, D; Bechtold, S

2009-11-01

Several therapeutic trials have been conducted over the past decade to evaluate the role of exogenous growth hormone (GH) as a means of correcting the growth deficiency seen in children with juvenile idiopathic arthritis (JIA). Early studies showed the benefit of GH treatment with respect to final height in patients with JIA. Of 13 patients receiving GH, 84% (11 patients) achieved a final height within their target range compared with only 22% (4 of 18 patients) of untreated patients. There are, however, factors that may limit the statural gains achieved with GH therapy including severe inflammation, severe statural deficiency at GH therapy initiation, long disease duration and delayed puberty. Data on the efficacy of GH replacement therapy in children with JIA and factors that influence the statural growth response will be reviewed. Results from therapeutic trials show that treatment with GH can decrease the statural deficit that occurs during the active phase of JIA, producing an adult height that is close to the genetically determined target height. Copyright 2009 S. Karger AG, Basel.

Concealed concern: fathers' experiences of having a child with juvenile idiopathic arthritis.

PubMed

Waite-Jones, J M; Madill, A

2008-01-01

Despite increased research into families of chronically ill children, more needs to be known about the father's experience. We address this issue through asking: 'What is it like to be the father of a child with Juvenile Idiopathic Arthritis?' (JIA). Four members of eight families with an adolescent diagnosed with JIA, including seven fathers, were interviewed and transcripts analysed using grounded theory. This study suggests that fathers of children with JIA experience several severe losses which are exacerbated through comparisons they make between their own situation and that of fathers of healthy children. In addition, the fathers faced several constraints which reduced their opportunities to communicate with their ill child through shared activities. Fathers appeared to conceal their distress by adopting strategies of denial and distraction however their adjustment was facilitated, to some extent, by social support. They could also develop greater acceptance of their situation over time as the care of their ill child became assimilated into family life and constraints upon their life gradually reduced through the increased maturity of their son or daughter with JIA. These findings have implications for healthcare professionals and voluntary organisations.

Novel PLS3 variants in X-linked osteoporosis: Exploring bone material properties.

PubMed

Balasubramanian, Meena; Fratzl-Zelman, Nadja; O'Sullivan, Rory; Bull, Mary; Fa Peel, Nicola; Pollitt, Rebecca C; Jones, Rebecca; Milne, Elizabeth; Smith, Kath; Roschger, Paul; Klaushofer, Klaus; Bishop, Nicholas J

2018-05-07

Idiopathic Juvenile Osteoporosis (IJO) refers to significantly lower than expected bone mass manifesting in childhood with no identifiable aetiology. IJO classically presents in early pubertal period with multiple fractures including metaphyseal and vertebral crush fractures, and low bone-mass. Here we describe two patients and provide information on their clinical phenotype, genotype and bone material analysis in one of the patients. Patient 1: 40-year old adult male diagnosed with IJO in childhood who re-presented with a hip fracture as an adult. Genetic analysis identified a pathogenic PLS3 hemizygous variant, c.1765del in exon 16. Patient 2: 15-year old boy with multiple vertebral fractures and bone biopsy findings suggestive of IJO who also has a diagnosis of autism spectrum disorder. Genetic analysis identified a maternally inherited PLS3 pathogenic c.1295T>A variant in exon 12. Analyses of the transiliac bone sample revealed severe reduction of trabecular volume and bone turnover indices and elevated bone matrix mineralisation. We propose that genetic testing for PLS3 should be undertaken in patients presenting with a current or previous history of IJO as this has implications for genetic counselling and cascade screening. The extensive evaluation of the transiliac biopsy sample of Patient 2 revealed a novel bone phenotype. This report includes a review of IJO and genetic causes of osteoporosis, and suggests that existing cases of IJO should be screened for PLS3. Through analysis of bone material properties in Patient 2, we can conclude that PLS3 does have a role in bone mineralisation. © 2018 Wiley Periodicals, Inc.

HLA B27 typing in 511 children with juvenile idiopathic arthritis from India.

PubMed

Srivastava, Rajni; Phatak, Sanat; Yadav, Akhilesh; Bajpai, Preeti; Aggarwal, Amita

2016-10-01

The enthesitis-related arthritis (ERA) category of juvenile idiopathic arthritis (JIA) is the most common category in India. HLA B27 has a high prevalence in ERA, and ILAR classification includes it in exclusion criteria for other categories, but due to its cost, it is not routinely done. We undertook this study to assess the prevalence of HLA B27 in ERA and other groups of juvenile arthritis in India. Consecutive patients of JIA ERA and select patients from other categories were recruited from a single tertiary care hospital over a span of 3 years. HLA B27 was tested using PCR. Five hundred and eleven children were studied: 312 had ERA, and 199 had other categories (29 oligoarthritis, 107 polyarthritis, 44 systemic onset JIA, 9 psoriatic arthritis and 10 undifferentiated). The prevalence of HLA B27 was highest in the ERA group (87 %) and correlated with the presence of sacroiliitis. Prevalence was 10.3 % in oligoarthritis, 16 % in polyarticular rheumatoid factor (RF)-positive arthritis, 26 % in RF-negative polyarticular arthritis, 66 % in psoriatic arthritis and 40 % in the unclassified and 0 % in systemic onset category. Twenty-seven children had a change in category of JIA as per ILAR owing to HLA B27 testing positive, most commonly in the RF-negative polyarthritis group. Only six of these had clinical features suggestive of Spondyloarthropathy. There is high prevalence of HLA B27 in ERA. Though HLA B27 testing helps in correct classification, a minority of these patients have features suggestive of spondyloarthropathy like back pain, enthesitis or sacroiliitis.

Circulating microRNA Signatures in Patients With Idiopathic and Postmenopausal Osteoporosis and Fragility Fractures.

PubMed

Kocijan, Roland; Muschitz, Christian; Geiger, Elisabeth; Skalicky, Susanna; Baierl, Andreas; Dormann, Rainer; Plachel, Fabian; Feichtinger, Xaver; Heimel, Patrick; Fahrleitner-Pammer, Astrid; Grillari, Johannes; Redl, Heinz; Resch, Heinrich; Hackl, Matthias

2016-11-01

Established bone turnover markers do not reflect fracture risk in idiopathic male and premenopausal osteoporosis and the role of microRNAs (miRNAs) in these patients is currently unclear. miRNAs are a class of small non-coding RNAs that regulate gene expression and bone tissue homeostasis. They are considered a new class of endocrine regulators with promising potential as biomarkers. Evaluation of circulating miRNA signatures in male and female subjects with idiopathic and postmenopausal osteoporotic low-traumatic fractures. This was a case-control study of cross-sectional design of 36 patients with prevalent low-traumatic fractures and 39 control subjects Main Outcome Measures: One hundred eighty-seven miRNAs were quantified in serum by qPCR, compared between groups and correlated with established bone turnover markers. Significant differences in serum levels of circulating miRNAs were identified in all three subgroups (46 in premenopausal, 52 in postmenopausal, 55 in male). A set of 19 miRNAs was consistently regulated in all three subgroups. Eight miRNAs [miR-152-3p, miR-30e-5p, miR-140-5p, miR-324-3p, miR-19b-3p, miR-335-5p, miR-19a-3p, miR-550a-3p] were excellent discriminators of patients with low-traumatic fractures, regardless of age and sex, with area under the curve values > 0.9. The 11 remaining miRNAs showed area under the curve values between 0.81 and 0.89. Correlation analysis identified significant correlations between miR-29b-3p and P1NP, and miR-365-5p and iPTH, TRAP5b, P1NP and Osteocalcin, as well as BMD L1-L4 and miR-19b-3p, miR-324-3p, miR-532-5p, and miR-93-5p. Specific serum miRNA profiles are strongly related to bone pathologies. Therefore miRNAs might be directly linked to bone tissue homeostasis. In particular, miR-29b-3p has previously been reported as regulator of osteogenic differentiation and could serve as a novel marker of bone turnover in osteoporotic patients as a member of a miRNA signature.

Consensus Treatment Plans for New-Onset Systemic Juvenile Idiopathic Arthritis

PubMed Central

DeWitt, Esi Morgan; Kimura, Yukiko; Beukelman, Timothy; Nigrovic, Peter A.; Onel, Karen; Prahalad, Sampath; Schneider, Rayfel; Stoll, Matthew L.; Angeles-Han, Sheila; Milojevic, Diana; Schikler, Kenneth N.; Vehe, Richard K.; Weiss, Jennifer E.; Weiss, Pamela; Ilowite, Norman T.; Wallace, Carol A.

2012-01-01

Objective There is wide variation in therapeutic approaches to systemic juvenile idiopathic arthritis (sJIA) among North American rheumatologists. Understanding the comparative effectiveness of the diverse therapeutic options available for treatment of sJIA can result in better health outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans and standardized assessment schedules for use in clinical practice to facilitate such studies. Methods Case-based surveys were administered to CARRA members to identify prevailing treatments for new-onset sJIA. A 2-day consensus conference in April 2010 employed modified nominal group technique to formulate preliminary treatment plans and determine important data elements for collection. Follow-up surveys were employed to refine the plans and assess clinical acceptability. Results The initial case-based survey identified significant variability among current treatment approaches for new onset sJIA, underscoring the utility of standardized plans to evaluate comparative effectiveness. We developed four consensus treatment plans for the first 9 months of therapy, as well as case definitions and clinical and laboratory monitoring schedules. The four treatment regimens included glucocorticoids only, or therapy with methotrexate, anakinra or tocilizumab, with or without glucocorticoids. This approach was approved by >78% of CARRA membership. Conclusion Four standardized treatment plans were developed for new-onset sJIA. Coupled with data collection at defined intervals, use of these treatment plans will create the opportunity to evaluate comparative effectiveness in an observational setting to optimize initial management of sJIA. PMID:22290637

Osteoporosis in young adults: pathophysiology, diagnosis, and management.

PubMed

Ferrari, S; Bianchi, M L; Eisman, J A; Foldes, A J; Adami, S; Wahl, D A; Stepan, J J; de Vernejoul, M-C; Kaufman, J-M

2012-12-01

Postmenopausal osteoporosis is mainly caused by increased bone remodeling resulting from estrogen deficiency. Indications for treatment are based on low areal bone mineral density (aBMD, T-score  ≤ -2.5), typical fragility fractures (spine or hip), and more recently, an elevated 10-year fracture probability (by FRAX®). In contrast, there is no clear definition of osteoporosis nor intervention thresholds in younger individuals. Low aBMD in a young adult may reflect a physiologically low peak bone mass, such as in lean but otherwise healthy persons, whereas fractures commonly occur with high-impact trauma, i.e., without bone fragility. Furthermore, low aBMD associated with vitamin D deficiency may be highly prevalent in some regions of the world. Nevertheless, true osteoporosis in the young can occur, which we define as a T-score below -2.5 at spine or hip in association with a chronic disease known to affect bone metabolism. In the absence of secondary causes, the presence of fragility fractures, such as in vertebrae, may point towards genetic or idiopathic osteoporosis. In turn, treatment of the underlying condition may improve bone mass as well. In rare cases, a bone-specific treatment may be indicated, although evidence is scarce for a true benefit on fracture risk. The International Osteoporosis Foundation (IOF) convened a working group to review pathophysiology, diagnosis, and management of osteoporosis in the young, excluding children and adolescents, and provide a screening strategy including laboratory exams for a systematic approach of this condition.

Juvenile Idiopathic Arthritis in the Era of International Cooperation.

PubMed

Uziel, Yosef

2017-01-30

Juvenile idiopathic arthritis (JIA) is the most common chronic disease of childhood. Improved understanding of its pathogenesis has led to international cooperation in clinical studies. Multicenter, international collaborations and research facilitate rapid enrollment of enough patients to enable a variety of studies, including those of epidemiology, diagnostic and classification criteria, genetic disease predisposition, pathogenesis, outcomes, and treatment protocols. In the last 20 years, the vision of the Pediatric Rheumatology International Trial Organization (PRINTO) has become a reality of worldwide collaboration in pediatric rheumatology research, including North American and European research groups. Major advances have been made in treating systemic JIA and its main complication, macrophage-activating syndrome (MAS). Single Hub and Access Point to Pediatric Rheumatology in Europe (SHARE) is a project of the European Society of Pediatric Rheumatology with the goal of improving clinical care. Based on evidence in the scientific literature, position papers regarding optimal clinical approaches and care have been published. Formal, validated assessment tools to evaluate response to treatment have been developed. Recommendations have been established to encourage international research collaborations, especially in light of major advances achieved in the genetics of pediatric rheumatologic diseases and the need to share biological samples among different countries and continents. Every participating country has disease information available for patients and families. Additionally, educational programs and updated syllabi for pediatric rheumatology have been written to promote similar, high-level academic training in different countries. These efforts have resulted in significant improvements in treatment and in patient prognosis. However, improved cooperation is needed to enhance research with biological and genetic samples. The Israeli Research Group for

Body experiences, emotional competence, and psychosocial functioning in juvenile idiopathic arthritis.

PubMed

Bomba, Monica; Meini, Antonella; Molinaro, Anna; Cattalini, Marco; Oggiano, Silvia; Fazzi, Elisa; Neri, Francesca; Plebani, Alessandro; Nacinovich, Renata

2013-08-01

We investigated self-image, psychological functioning, and quality of life in children and adolescents with juvenile idiopathic arthritis (JIA). Thirty-nine children with JIA were compared with 80 healthy peers. We first administered the Human Figure Drawing Test (HFDT) to all subjects; children also completed standardized questionnaires evaluating health-related quality of life (PEDSQL 4.0 Generic Core Scales) and the main aspects of psychological functioning: anxiety (SAFA-A) and depression (CDI). Parents were asked to complete the Child Behaviour Checklist (CBCL) and the PEDSQL 4.0. For each patient with JIA, clinical notes were gathered and a global disease assessment (visual analog scale--VAS) was performed. Compared to healthy peers, patients with JIA reported reduced maturity quotients at HFDT, more depressive traits, greater anxiety, and lower health-related quality of life. Among the subjects with JIA, HFDT revealed that adolescents had a greater impairment in all areas investigated. Furthermore, there was a significant correlation between the physical well-being rated by VAS and the perception of poorer quality of life in patients, mostly in the psychosocial domains. Children and adolescents with JIA exhibit emotional difficulties and a delay of psychological development leading to low self-esteem, a distorted self-image, more anxiety and depression traits, and a worse quality of life, when compared to healthy subjects.

Influence of etanercept on leptin and ghrelin secretion in children with juvenile idiopathic arthritis.

PubMed

Maciejewska-Paszek, Izabela; Grochowska-Niedworok, Elżbieta; Siwiec, Andrzej; Gruenpeter, Anna; Dul, Lechosław; Irzyniec, Tomasz

2017-04-01

Objective To assess possible changes in leptin and ghrelin secretion due to etanercept in juvenile idiopathic arthritis (JIA). Methods 50 patients with JIA and 16 age-matched controls were enrolled into this prospective, cross-sectional study. Serum leptin, total and acyl ghrelin were measured in addition to white blood cell (WBC) and lymphocyte counts. Results 25 patients received etanercept and 25 conventional therapies (including methotrexate) for JIA. There was no difference between treatment and control groups in leptin or ghrelin levels and no evidence of a relationship between leptin and ghrelin in patients with JIA. In all children with JIA there was a correlation between leptin and body mass index (BMI). However, compared with children in the conventional treatment group, children in the etanercept group showed a positive correlation between total ghrelin and BMI and those with a low BMI showed a negative correlation between acyl ghrelin and BMI. Conclusion No differences in leptin and ghrelin concentrations were found when patients with JIA and controls were compared or when patients who received etanercept were compared with those who received conventional treatment for JIA.

Recommendations for the Use of Ultrasound and Magnetic Resonance in Patients With Spondyloarthritis, Including Psoriatic Arthritis, and Patients With Juvenile Idiopathic Arthritis.

PubMed

Uson, Jacqueline; Loza, Estibaliz; Möller, Ingrid; Acebes, Carlos; Andreu, Jose Luis; Batlle, Enrique; Bueno, Ángel; Collado, Paz; Fernández-Gallardo, Juan Manuel; González, Carlos; Jiménez Palop, Mercedes; Lisbona, María Pilar; Macarrón, Pilar; Maymó, Joan; Narváez, Jose Antonio; Navarro-Compán, Victoria; Sanz, Jesús; Rosario, M Piedad; Vicente, Esther; Naredo, Esperanza

To develop evidence-based recommendations on the use of ultrasound (US) and magnetic resonance imaging in patients with spondyloarthritis, including psoriatic arthritis, and juvenile idiopathic arthritis. Recommendations were generated following a nominal group technique. A panel of experts (15 rheumatologists and 3 radiologists) was established in the first panel meeting to define the scope and purpose of the consensus document, as well as chapters, potential recommendations and systematic literature reviews (we used and updated those from previous EULAR documents). A first draft of recommendations and text was generated. Then, an electronic Delphi process (2 rounds) was carried out. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70% of participants voted≥7. The level of evidence and grade or recommendation was assessed using the Oxford Centre for Evidence Based Medicine levels of evidence. The full text was circulated and reviewed by the panel. The consensus was coordinated by an expert methodologist. A total of 12 recommendations were proposed for each disease. They include, along with explanations of the validity of US and magnetic resonance imaging regarding inflammation and damage detection, diagnosis, prediction (structural damage progression, flare, treatment response, etc.), monitoring and the use of US guided injections/biopsies. These recommendations will help clinicians use US and magnetic resonance imaging in patients with spondyloarthritis and juvenile idiopathic arthritis. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

Trial of Early Aggressive Therapy in Polyarticular Juvenile Idiopathic Arthritis

PubMed Central

Wallace, Carol A.; Giannini, Edward H.; Spalding, Steven J.; Hashkes, Philip J.; O’Neil, Kathleen M.; Zeft, Andrew S.; Szer, Ilona S.; Ringold, Sarah; Brunner, Hermine I.; Schanberg, Laura E.; Sundel, Robert P.; Milojevic, Diana; Punaro, Marilynn G.; Chira, Peter; Gottlieb, Beth S.; Higgins, Gloria C.; Ilowite, Norman T.; Kimura, Yukiko; Hamilton, Stephanie; Johnson, Anne; Huang, Bin; Lovell, Daniel J.

2011-01-01

OBJECTIVES To determine if aggressive treatment initiated early in the course of rheumatoid factor positive or negative polyarticular juvenile idiopathic arthritis (poly-JIA) can induce clinical inactive disease (CID) within 6 months. METHODS Between May 2007 and October 2010 a multi-center, prospective, double blind, randomized, placebo controlled trial of two aggressive treatments was conducted in 85 children aged 2 to 16 years with polyarticular JIA of less than 12 months duration. Patients received either methotrexate 0.5 mg/kg/wk SQ (40 mg max), etanercept 0.8 mg/kg/wk (50 mg max), prednisolone 0.5 mg/kg/d (60 mg max) tapered to 0 by 17 weeks (Arm 1), or methotrexate (same dose as Arm 1), etanercept placebo, and prednisolone placebo (Arm 2). The primary outcome was CID at 6 months. An exploratory phase determined the rate of clinical remission on medication (6 months of continuous CID) at 12 months. RESULTS By 6 months, 17 of 42 (40%) of patients in Arm 1 and 10 of 43 (23%) in Arm 2 had achieved CID (X2 = 2.91; p = 0.088). After 12 months, 9 patients in Arm 1 and 3 in Arm 2 achieved clinical remission on medication (p = 0.0534). There were no significant inter-arm differences in adverse events. CONCLUSIONS Although this study did not meet its primary endpoint, early aggressive therapy in this cohort of children with recent onset polyarticular JIA resulted in substantial proportions of patients in both arms achieving CID by 6 months and clinical remission on medication within 12 months of treatment. PMID:22183975

The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis.

PubMed

Lin, Yu-Tsan; Wang, Chen-Ti; Gershwin, M Eric; Chiang, Bor-Luen

2011-06-01

Juvenile idiopathic arthritis (JIA) has had a long and difficult problem with classification. It is clearly a heterogeneous and multi-factorial autoimmune disease but all too often the distinctions among subtypes were unclear. In fact, there is now increasing evidence of a distinct pathogenesis of oligo/polyarticular JIA compared to systemic JIA. Oligo/polyarticular JIA is an antigen-driven lymphocyte-mediated autoimmune disease with abnormality in the adaptive immune system. Cartilage-derived auto-antigens activate autoreactive T cells including Th1 and Th17 cells with production of pro-inflammatory cytokines IFN-γ and IL-17. On the other hand, the inhibition of regulatory T (Treg) cells including natural Foxp3(+) Treg and self-heat shock protein-induced Treg cells with decreased anti-inflammatory cytokine IL-10 results in the loss of immune tolerance. Imbalance between autoreactive Th1/Th17 and Treg cells leads to the failure of T cell tolerance to self-antigens, which contributes to the synovial inflammation of oligo/polyarticular JIA. By contrast, systemic JIA is an autoinflammatory disease with abnormality in the innate immune system. A loss of control of the alternative secretory pathway leading to aberrant activation of phagocytes including monocytes, macrophages and neutrophils seems to be involved in the release of pro-inflammatory cytokines IL-1, IL-6, IL-18 and pro-inflammatory S100-proteins, which contribute to the multisystem inflammation of systemic JIA. Markedly distinct pathogenesis of oligo/polyarticular JIA and systemic JIA implies that they might need different treatment strategies. Copyright © 2011 Elsevier B.V. All rights reserved.

A survey of national and multi-national registries and cohort studies in juvenile idiopathic arthritis: challenges and opportunities.

PubMed

Beukelman, Timothy; Anink, Janneke; Berntson, Lillemor; Duffy, Ciaran; Ellis, Justine A; Glerup, Mia; Guzman, Jaime; Horneff, Gerd; Kearsley-Fleet, Lianne; Klein, Ariane; Klotsche, Jens; Magnusson, Bo; Minden, Kirsten; Munro, Jane E; Niewerth, Martina; Nordal, Ellen; Ruperto, Nicolino; Santos, Maria Jose; Schanberg, Laura E; Thomson, Wendy; van Suijlekom-Smit, Lisette; Wulffraat, Nico; Hyrich, Kimme

2017-04-19

To characterize the existing national and multi-national registries and cohort studies in juvenile idiopathic arthritis (JIA) and identify differences as well as areas of potential future collaboration. We surveyed investigators from North America, Europe, and Australia about existing JIA cohort studies and registries. We excluded cross-sectional studies. We captured information about study design, duration, location, inclusion criteria, data elements and collection methods. We received survey results from 18 studies, including 11 national and 7 multi-national studies representing 37 countries in total. Study designs included inception cohorts, prevalent disease cohorts, and new treatment cohorts (several of which contribute to pharmacosurveillance activities). Despite numerous differences, the data elements collected across the studies was quite similar, with most studies collecting at least 5 of the 6 American College of Rheumatology core set variables and the data needed to calculate the 3-variable clinical juvenile disease activity score. Most studies were collecting medication initiation and discontinuation dates and were attempting to capture serious adverse events. There is a wide-range of large, ongoing JIA registries and cohort studies around the world. Our survey results indicate significant potential for future collaborative work using data from different studies and both combined and comparative analyses.

Infrapatellar bursitis in children with juvenile idiopathic arthritis: a case series.

PubMed

Alqanatish, Jubran T; Petty, Ross E; Houghton, Kristin M; Guzman, Jaime; Tucker, Lori B; Cabral, David A; Cairns, Robyn A

2011-02-01

Children with juvenile idiopathic arthritis (JIA) may infrequently present with localized anterior knee pain or swelling, in addition to generalize knee pain induced by JIA. We report five cases of deep infrapatellar bursitis in children with JIA. The clinical features, radiological findings, management, and outcome of five children with JIA and deep infrapatellar bursitis are reviewed. Three boys and two girls with a mean age of 9.8 years (range 6-14 years) were reviewed. Four children had persistent oligoarticular JIA, and one child had extended oligoarticular JIA. The presentation of deep infrapatellar bursitis was variable. In only one patient was the bursal swelling painful. Knee magnetic resonance imaging (MRI) was performed in four patients and demonstrated coexistent knee joint synovitis in three. Treatment included targeted corticosteroid injections into the deep infrapatellar bursa in two cases with complete resolution. One case was treated with corticosteroid injection by an outside health care provider with poor clinical response. Two cases are being treated with non-steroidal anti-inflammatory drugs and methotrexate. Deep infrapatellar bursitis can occur as an isolated finding or concurrently with knee joint synovitis in patients with JIA. Awareness of this entity is important because direct injection of the bursa may be needed for treatment, as the bursa does not communicate with the knee joint. Furthermore, when bursitis is suspected in JIA, MRI can be helpful to confirm the diagnosis, detect concurrent knee joint synovitis, and exclude other pathologies.

Emotion Regulation Predicts Pain and Functioning in Children With Juvenile Idiopathic Arthritis: An Electronic Diary Study

PubMed Central

Bromberg, Maggie H.; Anthony, Kelly K.; Gil, Karen M.; Franks, Lindsey; Schanberg, Laura E.

2012-01-01

Objectives This study utilized e-diaries to evaluate whether components of emotion regulation predict daily pain and function in children with juvenile idiopathic arthritis (JIA). Methods 43 children ages 8–17 years and their caregivers provided baseline reports of child emotion regulation. Children then completed thrice daily e-diary assessments of emotion, pain, and activity involvement for 28 days. E-diary ratings of negative and positive emotions were used to calculate emotion variability and to infer adaptive emotion modulation following periods of high or low emotion intensity. Hierarchical linear models were used to evaluate how emotion regulation related to pain and function. Results The attenuation of negative emotion following a period of high negative emotion predicted reduced pain; greater variability of negative emotion predicted higher pain and increased activity limitation. Indices of positive emotion regulation also significantly predicted pain. Conclusions Components of emotion regulation as captured by e-diaries predict important health outcomes in children with JIA. PMID:22037006

Intravenous immunoglobulin therapy leading to dramatic improvement in a patient with systemic juvenile idiopathic arthritis and severe pericarditis resistant to steroid pulse therapy.

PubMed

Aizawa-Yashiro, Tomomi; Oki, Eishin; Tsuruga, Kazushi; Nakahata, Tohru; Ito, Etsuro; Tanaka, Hiroshi

2012-05-01

A 7-year-old Japanese boy with a 4-month history of systemic juvenile idiopathic arthritis (s-JIA) experienced disease flare with spiking fever, exanthema and arthralgia. He then developed progressive dyspnea due to severe pericarditis, and proinflammatory hypercytokinemia was suspected. Methylprednisolone pulse therapy was ineffective and echocardiography showed massive pericardial effusion had persisted. Alternatively, subsequent intravenous immunoglobulin (IVIG) therapy resulted in dramatic resolution of the pericardial effusion, and his general condition significantly improved within a few days. This case report may lend further support the use of IVIG for selected patients with s-JIA and severe pericarditis.

Trial of early aggressive therapy in polyarticular juvenile idiopathic arthritis.

PubMed

Wallace, Carol A; Giannini, Edward H; Spalding, Steven J; Hashkes, Philip J; O'Neil, Kathleen M; Zeft, Andrew S; Szer, Ilona S; Ringold, Sarah; Brunner, Hermine I; Schanberg, Laura E; Sundel, Robert P; Milojevic, Diana; Punaro, Marilynn G; Chira, Peter; Gottlieb, Beth S; Higgins, Gloria C; Ilowite, Norman T; Kimura, Yukiko; Hamilton, Stephanie; Johnson, Anne; Huang, Bin; Lovell, Daniel J

2012-06-01

To determine whether aggressive treatment initiated early in the course of rheumatoid factor (RF)-positive or RF-negative polyarticular juvenile idiopathic arthritis (JIA) can induce clinical inactive disease within 6 months. Between May 2007 and October 2010, a multicenter, prospective, randomized, double-blind, placebo-controlled trial of 2 aggressive treatments was conducted in 85 children ages 2-16 years with polyarticular JIA of <12 months' duration. Patients received either methotrexate (MTX) 0.5 mg/kg/week (maximum 40 mg) subcutaneously, etanercept 0.8 mg/kg/week (maximum 50 mg), and prednisolone 0.5 mg/kg/day (maximum 60 mg) tapered to 0 by 17 weeks (arm 1), or MTX (same dosage as arm 1), etanercept placebo, and prednisolone placebo (arm 2). The primary outcome measure was clinical inactive disease at 6 months. An exploratory phase determined the rate of clinical remission on medication (6 months of continuous clinical inactive disease) at 12 months. By 6 months, clinical inactive disease had been achieved in 17 (40%) of 42 patients in arm 1 and 10 (23%) of 43 patients in arm 2 (χ(2) = 2.91, P = 0.088). After 12 months, clinical remission on medication was achieved in 9 patients in arm 1 and 3 patients in arm 2 (P = 0.053). There were no significant interarm differences in adverse events. Although this study did not meet its primary end point, early aggressive therapy in this cohort of children with recent-onset polyarticular JIA resulted in clinical inactive disease by 6 months and clinical remission on medication within 12 months of treatment in substantial proportions of patients in both arms. Copyright © 2012 by the American College of Rheumatology.

Imaging in juvenile idiopathic arthritis with a focus on ultrasonography.

PubMed

Laurell, Louise; Court-Payen, Michel; Boesen, Mikael; Fasth, Anders

2013-01-01

Early therapeutic intervention and use of new highly efficacious treatments have improved the outcome in many patients with juvenile idiopathic arthritis (JIA), but have also led to the need for more precise methods to evaluate disease activity. In adult rheumatology, numerous studies have established the importance of magnetic resonance imaging (MRI) and ultrasonography (US), and MRI is considered the reference standard. Nevertheless, due to differences in disease characteristics and the unique features of the growing skeleton, the findings obtained in adults are not directly applicable to children and adolescents. For paediatric patients, US offers specific advantages over MRI, because it is non-invasive, does not require sedation or general anesthesia (which facilitates repeated examinations for follow-up), is quickly accessible bedside, and is easy to combine with clinical assessment (interactivity). Agitation of the patient is rarely a problem, and hence young children can be seated on a parent's lap or play while being examined, and multiple locations can be assessed during a single session. Furthermore, modern high-frequency US transducers used by experienced US examiners can provide unsurpassed resolution of the superficial musculoskeletal structures in children. US is also the best available technique for imaging guidance of steroid injections. Unfortunately, there are still no validated MRI or US scoring systems for evaluating inflammatory and joint damage abnormalities in JIA, and few US studies have been conducted. Sonographic assessment of disease activity has, however, been proven to be more informative than clinical examination and is also readily available at points of care. This review summarises the literature on imaging in JIA, focusing on US and the important role this technique will play in JIA in the future.

Intra-articular corticosteroids versus intra-articular corticosteroids plus methotrexate in oligoarticular juvenile idiopathic arthritis: a multicentre, prospective, randomised, open-label trial.

PubMed

Ravelli, Angelo; Davì, Sergio; Bracciolini, Giulia; Pistorio, Angela; Consolaro, Alessandro; van Dijkhuizen, Evert Hendrik Pieter; Lattanzi, Bianca; Filocamo, Giovanni; Verazza, Sara; Gerloni, Valeria; Gattinara, Maurizio; Pontikaki, Irene; Insalaco, Antonella; De Benedetti, Fabrizio; Civino, Adele; Presta, Giuseppe; Breda, Luciana; Marzetti, Valentina; Pastore, Serena; Magni-Manzoni, Silvia; Maggio, Maria Cristina; Garofalo, Franco; Rigante, Donato; Gattorno, Marco; Malattia, Clara; Picco, Paolo; Viola, Stefania; Lanni, Stefano; Ruperto, Nicolino; Martini, Alberto

2017-03-04

Little evidence-based information is available to guide the treatment of oligoarticular juvenile idiopathic arthritis. We aimed to investigate whether oral methotrexate increases the efficacy of intra-articular corticosteroid therapy. We did this prospective, open-label, randomised trial at ten hospitals in Italy. Using a concealed computer-generated list, children younger than 18 years with oligoarticular-onset disease were randomly assigned (1:1) to intra-articular corticosteroids alone or in combination with oral methotrexate (15 mg/m 2 ; maximum 20 mg). Corticosteroids used were triamcinolone hexacetonide (shoulder, elbow, wrist, knee, and tibiotalar joints) or methylprednisolone acetate (ie, subtalar and tarsal joints). We did not mask patients or investigators to treatment assignments. Our primary outcome was the proportion of patients in the intention-to-treat population who had remission of arthritis in all injected joints at 12 months. This trial is registered with European Union Clinical Trials Register, EudraCT number 2008-006741-70. Between July 7, 2009, and March 31, 2013, we screened 226 participants and randomly assigned 102 to intra-articular corticosteroids alone and 105 to intra-articular corticosteroids plus methotrexate. 33 (32%) patients assigned to intra-articular corticosteroids alone and 39 (37%) assigned to intra-articular corticosteroids and methotrexate therapy had remission of arthritis in all injected joints (p=0·48). Adverse events were recorded for 20 (17%) patients who received methotrexate, which led to permanent treatment discontinuation in two patients (one due to increased liver transaminases and one due to gastrointestinal discomfort). No patient had a serious adverse event. Concomitant administration of methotrexate did not augment the effectiveness of intra-articular corticosteroid therapy. Future studies are needed to define the optimal therapeutic strategies for oligoarticular juvenile idiopathic arthritis. Italian Agency

Association of systemic-onset juvenile idiopathic arthritis and celiac disease - a case report.

PubMed

Michelin, Cintia Maria; Aikawa, Nadia Emi; Diniz, João Carlos; Jesus, Adriana Almeida; Koda, Yu Kar; Silva, Clovis Artur

2011-01-01

In a 28-year period, 5508 patients were followed at our Paediatric Rheumatology Division and 712 (13%) patients had juvenile idiopathic arthritis (JIA) (ILAR criteria). One (0.14%) of them had association with celiac disease (CD), with predominance of gastrointestinal manifestations and this case was described herein. A 10-years-old female patient was hospitalized with persistent fever, weight loss, asthenia, anorexia and an evanescent pink macular rash. After one week, she presented arthritis of left knee and ankle with duration of 75 days. The initial laboratory exams revealed anemia and elevation of inflammatory markers. Immunological tests were positive for anti-endomysial antibodies IgA and anti-thyroglobulin antibody. The diagnosis of systemic JIA was established and indomethacin (2.0 mg/kg/day) was started with improvement of arthritis. The patient evolved with vomiting, diarrhea and abdominal pain and upper gastrointestinal barium study showed areas of small bowel dilatation and thickening of folds, suggestive of malabsorption syndrome. Colonoscopy was normal and small intestinal biopsy was compatible with CD. We reported a case of a rare association of early diagnosis of systemic JIA occurring simultaneously with CD. This study reinforces the importance of taking into account the possible association of organ-specific autoimmune diseases during JIA course.

Seroprevalence of parvovirus B19 IgG in children affected by juvenile idiopathic arthritis

PubMed Central

Weissbrich, Benedikt; Süß-Fröhlich, Yvonne; Girschick, Hermann J

2007-01-01

Parvovirus (PV) B19 is the causative agent of the childhood disease erythema infectiosum. An association of PV B19 with chronic arthropathies, sometimes resembling rheumatoid arthritis or juvenile idiopathic arthritis (JIA), has repeatedly been described. Other studies, however, have failed to identify any such relationship. In order to study further whether there is a link between PV B19 and JIA, we determined the prevalence of PV B19 specific IgG antibodies in serum samples from children with rheumatoid diseases and compared it with the prevalence in unaffected children We reasoned that if there is an association between PV B19 and JIA, then the prevalence of PV B19 IgG in the children with JIA should be higher than in the control group. PV B19 IgG status was tested in 406 children with JIA and related diseases, and in 146 children constituting a control group. The percentage of PV B19 IgG positive children was not significantly elevated in the disease subgroups compared with age-matched control groups. In conclusion, our findings do not support the hypothesis that human parvovirus B19 is involved in the pathogenesis of JIA. PMID:17760961

Influence of etanercept on leptin and ghrelin secretion in children with juvenile idiopathic arthritis

PubMed Central

Maciejewska-Paszek, Izabela; Grochowska-Niedworok, Elżbieta; Siwiec, Andrzej; Gruenpeter, Anna; Dul, Lechosław

2017-01-01

Objective To assess possible changes in leptin and ghrelin secretion due to etanercept in juvenile idiopathic arthritis (JIA). Methods 50 patients with JIA and 16 age-matched controls were enrolled into this prospective, cross-sectional study. Serum leptin, total and acyl ghrelin were measured in addition to white blood cell (WBC) and lymphocyte counts. Results 25 patients received etanercept and 25 conventional therapies (including methotrexate) for JIA. There was no difference between treatment and control groups in leptin or ghrelin levels and no evidence of a relationship between leptin and ghrelin in patients with JIA. In all children with JIA there was a correlation between leptin and body mass index (BMI). However, compared with children in the conventional treatment group, children in the etanercept group showed a positive correlation between total ghrelin and BMI and those with a low BMI showed a negative correlation between acyl ghrelin and BMI. Conclusion No differences in leptin and ghrelin concentrations were found when patients with JIA and controls were compared or when patients who received etanercept were compared with those who received conventional treatment for JIA. PMID:28415953

Analysis of preexistent vertebral rotation in the normal infantile, juvenile, and adolescent spine.

PubMed

Janssen, Michiel M A; Kouwenhoven, Jan-Willem M; Schlösser, Tom P C; Viergever, Max A; Bartels, Lambertus W; Castelein, René M; Vincken, Koen L

2011-04-01

Vertebral rotation was systematically analyzed in the normal, nonscoliotic thoracic spine of children aged 0 to 16 years. Subgroups were created to match the infantile, juvenile, and adolescent age groups according to the criteria of the Scoliosis Research Society. To determine whether a distinct pattern of vertebral rotation in the transverse plane exists in the normal, nonscoliotic infantile, juvenile, and adolescent spine. We assume that, once the spine starts to deteriorate into a scoliotic deformity, it will follow a preexisting rotational pattern. Recently, we identified a rotational pattern in the normal nonscoliotic adult spine that corresponds to the most common curve types in adolescent idiopathic scoliosis. In infantile idiopathic scoliosis, curves are typically left sided and boys are affected more often than girls, whereas in adolescent idiopathic scoliosis, the thoracic curve is typically right sided and predominantly girls are affected. The present study is the first systematic analysis of vertebral rotation in the normal children's spine. Vertebral rotation in the transverse plane of T2-T12 was measured by using a semiautomatic method on 146 computed tomographic scans of children (0-16 years old) without clinical or radiologic evidence of spinal pathology. Scans were mainly made for reasons such as recurrent respiratory tract infections, malignancies, or immune disorders. Vertebral rotational patterns were analyzed in the infantile (0-3-year-old), juvenile (4-9-year-old), and adolescent (10-16-year-old) boys and girls. In the infantile spine, vertebrae T2-T6 were significantly rotated to the left (P < 0.001). In the juvenile spine, T4 was significantly rotated to the left. In the adolescent spine, T6-T12 were significantly rotated to the right (P ≤ 0.001). Rotation to the left was more pronounced in infantile boys than in the girls (P = 0.023). In juvenile and adolescent children, no statistical differences in rotation were found between the sexes

CLINICAL CHARACTERISTICS OF IDIOPATHIC FOVEOMACULAR RETINOSCHISIS.

PubMed

Maruko, Ichiro; Morizane, Yuki; Kimura, Shuhei; Shiode, Yusuke; Hosokawa, Mio; Sekiryu, Tetsuju; Iida, Tomohiro; Shiraga, Fumio

2016-08-01

To describe the clinical features of idiopathic foveomacular retinoschisis not in association with myopia, glaucoma, optic disk pit, or juvenile retinoschisis. Retrospective observational case series. Five eyes of five patients with idiopathic foveomacular retinoschisis were included. The patients were 2 men and 3 women (average age, 75.2 years; range, 71-78 years). The average spherical equivalent was +2.40 diopters (range, +0.88 to +5.75 diopters), and the average axial length was 22.0 mm (range, 21.1-23.1 mm). All patients had retinoschisis from the macula to the optic disk in the affected eye. No patients had retinoschisis in the fellow eye. The average best-corrected visual acuity was 20/44 (68 Early Treatment Diabetic Retinopathy Study letter score). Idiopathic foveomacular retinoschisis is not inherited or associated with myopia, vitreomacular traction syndrome, optic pit, or glaucoma but is associated with older age, unilaterality, hyperopia with short axial length, complete posterior vitreous detachment, and weak leakage from the optic disk on fluorescein angiography.

Screening of differentially expressed genes in male idiopathic osteoporosis via RNA sequencing.

PubMed

Feng, Li; Wang, Yan; Zhou, Jing; Tian, Baofang; Xia, Bo

2018-05-07

As a type of osteoporosis (OP), male idiopathic OP (MIO) is a bone disorder that occurs in young males and is a public health problem worldwide. However, the detailed pathogenesis of MIO remains to be elucidated. In the present study, blood samples of patients with MIO, senile OP, postmenopausal OP and normal controls (NCs) were obtained for RNA sequencing. Compared with the NC group, differentially expressed genes (DEGs) in the three types of OP were identified. DEGs that were common among the three types of OP and the DEGs that were unique to patients with MIO were determined. Gene ontology enrichment analysis and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analyses were conducted. MIO‑specific and OP‑specific protein‑protein interaction (PPI) networks were constructed. Compared with NCs, a total of 519, 368 and 1,472 DEGs were identified in samples from MIO, senile OP and postmenopausal OP, respectively. Tetraspanin 5 (TSPAN5) and α‑synuclein (SNCA) were unique DEGs in MIO that were not identified in the other two types of OP compared with NCs. Furthermore, the expression of carbonic anhydrase 1 (CA1) and S100 calcium‑binding protein P (S100P) in MIO was significantly different compared with senile OP, postmenopausal OP and NC samples. 'MAPK signaling pathway', 'type I diabetes mellitus' and 'hematopoietic cell lineage' were among significantly enriched pathways of DEGs in MIO. SNCA and CDC‑like kinase 1 were the hub genes in the MIO‑specific PPI network. In conclusion, the mitogen‑activated protein kinase signaling and type I diabetes mellitus pathways may be involved in bone formation; SNCA and TSPAN5 may be associated with bone resorption. These two pathways and two genes may serve a role in MIO. CA1 and S100P may regulate the process of MIO by modulation of calcification and dysregulation of calcium binding. These findings may have provided an experimental basis for elucidating the underlying mechanisms and developing

Allogeneic hematopoietic stem cell transplantation for severe, refractory juvenile idiopathic arthritis

PubMed Central

Ladomenou, Fani; Carpenter, Ben; Chandra, Sharat; Sedlacek, Petr; Formankova, Renata; Grandage, Vicky; Friswell, Mark; Cant, Andrew J.; Nademi, Zohreh; Slatter, Mary A.; Gennery, Andrew R.; Hambleton, Sophie; Flood, Terence J.; Lucchini, Giovanna; Chiesa, Robert; Rao, Kanchan; Amrolia, Persis J.; Brogan, Paul; Wedderburn, Lucy R.; Glanville, Julie M.; Hough, Rachael; Marsh, Rebecca; Abinun, Mario; Veys, Paul

2018-01-01

Patients with juvenile idiopathic arthritis (JIA) can experience a severe disease course, with progressive destructive polyarthritis refractory to conventional therapy with disease-modifying antirheumatic drugs including biologics, as well as life-threatening complications including macrophage activation syndrome (MAS). Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a potentially curative immunomodulatory strategy for patients with such refractory disease. We treated 16 patients in 5 transplant centers between 2007 and 2016: 11 children with systemic JIA and 5 with rheumatoid factor–negative polyarticular JIA; all were either refractory to standard therapy, had developed secondary hemophagocytic lymphohistiocytosis/MAS poorly responsive to treatment, or had failed autologous HSCT. All children received reduced toxicity fludarabine-based conditioning regimens and serotherapy with alemtuzumab. Fourteen of 16 patients are alive with a median follow-up of 29 months (range, 2.8-96 months). All patients had hematological recovery. Three patients had grade II-IV acute graft-versus-host disease. The incidence of viral infections after HSCT was high, likely due to the use of alemtuzumab in already heavily immunosuppressed patients. All patients had significant improvement of arthritis, resolution of MAS, and improved quality of life early following allo-HSCT; most importantly, 11 children achieved complete drug-free remission at the last follow-up. Allo-HSCT using alemtuzumab and reduced toxicity conditioning is a promising therapeutic option for patients with JIA refractory to conventional therapy and/or complicated by MAS. Long-term follow-up is required to ascertain whether disease control following HSCT continues indefinitely. PMID:29618462

Clinical responsiveness of self-report functional assessment measures for children with juvenile idiopathic arthritis undergoing intraarticular corticosteroid injections.

PubMed

Brown, G Ted; Wright, F Virginia; Lang, Bianca A; Birdi, Nina; Oen, Kim; Stephens, Derek; McComas, Joan; Feldman, Brian M

2005-12-15

The Childhood Health Assessment Questionnaire (CHAQ), Juvenile Arthritis Functional Assessment Report (JAFAR), and Juvenile Arthritis Functional Status Index (JASI) are widely used functional measures for juvenile idiopathic arthritis (JIA) that differ in content, format, and completion time. We compared the responsiveness and child-parent agreement of the JAFAR, CHAQ, and JASI in a prospective, multicenter study. Children and adolescents from 5 rheumatology centers were enrolled. Subjects were about to undergo therapy (intraarticular corticosteroid injections [IAS] and methotrexate or hip surgery (MTX/hip]) expected to produce a functional improvement. All subjects were studied before the intervention and at 6 weeks and 6 months posttreatment. At each study visit, the 3 measures were administered in randomized, balanced order to both parents and children. A total of 92 subjects (mean age 12.8 years) were enrolled in the study, 74 of which were in the IAS group. The responsiveness of all 3 measures was moderate to strong. The standardized response mean at 6 weeks for the IAS group on the JAFAR, CHAQ, and JASI was 0.41 (95% confidence interval [95% CI] 0.18, 0.64), 0.70 (95% CI 0.47, 0.93), and 0.36 (95% CI 0.13, 0.59), respectively. The CHAQ was somewhat more responsive to change at 6 weeks (IAS group: relative efficiency 0.34 [JAFAR], 0.27 [JASI]), but less responsive at 6 months (MTX/hip group: relative efficiency 5.1 [JAFAR], 3.9 [JASI]). All 3 questionnaires showed acceptable parent-child agreement, and overall, there were few differences between the 3 questionnaires. The functional outcome measures currently used for JIA are all adequately responsive for use in trials or in the clinic setting. The choice of which measure to use should therefore be based on the time available for completion, the intended clinical/research use, and the depth of content required.

Substance use and sexual function in juvenile idiopathic arthritis.

PubMed

van Weelden, Marlon; Lourenço, Benito; Viola, Gabriela R; Aikawa, Nadia E; Queiroz, Lígia B; Silva, Clovis A

2016-02-13

to evaluate alcohol/tobacco/illicit drug use and sexual function in adolescent juvenile idiopathic arthritis (JIA) and healthy controls. 174 adolescents with pediatric rheumatic diseases were selected. A cross-sectional study with 54 JIA patients and 35 controls included demographic/anthropometric data and puberty markers assessments, physician-conducted CRAFFT (car/relax/alone/forget/friends/trouble) screen tool for substance abuse/dependence high risk and a questionnaire that evaluated sexual function, bullying and alcohol/tobacco/illicit drug use. Clinical/laboratorial data and treatment were also assessed in JIA. The median current age was similar between JIA patients and controls [15(10-19) vs. 15(12-18)years, p=0.506]. Frequencies of alcohol/tobacco/illicit drug use were high and similar in both JIA and controls (43% vs. 46%, p=0.829). However, age at alcohol onset was significantly higher in those with JIA [15(11-18) vs. 14(7-18)years, p=0.032], particularly in poliarticular onset (p=0.040). High risk for substance abuse/dependence (CRAFFT score≥2) was found in both groups (13% vs. 15%, p=1.000), likewise bullying (p=0.088). Further analysis of JIA patients regarding alcohol/tobacco/illicit drug use showed that the median current age [17(14-19) vs. 13(10-19)years, p<0.001] and education years [11(6-13) vs. 7(3-12)years, p<0.001] were significant higher in those that used substances. Sexual activity was significantly higher in the former group (48% vs. 7%, p<0.001). A positive correlation was evidenced between CRAFFT score and current age in JIA patients (p=0.032, r=+0.296). A high risk for substance abuse/dependence was observed in both JIA and controls. JIA substance users were more likely to have sexual intercourse. Therefore, routine screening is suggested in all visits of JIA adolescents. Copyright © 2016 Elsevier Editora Ltda. All rights reserved.

Substance use and sexual function in juvenile idiopathic arthritis.

PubMed

van Weelden, Marlon; Lourenço, Benito; Viola, Gabriela R; Aikawa, Nadia E; Queiroz, Lígia B; Silva, Clovis A

2016-01-01

To evaluate alcohol/tobacco/illicit drug use and sexual function in adolescent juvenile idiopathic arthritis (JIA) and healthy controls. 174 adolescents with pediatric rheumatic diseases were selected. A cross-sectional study with 54 JIA patients and 35 controls included demographic/anthropometric data and puberty markers assessments, physician-conducted CRAFFT (car/relax/alone/forget/friends/trouble) screen tool for substance abuse/dependence high risk and a questionnaire that evaluated sexual function, bullying and alcohol/tobacco/illicit drug use. Clinical/laboratorial data and treatment were also assessed in JIA. The median current age was similar between JIA patients and controls [15(10-19) vs. 15(12-18) years, p=0.506]. Frequencies of alcohol/tobacco/illicit drug use were high and similar in both JIA and controls (43% vs. 46%, p=0.829). However, age at alcohol onset was significantly higher in those with JIA [15(11-18) vs. 14(7-18) years, p=0.032], particularly in polyarticular onset (p=0.040). High risk for substance abuse/dependence (CRAFFT score≥2) was found in both groups (13% vs. 15%, p=1.000), likewise bullying (p=0.088). Further analysis of JIA patients regarding alcohol/tobacco/illicit drug use showed that the median current age [17(14-19) vs. 13(10-19)years, p<0.001] and education years [11(6-13) vs. 7(3-12)years, p<0.001] were significant higher in those that used substances. Sexual activity was significantly higher in the former group (48% vs. 7%, p<0.001). A positive correlation was evidenced between CRAFFT score and current age in JIA patients (p=0.032, r=+0.296). A high risk for substance abuse/dependence was observed in both JIA and controls. JIA substance users were more likely to have sexual intercourse. Therefore, routine screening is suggested in all visits of JIA adolescents. Copyright © 2016 Elsevier Editora Ltda. All rights reserved.

Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies.

PubMed

Huber, Adam M; Feldman, Brian M; Rennebohm, Robert M; Hicks, Jeanne E; Lindsley, Carol B; Perez, Maria D; Zemel, Lawrence S; Wallace, Carol A; Ballinger, Susan H; Passo, Murray H; Reed, Ann M; Summers, Ronald M; White, Patience H; Katona, Ildy M; Miller, Frederick W; Lachenbruch, Peter A; Rider, Lisa G

2004-05-01

To examine the measurement characteristics of the Childhood Myositis Assessment Scale (CMAS) in children with juvenile idiopathic inflammatory myopathy (juvenile IIM), and to obtain preliminary data on the clinical significance of CMAS scores. One hundred eight children with juvenile IIM were evaluated on 2 occasions, 7-9 months apart, using various measures of physical function, strength, and disease activity. Interrater reliability, construct validity, and responsiveness of the CMAS were examined. The minimum clinically important difference (MID) and CMAS scores corresponding to various degrees of physical disability were estimated. The intraclass correlation coefficient for 26 patients assessed by 2 examiners was 0.89, indicating very good interrater reliability. The CMAS score correlated highly with the Childhood Health Assessment Questionnaire (C-HAQ) score and with findings on manual muscle testing (MMT) (r(s) = -0.73 and 0.73, respectively) and moderately with physician-assessed global disease activity and skin activity, parent-assessed global disease severity, and muscle magnetic resonance imaging (r(s) = -0.44 to -0.61), thereby demonstrating good construct validity. The standardized response mean was 0.81 (95% confidence interval 0.53, 1.09) in patients with at least 0.8 cm improvement on a 10-cm visual analog scale for physician-assessed global disease activity, indicating strong responsiveness. In bivariate regression models predicting physician-assessed global disease activity, MMT remained significant in models containing the CMAS (P = 0.03) while the C-HAQ did not (P = 0.4). Estimates of the MID ranged from 1.5 to 3.0 points on a 0-52-point scale. CMAS scores corresponding to no, mild, mild-to-moderate, and moderate physical disability, respectively, were 48, 45, 39, and 30. The CMAS exhibits good reliability, construct validity, and responsiveness, and is therefore a valid instrument for the assessment of physical function, muscle strength, and

Osteoporosis in unstable adult scoliosis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Velis, K.P.; Healey, J.H.; Schneider, R.

1988-12-01

New noninvasive techniques as well as conventional methods were used to evaluate skeletal mass in the following three populations of adult white women as follows: (1) 79 subjects with preexisting idiopathic scoliosis designated as unstable (US) because of the associated presence in the lumbar spine of lateral spondylolisthesis with segmental instability; (2) 67 subjects with preexisting idiopathic scoliosis without lateral spondylolisthesis designated as stable (SS); and (3) 248 age-matched nonscoliotic controls. Ages in all three groups were categorized into premenopausal (25-44 years), perimenopausal (45-54 years), and postmenopausal (55-84 years). The results showed higher scoliosis morbidity in the US compared tomore » the SS populations. The prevalence and severity of osteoporosis were markedly increased in US versus SS populations. Femoral neck density determined by dual-photon absorptiometry techniques averaged 26% to 48% lower in all age categories of US patients compared to controls. These changes were found in the youngest age groups, indicating reductions in bone mineral content earlier in the adult life of white women with a specific type of high-morbidity US characterized by the marker of lateral spondylolisthesis.« less

Juvenile idiopathic arthritis and athletic participation: are we adequately preparing for sports integration?

PubMed

Taxter, Alysha; Foss, Kim Barber; Melson, Paula; Ford, Kevin R; Shaffer, Michael; Myer, Gregory D

2012-09-01

Children with juvenile idiopathic arthritis (JIA) now have well-controlled disease due to improved therapies and management strategies. Children with JIA are more active than in the past and often participate in dynamic, high-loading sports. Standard measures of disease control include examination findings, laboratory values, and patient-directed surveys. However, these standards do not address the subtle deficits in biomechanics and neuromuscular control, which could place affected joints at higher risk for injury. Currently, there are limited evidence-based guidelines to structure conditioning recommendations as to the fitness and mechanics needed to provide safe integration into sports in this population; therefore, tools that objectively measure function with high accuracy and precision may be warranted. Previous work using 3-dimensional motion analysis demonstrated usefulness in guiding physical therapy treatment to correct these deficits. The use of a multidisciplinary team, including physical therapy, rheumatology, and sports medicine, is crucial for preparing these children to return to play. We suggest that the child transition into a sport preparatory-conditioning program to address any underlying deficits. A pediatric exercise specialist who is sensitive to the needs of this population can work with a physical therapist to then appropriately integrate the child safely into sport. Encouraging an active lifestyle is vital to the management of JIA and does not worsen the symptoms associated with childhood arthritis.

Methotrexate is an effective treatment for chronic uveitis associated with juvenile idiopathic arthritis.

PubMed

Foeldvari, Ivan; Wierk, Angela

2005-02-01

To assess the effectiveness of methotrexate (MTX) in the treatment of juvenile idiopathic arthritis (JIA) associated uveitis, which is still one of the most common causes of visual impairment. A retrospective chart review of patients with the diagnosis of uveitis associated with JIA between July 1, 2002, and December 31, 2002. Four hundred sixty-seven patients with JIA were followed. Thirty-eight had uveitis: 31 associated with oligoarticular JIA and 7 with psoriatic JIA. Twenty-five of the 38 patients received MTX; in 23 patients uveitis was the indication for MTX therapy. In the MTX treated group 46/50 eyes had uveitis, the mean (range) age at onset of uveitis was 7.82 years (1.8-15.8), and the mean age at onset of arthritis was 7.25 years (1.25-15.7). MTX treatment was started an average of 11.4 months (0-72) after the onset of uveitis. The mean MTX dose was 15.6 mg/m2. Remission occurred after 4.25 months (1-12). Mean duration of remission was 10.3 months (3-27). The total duration of MTX therapy was 661 months and patients were in remission for 417/661 months. In 6 patients MTX was discontinued after 12 months of remission. Four patients were still in remission after 7.5 months (1-14). MTX seems to be an effective therapy for JIA associated uveitis.

Azathioprine as a treatment option for uveitis in patients with juvenile idiopathic arthritis.

PubMed

Goebel, J C; Roesel, M; Heinz, C; Michels, H; Ganser, G; Heiligenhaus, A

2011-02-01

To investigate the therapeutic value of azathioprine as monotherapy or combined with other immunosuppressive drugs for uveitis in patients with juvenile idiopathic arthritis (JIA). A retrospective multicentre study including 41 children with JIA (28 (68.2%) female) with unilateral or bilateral (n=28) chronic anterior uveitis. Azathioprine was used to treat uveitis that was active in patients receiving topical or systemic corticosteroids, methotrexate or other immunosuppressive drugs. The primary end point was assessment of uveitis inactivity. Secondary end points comprised dose sparing of topical steroids and systemic corticosteroids, and immunosuppression. At 1 year, uveitis inactivity was achieved in 13/17 (76.5%) patients by using azathioprine as systemic monotherapy and in 5/9 (56.6%) as combination therapy. During the entire azathioprine treatment period (mean 26 months), inactivity was obtained in 16/26 patients (61.5%) with monotherapy and in 10/15 (66.7%) when combined with other immunosuppressives (p=1.0). With azathioprine, dosages of systemic immunosuppression and steroids could be reduced by ≥ 50% (n=12) or topical steroids reduced to ≤ 2 drops/eye/day in six patients. In three patients (7.3%), azathioprine was discontinued because of nausea and stomach pain. Conclusions Azathioprine may be reconsidered in the stepladder approach for the treatment of JIA-associated uveitis. The addition of azathioprine may also be beneficial for patients not responding properly to methotrexate.

Influence of past breast feeding on pattern and severity of presentation of juvenile idiopathic arthritis.

PubMed

Hyrich, Kimme L; Baildam, Eileen; Pickford, Hannah; Chieng, Alice; Davidson, Joyce E; Foster, Helen; Gardner-Medwin, Janet; Wedderburn, Lucy R; Thomson, Wendy

2016-04-01

This analysis aimed to study the influence of breast feeding on the pattern and severity of juvenile idiopathic arthritis (JIA) at presentation. The association between ever versus never breast feeding and disease severity at onset was compared in 923 children with JIA recruited to the UK Childhood Arthritis Prospective Study at first presentation to rheumatology. Fifty six per cent of children were ever breast fed (median 3.7 months). Breastfed children reported a lower median age at onset, a lower Childhood Health Assessment Questionnaire (CHAQ), a measure of disease severity, lower parent general evaluation scores and lower pain at presentation. There was a trend towards a higher proportion of breastfed children with rheumatoid factor-negative polyarthritis, but lesser enthesitis-related and psoriatic arthritis. There was a statistically significant inverse association between breast feeding and high CHAQ, even after adjusting for differences in socioeconomic status (adjusted OR 0.61 (95% CI 0.39 to 0.95)). Further work to understand the reasons behind these associations is required. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Juvenile idiopathic arthritis-and now?: a systematic literature review of changes in craniofacial morphology.

PubMed

von Bremen, Julia; Ruf, Sabine

2012-08-01

To conduct a systematic literature review on the impact of juvenile idiopathic arthritis (JIA) on craniofacial morphology. Several electronic databases (PubMed, Medpilot, Web of Science, DIMDI) were systematically searched for studies that were published up to and including May 2011. In addition, a manual search of the orthodontic and rheumatologic literature was conducted, and reference lists of the selected articles were checked for relevant publications. The identified articles were independently assessed by two investigators and selected according to a three-step process (title/abstract/full text). After completion of the selection procedure, 19 articles were identified possessing great heterogeneity. In most of them, no differentiated analysis of the various JIA subtypes was performed, and type-specific analyses according to mandibular joint effects were seldom. Additional factors such as patient age, disease duration, medication, previous orthodontic treatment as well as the inclusion of a control group were also highly inhomogeneous, which made a meta-analysis of the data impossible. Nevertheless, it appears as if JIA patients tend to develop a hyperdivergent vertical jaw base relationship and a skeletal Class II pattern. Due to the heterogeneous patient samples, it is currently not possible to draw a differentiated conclusion on the influence of various types of JIA on craniofacial morphology.

Application of Rasch analysis to the parent adherence report questionnaire in juvenile idiopathic arthritis.

PubMed

Toupin April, Karine; Higgins, Johanne; Ehrmann Feldman, Debbie

2016-07-28

Adherence to treatment in children with juvenile idiopathic arthritis (JIA) is associated with better outcomes. Assessing patient adherence in JIA, as well as attitudes and beliefs about prescribed treatments, is important for the clinician in order to optimize patient management. The objective of the current study was to evaluate the psychometric properties of the Parent (proxy-report) Adherence Report Questionnaires (PARQ), which assesses beliefs and behaviors related to adherence to treatments prescribed for JIA. A Rasch analysis was conducted on data collected with parents of children with JIA from two studies in which the PARQ was used as a measure of adherence. The PARQ showed preliminary evidence of multidimensionality with two factors, accounting for 38 % and 27 % of the variance respectively. The PARQ in its original version does not adhere to expectations of the Rasch model. A transformed version of the PARQ obtained by deletion of the general adherence scale and modification of visual analog scales into 5-point likert scales improved fit to the model and showed preliminary evidence of unidimensionality. The PARQ was transformed based on the results of the Rasch analysis. The transformed version of the PARQ shows preliminary evidence of unidimensionality and may allow computation of a total score, although further testing is needed to verify these findings.

Comparison of intra-articular triamcinolone hexacetonide and triamcinolone acetonide in oligoarticular juvenile idiopathic arthritis.

PubMed

Zulian, F; Martini, G; Gobber, D; Agosto, C; Gigante, C; Zacchello, F

2003-10-01

To compare the efficacy and safety of intra-articular triamcinolone hexacetonide (TH) and triamcinolone acetonide (TA) in children with oligoarticular juvenile idiopathic arthritis (JIA). One hundred and thirty joints of 85 patients undergoing intra-articular injections were randomly treated with either TH or TA depending on the availability of the drug. The efficacy of both treatments was evaluated prospectively in a blinded fashion. A good response was defined as a decrease in the articular score of > or =60% from baseline. Clinical, laboratory and immunological variables were noted in order to examine possible factors, other than treatment, predictive of the result. Seventy injections were performed using TH and 60 with TA. The two groups were comparable for clinical, immunological and laboratory characteristics. The rate of response was significantly higher with TH than with TA: 81.4% vs 53.3% (P = 0.001) at 6 months, 67.1 vs 43.3% (P = 0.006) at 12 months, and 60 vs 33.3% (P = 0.002) at 24 months. At comparable doses TH appeared to be much more effective than TA for intra-articular use, in both short- and long-term follow-up. This result was not affected by disease duration or degree of local and systemic inflammation.

Anti-adalimumab antibodies in a cohort of patients with juvenile idiopathic arthritis: incidence and clinical correlations.

PubMed

Marino, Achille; Real-Fernández, Feliciana; Rovero, Paolo; Giani, Teresa; Pagnini, Ilaria; Cimaz, Rolando; Simonini, Gabriele

2018-05-01

Adalimumab is a TNF-α blocker antibody similar in structure and function to natural human IgG1. Even if adalimumab is fully humanized, the development of anti-drug antibodies has been reported in several inflammatory conditions. The objective of our study was to assess the presence of anti-adalimumab antibodies (AAA) and their clinical relevance in a cohort of juvenile idiopathic arthritis (JIA) patients on adalimumab. This is a prospective observational cohort study recruiting JIA children. Experiments were performed using a validated surface plasmon resonance (SPR)-based optical assay (Biacore® T100). Disease activity was evaluated using the Juvenile Arthritis Disease Activity Score with 10 joint count (JADAS-10). The Mann-Whitney U test, Wilcoxon signed-rank test for paired samples, chi-square, and Fisher exact test were used to compare data. Pearson's and Spearman's correlation tests were used to determine correlation coefficients for entered variables: demographic, clinical, and serological data. Ten (37%) out of 27 patients included in the study had at least one AAA-positive sample. Patients developed AAA between 3 and 38 months after starting adalimumab. Seven (70%) out of 10 children with AAA positivity experienced at least a relapse compared to 4 (23.5%) out of 17 AAA-negative children (r s 0.45, p < 0.017). In conclusion, using an innovative and accurate assay method, we found a high incidence of anti-drug antibodies in a cohort of adalimumab-treated JIA patients observed over a mean period of 40 weeks; the presence of anti-adalimumab antibodies seemed to be related to the number of relapses.

Comics as an educational tool for children with juvenile idiopathic arthritis.

PubMed

Mendelson, Amir; Rabinowicz, Noa; Reis, Yonit; Amarilyo, Gil; Harel, Liora; Hashkes, Philip J; Uziel, Yosef

2017-09-02

This study examined whether the comic book Neta and the Medikidz Explain JIA would improve disease-related knowledge and treatment adherence among patients with juvenile idiopathic arthritis (JIA). In this prospective cohort study, JIA patients answered 20 multiple-choice knowledge questions about their disease, before and after reading the comic book. Demographic, clinical, health-related quality of life and adherence data were recorded and correlated to the responses. We studied 61 patients with a mean age of 14 ± 3.3 (range 8-18) years, 67% female, 83% Jewish and 17% non-Jewish. Thirty-nine percent had oligoarthritis, 13% systemic, 32% polyarthritis 11% psoriatic and 5% enthesitis-related type JIA. The disease was active in 46%, 40% were treated with biologics/disease modifying anti-rheumatic drugs, and 34% were in remission on medication. Among the 53 patients who completed before and after quizzes, average score increased from 63 to 80% (P < 0.001). Non-Jewish patients initially scored lower than Jewish patients (48%), but their score increased to 79% after reading the comic book. Twenty-seven patients who also completed the quiz 1 year after the first reading retained their knowledge (79%). We did not find a statistically significant correlation between knowledge and age, sex, disease subtype, or Child Health Questionnaire quality of life scores. Adherence to medication use, physical therapy and rheumatology clinic visits were high at baseline; thus, these did not change after reading the comic. The comic booklet Neta and the Medikidz Explain JIA is a good educational tool for increasing disease-related knowledge in children with JIA.

Prevalence of overweight in children and adolescents with juvenile idiopathic arthritis.

PubMed

Schenck, S; Niewerth, M; Sengler, C; Trauzeddel, R; Thon, A; Minden, K; Klotsche, J

2015-01-01

To assess the prevalence of overweight in patients with juvenile idiopathic arthritis (JIA) between 2003 and 2012 and to determine correlates of overweight relevant to the change in the overweight rate. Annual overweight prevalence was determined in the National Paediatric Rheumatological Database (NPRD) between 2003 and 2012. The prevalence of overweight in JIA was compared to representative data from Germany in 2005. The median age of JIA patients was 11.5 years and the mean disease duration 4 years. Almost 50% of JIA patients had persistent oligoarthritis, followed by rheumatoid factor (RF)-negative polyarthritis (14%). The overweight prevalence decreased significantly from 14.2% in 2003 to 8.3% in 2012 [odds ratio (OR) 0.92, 95% confidence interval (CI) 0.89-0.95]. Higher levels of physical activity and less frequent treatment with high-dose glucocorticoids (GCs) were associated with decreasing overweight rates. Systemic JIA had the highest decrease in the overweight rate over time. Patients with JIA had an overweight rate comparable to that of children and adolescents in the general population. However, systemic JIA and enthesitis-related arthritis were more likely to be associated with overweight. The use of high-dose GCs, lower functional limitations, and a lower level (or lack) of participation in school sports were significant predictors of overweight in multivariable analyses. The prevalence of overweight in JIA was comparable to the general population and decreased significantly over time. The decrease was associated with higher functional ability and JIA patients should be encouraged to be more physically active. The role of an elevated body mass index (BMI) in the long-term outcome of JIA needs to be addressed in future studies.

Parents' willingness to pay for biologic treatments in juvenile idiopathic arthritis.

PubMed

Burnett, Heather F; Ungar, Wendy J; Regier, Dean A; Feldman, Brian M; Miller, Fiona A

2014-12-01

Biologic therapies are considered the standard of care for children with the most severe forms of juvenile idiopathic arthritis (JIA). Inconsistent and inadequate drug coverage, however, prevents many children from receiving timely and equitable access to the best treatment. The objective of this study was to evaluate parents' willingness to pay (WTP) for biologic and nonbiologic disease-modifying antirheumatic drugs (DMARDs) used to treat JIA. Utility weights from a discrete choice experiment were used to estimate the WTP for treatment characteristics including child-reported pain, participation in daily activities, side effects, days missed from school, drug treatment, and cost. Conditional logit regression was used to estimate utilities for each attribute level, and expected compensating variation was used to estimate the WTP. Bootstrapping was used to generate 95% confidence intervals for all WTP estimates. Parents had the highest marginal WTP for improved participation in daily activities and pain relief followed by the elimination of side effects of treatment. Parents were willing to pay $2080 (95% confidence interval $698-$4065) more for biologic DMARDs than for nonbiologic DMARDs if the biologic DMARD was more effective. Parents' WTP indicates their preference for treatments that reduce pain and improve daily functioning without side effects by estimating the monetary equivalent of utility for drug treatments in JIA. In addition to evidence of safety and efficacy, assessments of parents' preferences provide a broader perspective to decision makers by helping them understand the aspects of drug treatments in JIA that are most valued by families. Copyright © 2014 International Society for Pharmacoeconomics and Outcomes Research (ISPOR). Published by Elsevier Inc. All rights reserved.

Genome-wide data reveal novel genes for methotrexate response in a large cohort of juvenile idiopathic arthritis cases.

PubMed

Cobb, J; Cule, E; Moncrieffe, H; Hinks, A; Ursu, S; Patrick, F; Kassoumeri, L; Flynn, E; Bulatović, M; Wulffraat, N; van Zelst, B; de Jonge, R; Bohm, M; Dolezalova, P; Hirani, S; Newman, S; Whitworth, P; Southwood, T R; De Iorio, M; Wedderburn, L R; Thomson, W

2014-08-01

Clinical response to methotrexate (MTX) treatment for children with juvenile idiopathic arthritis (JIA) displays considerable heterogeneity. Currently, there are no reliable predictors to identify non-responders: earlier identification could lead to a targeted treatment. We genotyped 759 JIA cases from the UK, the Netherlands and Czech Republic. Clinical variables were measured at baseline and 6 months after start of the treatment. In Phase I analysis, samples were analysed for the association with MTX response using ordinal regression of ACR-pedi categories and linear regression of change in clinical variables, and identified 31 genetic regions (P<0.001). Phase II analysis increased SNP density in the most strongly associated regions, identifying 14 regions (P<1 × 10(-5)): three contain genes of particular biological interest (ZMIZ1, TGIF1 and CFTR). These data suggest a role for novel pathways in MTX response and further investigations within associated regions will help to reach our goal of predicting response to MTX in JIA.

Effectiveness of Dexamethasone Iontophoresis for Temporomandibular Joint Involvement in Juvenile Idiopathic Arthritis

PubMed Central

Mina, Rina; Melson, Paula; Powell, Stephanie; Rao, Marepalli; Hinze, Claas; Passo, Murray; Graham, T. Brent; Brunner, Hermine I.

2011-01-01

Objective Temporomandibular joint (TMJ) involvement is common in Juvenile Idiopathic Arthritis (JIA). Dexamethasone iontophoresis (DIP) uses low-grade electric currents for transdermal dexamethasone delivery into deeper anatomic structures. The purpose of this study was to assess the safety and effectiveness of DIP for the treatment of TMJ involvement in JIA, and to delineate variables that are associated with improvement after DIP. Methods Medical records of all JIA patients who underwent DIP for TMJ involvement at a larger tertiary pediatric rheumatology center from 1997 to 2011 were reviewed. DIP was performed using a standard protocol. The effectiveness of DIP was assessed by comparing the maximal inter-incisor opening (MIOTMJ) and the maximal lateral excursion (MLETMJ) before and after treatment. Results Twenty-eight patients (ages 2– 21 years) who received an average of eight DIP treatment sessions per involved TMJ were included in the analysis. Statistically significant improvement in the median MIOTMJ (p< 0.0001) was observed in 68%. The median MLETMJ (p= 0.03) improved in 69%, and resolution of TMJ pain occurred in 73% of the patients who had TMJ pain at baseline. Side effects of DIP were transient site erythema (86%), skin blister (4%), and metallic taste (4%). Improvement in TMJ range of motion from DIP is associated with lower MIOTMJ, lower MLETMJ, and absence of TMJ crepitus at baseline. Conclusion In this pilot study DIP appeared to be an effective and safe initial treatment of TMJ involvement in JIA, especially among patients with decreased TMJ measurements. Prospective controlled studies are needed. PMID:22034112

Osteoporosis

MedlinePlus

... Staying Safe Videos for Educators Search English Español Osteoporosis KidsHealth / For Kids / Osteoporosis What's in this article? ... you're in your mid-20s. What Is Osteoporosis? If someone has osteoporosis (say: oss-tee-oh- ...

Accelerometry-based monitoring of daily physical activity in children with juvenile idiopathic arthritis.

PubMed

Nørgaard, M; Twilt, M; Andersen, L B; Herlin, T

2016-01-01

Juvenile idiopathic arthritis (JIA) may cause functional impairment, reduced participation in physical activity (PA) and, over time, physical deconditioning. The aim of this study was to objectively monitor daily free-living PA in 10-16-year-old children with JIA using accelerometry with regard to disease activity and physical variables and to compare the data with those from healthy age- and gender-matched controls. Patients underwent an evaluation of disease activity, functional ability, physical capacity, and pain. Accelerometer monitoring was assessed using the GT1M ActiGraph. Normative data from two major studies on PA in Danish schoolchildren were used for comparison. Data of accelerometry were available for 61 JIA patients and 2055 healthy controls. Of the JIA patients, 57% showed below-average values of maximal physical capacity (fitness level). JIA patients showed low disease activity and pain and were physically well functioning. Accelerometer counts were lower in JIA patients than in controls. Accelerometer measurements were negatively correlated with disease activity, erythrocyte sedimentation rate (ESR), and number of joints with swelling and/or limited range of motion (ROM). No correlation was found between PA and pain scores, functional ability, and hypermobility. Patients with involvement of ankles or hips demonstrated significantly lower levels of PA. Children with JIA are less physically active and have lower physical capacity and fitness than their age- and gender-matched healthy peers despite good disease control. The involvement of hips or ankles is associated with lower PA.

Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis.

PubMed

De Benedetti, Fabrizio; Brunner, Hermine I; Ruperto, Nicolino; Kenwright, Andrew; Wright, Stephen; Calvo, Inmaculada; Cuttica, Ruben; Ravelli, Angelo; Schneider, Rayfel; Woo, Patricia; Wouters, Carine; Xavier, Ricardo; Zemel, Lawrence; Baildam, Eileen; Burgos-Vargas, Ruben; Dolezalova, Pavla; Garay, Stella M; Merino, Rosa; Joos, Rik; Grom, Alexei; Wulffraat, Nico; Zuber, Zbigniew; Zulian, Francesco; Lovell, Daniel; Martini, Alberto

2012-12-20

Systemic juvenile idiopathic arthritis (JIA) is the most severe subtype of JIA; treatment options are limited. Interleukin-6 plays a pathogenic role in systemic JIA. We randomly assigned 112 children, 2 to 17 years of age, with active systemic JIA (duration of ≥6 months and inadequate responses to nonsteroidal antiinflammatory drugs and glucocorticoids) to the anti-interleukin-6 receptor antibody tocilizumab (at a dose of 8 mg per kilogram of body weight if the weight was ≥30 kg or 12 mg per kilogram if the weight was <30 kg) or placebo given intravenously every 2 weeks during the 12-week, double-blind phase. Patients meeting the predefined criteria for nonresponse were offered open-label tocilizumab. All patients could enter an open-label extension. At week 12, the primary end point (an absence of fever and an improvement of 30% or more on at least three of the six variables in the American College of Rheumatology [ACR] core set for JIA, with no more than one variable worsening by more than 30%) was met in significantly more patients in the tocilizumab group than in the placebo group (64 of 75 [85%] vs. 9 of 37 [24%], P<0.001). At week 52, 80% of the patients who received tocilizumab had at least 70% improvement with no fever, including 59% who had 90% improvement; in addition, 48% of the patients had no joints with active arthritis, and 52% had discontinued oral glucocorticoids. In the double-blind phase, 159 adverse events, including 60 infections (2 serious), occurred in the tocilizumab group, as compared with 38, including 15 infections, in the placebo group. In the double-blind and extension periods combined, 39 serious adverse events (0.25 per patient-year), including 18 serious infections (0.11 per patient-year), occurred in patients who received tocilizumab. Neutropenia developed in 19 patients (17 patients with grade 3 and 2 patients with grade 4), and 21 had aminotransferase levels that were more than 2.5 times the upper limit of the normal range

Adalimumab plus Methotrexate for Uveitis in Juvenile Idiopathic Arthritis.

PubMed

Ramanan, Athimalaipet V; Dick, Andrew D; Jones, Ashley P; McKay, Andrew; Williamson, Paula R; Compeyrot-Lacassagne, Sandrine; Hardwick, Ben; Hickey, Helen; Hughes, Dyfrig; Woo, Patricia; Benton, Diana; Edelsten, Clive; Beresford, Michael W

2017-04-27

Adalimumab, a fully human anti-tumor necrosis factor α monoclonal antibody, is effective in the treatment of juvenile idiopathic arthritis (JIA). We tested the efficacy of adalimumab in the treatment of JIA-associated uveitis. In this multicenter, double-blind, randomized, placebo-controlled trial, we assessed the efficacy and safety of adalimumab in children and adolescents 2 years of age or older who had active JIA-associated uveitis. Patients who were taking a stable dose of methotrexate were randomly assigned in a 2:1 ratio to receive either adalimumab (at a dose of 20 mg or 40 mg, according to body weight) or placebo, administered subcutaneously every 2 weeks. Patients continued the trial regimen until treatment failure or until 18 months had elapsed. They were followed for up to 2 years after randomization. The primary end point was the time to treatment failure, defined according to a multicomponent intraocular inflammation score that was based on the Standardization of Uveitis Nomenclature criteria. The prespecified stopping criteria were met after the enrollment of 90 of 114 patients. We observed 16 treatment failures in 60 patients (27%) in the adalimumab group versus 18 treatment failures in 30 patients (60%) in the placebo group (hazard ratio, 0.25; 95% confidence interval [CI], 0.12 to 0.49; P<0.0001 [the prespecified stopping boundary]). Adverse events were reported more frequently in patients receiving adalimumab than in those receiving placebo (10.07 events per patient-year [95% CI, 9.26 to 10.89] vs. 6.51 events per patient-year [95% CI, 5.26 to 7.77]), as were serious adverse events (0.29 events per patient-year [95% CI, 0.15 to 0.43] vs. 0.19 events per patient-year [95% CI, 0.00 to 0.40]). Adalimumab therapy controlled inflammation and was associated with a lower rate of treatment failure than placebo among children and adolescents with active JIA-associated uveitis who were taking a stable dose of methotrexate. Patients who received adalimumab

HLA-DRB1 alleles and juvenile idiopathic arthritis: Diagnostic clues emerging from a meta-analysis.

PubMed

De Silvestri, Annalisa; Capittini, Cristina; Poddighe, Dimitri; Marseglia, Gian Luigi; Mascaretti, Luca; Bevilacqua, Elena; Scotti, Valeria; Rebuffi, Chiara; Pasi, Annamaria; Martinetti, Miryam; Tinelli, Carmine

2017-12-01

Juvenile Idiopathic Arthritis (JIA) is characterized with a variable pattern of articular involvement and systemic symptoms and, thus, it has been classified in several subtypes. Genetic predisposition to JIA is mainly due to HLA class II molecules (HLA-DRB1, HLA-DPB1), although HLA class I molecules and non-HLA genes have been implicated, too. Here, we carried out a meta-analysis including selected studies designed to assess HLA genetic background of JIA patients, compared to healthy controls; particularly, we focused our attention on HLA-DRB1. In summary, our meta-analysis showed four main findings regarding HLA-DRB1 locus as a genetic factor of JIA: i) HLA-DRB1*08 is a strong factor predisposing to JIA, both for oligo-articular and poly-articular forms (oJIA>pJIA); ii) HLA-DRB1*01 and HLA-DRB1*04 may be involved in the genetic predisposition of Rheumatoid Factor (RF) positive forms of JIA; iii) HLA-DRB1*11 was confirmed to be predisposing to oligo-articular JIA; iv) HLA-DRB1*04 was confirmed to have a role in systemic JIA. Importantly, RF positivity seems to select the JIA clinical subset with the strongest immunogenetic similarities with adult rheumatoid arthritis. Copyright © 2017 Elsevier B.V. All rights reserved.

Foot function is well preserved in children and adolescents with juvenile idiopathic arthritis who are optimally managed

PubMed Central

Hendry, Gordon J.; Rafferty, Danny; Barn, Ruth; Gardner-Medwin, Janet; Turner, Debbie E.; Woodburn, James

2013-01-01

Purpose The objective of this study was to compare disease activity, impairments, disability, foot function and gait characteristics between a well described cohort of juvenile idiopathic arthritis (JIA) patients and normal healthy controls using a 7-segment foot model and three-dimensional gait analysis. Methods Fourteen patients with JIA (mean (standard deviation) age of 12.4 years (3.2)) and a history of foot disease and 10 healthy children (mean (standard deviation) age of 12.5 years (3.4)) underwent three-dimensional gait analysis and plantar pressure analysis to measure biomechanical foot function. Localised disease impact and foot-specific disease activity were determined using the juvenile arthritis foot disability index, rear- and forefoot deformity scores, and clinical and musculoskeletal ultrasound examinations respectively. Mean differences between groups with associated 95% confidence intervals were calculated using the t distribution. Results Mild-to-moderate foot impairments and disability but low levels of disease activity were detected in the JIA group. In comparison with healthy subjects, minor trends towards increased midfoot dorsiflexion and reduced lateral forefoot abduction within a 3–5° range were observed in patients with JIA. The magnitude and timing of remaining kinematic, kinetic and plantar pressure distribution variables during the stance phase were similar for both groups. Conclusion In children and adolescents with JIA, foot function as determined by a multi-segment foot model did not differ from that of normal age- and gender-matched subjects despite moderate foot impairments and disability scores. These findings may indicate that tight control of active foot disease may prevent joint destruction and associated structural and functional impairments. PMID:23142184

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

PubMed Central

Vernie, Lenneke A.; Rothova, Aniki; v. d. Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.

2016-01-01

Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as ‘JIA-uveitis’) has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze uveitis activity, complications and visual prognosis in adulthood. Methods In this multicenter study, 67 adult patients (129 affected eyes) with JIA-uveitis were retrospectively studied for best corrected visual acuity, visual fields, uveitis activity, topical/systemic treatments, ocular complications, and ocular surgeries during their 18th, 22nd and 30th year of life. Because treatment strategies changed after the year 1990, outcomes were stratified for onset of uveitis before and after 1990. Results Sixty-two of all 67 included patients (93%) had bilateral uveitis. During their 18th life year, 4/52 patients (8%) had complete remission, 28/52 (54%) had uveitis activity and 37/51 patients (73%) were on systemic immunomodulatory treatment. Bilateral visual impairment or legal blindness occurred in 2/51 patients (4%); unilateral visual impairment or legal blindness occurred in 17/51 patients (33%) aged 18 years. The visual prognosis appeared to be slightly better for patients with uveitis onset after the year 1990 (for uveitis onset before 1990 (n = 7) four patients (58%) and for uveitis onset after 1990 (n = 44) 13 patients (30%) were either visual impaired or blind). At least one ocular surgery was performed in 10/24 patients (42%) between their 18th and 22nd year of life. Conclusions Bilateral visual outcome in early adulthood in patients with JIA-uveitis appears to be fairly good, although one third of the patients developed one visually impaired or blind eye. However, a fair amount of the patients suffered from ongoing uveitis activity and needed ongoing treatment as well as surgical interventions. Awareness of these findings is important for ophthalmologists and

Medication use in juvenile uveitis patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry.

PubMed

Henderson, Lauren A; Zurakowski, David; Angeles-Han, Sheila T; Lasky, Andrew; Rabinovich, C Egla; Lo, Mindy S

2016-02-16

There is not yet a commonly accepted, standardized approach in the treatment of juvenile idiopathic uveitis when initial steroid therapy is insufficient. We sought to assess current practice patterns within a large cohort of children with juvenile uveitis. This is a cross-sectional cohort study of patients with uveitis enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRAnet) registry. Clinical information including, demographic information, presenting features, disease complications, and medications were collected. Chi-square and Fisher's exact tests were used to assess for associations between medications and clinical characteristics. Ninety-two children with idiopathic and 656 with juvenile idiopathic arthritis (JIA)-associated uveitis were identified. Indication (arthritis or uveitis) for medication use was not available for JIA patients; therefore, detailed analysis was limited to children with idiopathic uveitis. In this group, 94 % had received systemic steroids. Methotrexate (MTX) was used in 76 % of patients, with oral and subcutaneous forms given at similar rates. In multivariable analysis, non-Caucasians were more likely to be treated initially with subcutaneous MTX (P = 0.003). Of the 53 % of patients treated with a biologic DMARD, all received a tumor necrosis factor (TNF) inhibitor. TNF inhibitor use was associated with a higher frequency of cataracts (52 % vs 21 %; P = 0.001) and antinuclear antibody positivity (49 % vs 29 %; P = 0.04), although overall complication rates were not higher in these patients. Among idiopathic uveitis patients enrolled in the CARRAnet registry, MTX was the most commonly used DMARD, with subcutaneous and oral forms equally favored. Patients who received a TNF inhibitor were more likely to be ANA positive and have cataracts.

[Recommendations for the use of methotrexate in patients with juvenile idiopathic arthritis].

PubMed

Calvo, I; Antón, J; López Robledillo, J C; de Inocencio, J; Gamir, M L; Merino, R; Lacruz, L; Camacho, M; Rua, M J; Bustabad, S; Díaz Cordovés-Rego, G

2016-03-01

To develop a consensus document of recommendations for the use of methotrexate (MTX) in patients with juvenile idiopathic arthritis (JIA). A group of eleven experts proposed several clinical questions on the use of MTX in patients with JIA. A systematic review was conducted and the evidence and recommendations for each question were extracted. The results were discussed and validated by the experts in a work session to establish the final recommendations. MTX is recommended as the first drug for inducing remission in JIA, and its indication should be made according to the clinical category of the patient. Prior to treatment, it is recommended to perform a complete blood count, including white cells, levels of liver enzymes, serum creatinine, and other analytical parameters according to specific risk factors. Treatment should be initiated with a dose of 10-15 mg/m(2)/week. In cases of uveitis or polyarthritis, an initial dose of 15 mg/m(2)/week should be considered. For a better bioavailability and tolerability, it is preferable to administer MTX parenterally if the dose is ≥15 mg/m(2)/week. It is necessary to periodically perform an analytical monitoring of the patient and to assess possible alterations in liver enzymes to make changes if necessary. Combinations with biological agents may be necessary, as well as the concomitant addition of folic or folinic acid. This document describes the main recommendations for the appropriate use of MTX in JIA patients, according to scientific evidence and clinical experience. Copyright © 2015 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.

Overlap of disease susceptibility loci for rheumatoid arthritis and juvenile idiopathic arthritis

PubMed Central

Hinks, Anne; Eyre, Steve; Ke, Xiayi; Barton, Anne; Martin, Paul; Flynn, Edward; Packham, Jon; Worthington, Jane; Thomson, Wendy

2010-01-01

Background Genome-wide association studies (GWAS) have been extremely successful in the search for susceptibility risk factors for complex genetic autoimmune diseases. As more studies are published, evidence is emerging of considerable overlap of loci between these diseases. In juvenile idiopathic arthritis (JIA), another complex genetic autoimmune disease, the strategy of using information from autoimmune disease GWAS or candidate gene studies to help in the search for novel JIA susceptibility loci has been successful, with confirmed association with two genes, PTPN22 and IL2RA. Rheumatoid arthritis (RA) is an autoimmune disease that shares similar clinical and pathological features with JIA and, therefore, recently identified confirmed RA susceptibility loci are also excellent JIA candidate loci. Objective To determine the overlap of disease susceptibility loci for RA and JIA. Methods Fifteen single nucleotide polymorphisms (SNPs) at nine RA-associated loci were genotyped in Caucasian patients with JIA (n=1054) and controls (n=3531) and tested for association with JIA. Allele and genotype frequencies were compared between cases and controls using the genetic analysis software, PLINK. Results Two JIA susceptibility loci were identified, one of which was a novel JIA association (STAT4) and the second confirmed previously published associations of the TRAF1/C5 locus with JIA. Weak evidence of association of JIA with three additional loci (Chr6q23, KIF5A and PRKCQ) was also obtained, which warrants further investigation. Conclusion All these loci are good candidates in view of the known pathogenesis of JIA, as genes within these regions (TRAF1, STAT4, TNFAIP3, PRKCQ) are known to be involved in T-cell receptor signalling or activation pathways. PMID:19674979

Nailfold capillaroscopy in juvenile rheumatic diseases: known measures, patterns and indications.

PubMed

Gerhold, K; Becker, M O

2014-01-01

Nailfold capillaroscopy has become an established method in adults for the evaluation of structural abnormalities of the microcirculation associated with rheumatic disease. It is a cornerstone for the diagnostic work-up of patients with Raynaud's phenomenon and the early diagnosis of systemic sclerosis. However, this non-invasive examination may also be valuable in children and adolescents with rheumatic diseases. Based on the scarce data available, this review focuses on capillaroscopic findings in healthy children and adolescents as well as in children with juvenile systemic sclerosis, juvenile dermatomyositis, juvenile idiopathic arthritis, and Raynaud's phenomenon. In addition, it outlines the potential benefits and limitations of nailfold capillaroscopy for routine care in paediatric rheumatology.

Osteoporosis in men with idiopathic hypogonadotropic hypogonadism

DOE Office of Scientific and Technical Information (OSTI.GOV)

Finkelstein, J.S.; Klibanski, A.; Neer, R.M.

To assess the effect of testosterone deficiency on skeletal integrity in men, we determined bone density in 23 hypogonadal men with isolated gonadotropin-releasing hormone deficiency and compared those values with ones from controls. Cortical bone density, as assessed by single-photon absorptiometry of the nondominant radius, ranged from 0.57 to 0.86 g/cm2 (mean +/- SE, 0.71 +/- 0.02) in patients with fused epiphyses and from 0.57 to 0.67 g/cm2 (mean, 0.61 +/- 0.01) in patients with open epiphyses, both of which were significantly (p less than 0.001) lower than normal. Spinal trabecular bone density, as assessed by computed tomography, was similarlymore » decreased (p less than 0.0001) and ranged from 42 to 177 mg K2HPO4/cm3 (mean, 112 +/- 7). Cortical bone density was at least 2 SD below normal in 16 of 23 men, and 8 men had spinal bone densities below the fracture threshold of 80 to 100 mg K2HPO4/cm3. Osteopenia was equally severe in men with immature and mature bone ages, suggesting that abnormal bone development plays an important role in the osteopenia of men with idiopathic hypogonadotropic hypogonadism.« less

Osteoporosis

MedlinePlus

... to restore the height of the vertebrae) Spinal fusion (bones of your spine are joined together so ... osteoporosis Patient Instructions Hip fracture - discharge Preventing falls Images Compression fracture Bone density scan Osteoporosis Osteoporosis Hip ...

Vitamin D Receptor gene (VDR) transcripts in bone, cartilage, muscles and blood and microarray analysis of vitamin D responsive genes expression in paravertebral muscles of Juvenile and Adolescent Idiopathic Scoliosis patients

PubMed Central

2012-01-01

Background VDR may be considered as a candidate gene potentially related to Idiopathic Scoliosis susceptibility and natural history. Transcriptional profile of VDR mRNA isoforms might be changed in the structural tissues of the scoliotic spine and potentially influence the expression of VDR responsive genes. The purpose of the study was to determine differences in mRNA abundance of VDR isoforms in bone, cartilage and paravertebral muscles between tissues from curve concavity and convexity, between JIS and AIS and to identify VDR responsive genes differentiating Juvenile and Adolescent Idiopathic Scoliosis in paravertebral muscles. Methods In a group of 29 patients with JIS and AIS, specimens of bone, cartilage, paravertebral muscles were harvested at the both sides of the curve apex together with peripheral blood samples. Extracted total RNA served as a matrix for VDRs and VDRl mRNA quantification by QRT PCR. Subsequent microarray analysis of paravertebral muscular tissue samples was performed with HG U133A chips (Affymetrix). Quantitative data were compared by a nonparametric Mann Whitney U test. Microarray results were analyzed with GeneSpring 11GX application. Matrix plot of normalized log-intensities visualized the degree of differentiation between muscular tissue transcriptomes of JIS and AIS group. Fold Change Analysis with cutoff of Fold Change ≥2 identified differentially expressed VDR responsive genes in paravertebral muscles of JIS and AIS. Results No significant differences in transcript abundance of VDR isoforms between tissues of the curve concavity and convexity were found. Statistically significant difference between JIS and AIS group in mRNA abundance of VDRl isoform was found in paravertebral muscles of curve concavity. Higher degree of muscular transcriptome differentiation between curve concavity and convexity was visualized in JIS group. In paravertebral muscles Tob2 and MED13 were selected as genes differentially expressed in JIS and AIS

How do parents of children with juvenile idiopathic arthritis (JIA) perceive their therapies?

PubMed

Rouster-Stevens, Kelly; Nageswaran, Savithri; Arcury, Thomas A; Kemper, Kathi J

2008-06-02

Complementary and alternative medical (CAM) therapies are commonly used by pediatric patients with chronic medical conditions. Little is known about parents' perceptions of these therapies. This study describes the views of parents of patients with juvenile idiopathic arthritis (JIA) regarding conventional and CAM therapies. Parents of children with JIA seen at a pediatric rheumatology clinic were surveyed between June 1 and July 31, 2007. Questionnaires asked about patients' use of over 75 therapies in the past 30 days, their perceived helpfulness (0 = not helpful; 3 = very helpful), perceived side effects (0 = none; 3 = severe), and whether each therapy would be recommended to other patients with JIA (Yes, No, Not sure). Questionnaires were returned by 52/76 (68%) parents; patients' average age was 10.9 years and 87% were Caucasian. Medications were used by 45 (88%) patients; heat (67%) and extra rest (54%) were also commonly used. CAM therapies were used by 48 (92%), e.g., massage (54%), vitamins and other supplements (54%), avoiding foods that worsened pain (35%) and stress management techniques (33%). Among the therapies rated by 3 or more parents, those that scored 2.5 or higher on helpfulness were: biologic medications, methotrexate, naproxen, wheelchairs, orthotics, heat, vitamins C and D, music, support groups and prayer. CAM therapies had 0 median side effects and parents would recommend many of them to other families. JIA patients use diverse therapies. Parents report that many CAM therapies are helpful and would recommend them to other parents. These data can be used in counseling patients and guiding future research.

The Cutaneous Assessment Tool (CAT): Development and Reliability in Juvenile Idiopathic Inflammatory Myopathy

PubMed Central

Huber, Adam M.; Dugan, Elizabeth M.; Lachenbruch, Peter A.; Feldman, Brian M.; Perez, Maria D.; Zemel, Lawrence S.; Lindsley, Carol B.; Rennebohm, Robert M.; Wallace, Carol A.; Passo, Murray H.; Reed, Ann M.; Bowyer, Suzanne L.; Ballinger, Susan H.; Miller, Frederick W.; Rider, Lisa G.

2007-01-01

Objectives Clinical care and therapeutic trials in idiopathic inflammatory myopathies (IIM) require accurate and consistent assessment of cutaneous involvement. The Cutaneous Assessment Tool (CAT) was designed to measure skin activity and damage in IIM. We describe the development and inter-rater reliability of the CAT, and the frequency of lesions endorsed in a large population of juvenile IIM patients. Methods The CAT includes 10 activity, 4 damage and 7 combined lesions. Thirty-two photographic slides depicting IIM skin lesions were assessed by 11 raters. One hundred and twenty three children were assessed by 11 pediatric rheumatologists at ten centers. Inter-rater reliability was assessed using simple agreements and intra-class correlation coefficients (ICC). Results Simple agreements in recognizing lesions as present or absent were generally high (0.5 – 1.0). ICC's for CAT lesions were moderate (0.4 – 0.75) in both slides and real patients. ICC's for the CAT activity and damage scores were 0.71 and 0.81, respectively. CAT activity scores ranged from 0 – 44 (median 7, potential range 0 – 96) and CAT damage scores ranged from 0 – 13 (median 1, potential range 0 – 22). The most common cutaneous lesions endorsed were periungual capillary loop changes (63%), Gottron's papules/sign (53%), heliotrope rash (49%) and malar/facial erythema (49%). Conclusions Total CAT activity and damage scores have moderate to good reliability. Assessors generally agree on the presence of a variety of cutaneous lesions. The CAT is a promising, semi-quantitative tool to comprehensively assess skin disease activity and damage in IIM. PMID:17890275

Associates of school impairment in Egyptian patients with juvenile idiopathic arthritis: Sharkia Governorate.

PubMed

Abdul-Sattar, Amal; Magd, Sahar Abou El; Negm, Mohamed G

2014-01-01

The aim of this study was to investigate the factors associated with school absenteeism and poor school functioning in Egyptian children and adolescents with juvenile idiopathic arthritis (JIA). We studied 52 consecutive patients of JIA with age ≥ 7 years and duration of disease ≥ 1 year. All of the patients underwent assessment of socioeconomic and demographic characteristics, disease activity (JIDAS-27), functional ability (CHAQ), depressive symptoms (CDI score), and school functioning (PedsQL™ 4.0). Multivariate modeling was applied to determine the factors that associated with school absenteeism and poor school functioning. A total of 69% of the sample missed 3 weeks or more of school during past academic year. The mean percentage of missed school days was 12.5% (equivalent to 25 absent days). A total of 46% of the patients had poor school functioning (school functioning subscale score of HRQOL ≥ 1 SD below the mean of healthy children). In multiple regression analyses, high CHAQ scores, disease activity, and depressive symptoms were independent predictors for both of school absenteeism and of poor school functioning. However, living in rural regions was independently associated only with high school absenteeism in patients with JIA. Disease activity, functional disability, and high depressive symptoms are predictors of school absenteeism and poor school functioning. These findings underscore the critical need for treatment strategies that have the ability to better control disease activity, to minimize functional disability, and depressive symptoms. More attention should be given to JIA patients who live in rural regions.

Long-term safety of etanercept and adalimumab compared to methotrexate in patients with juvenile idiopathic arthritis (JIA).

PubMed

Klotsche, Jens; Niewerth, Martina; Haas, Johannes-Peter; Huppertz, Hans-Iko; Zink, Angela; Horneff, Gerd; Minden, Kirsten

2016-05-01

Published evidence on the long-term safety of etanercept (ETA) and adalimumab (ADA) in patients with polyarticular juvenile idiopathic arthritis (pJIA) is still limited. To investigate the rates of serious adverse events (SAE) and of events of special interest (ESI) under ETA and ADA treatment. Patients with pJIA were prospectively observed in the national JIA biological register, Biologika in der Kinderrheumatologie, and its follow-up register, Juvenile arthritis Methotrexate/Biologics long-term Observation. We calculated the relative risks of SAE and ESI for ETA and ADA compared with methotrexate (MTX). Among the 1414 patients treated with ETA (n=1414; 4461 exposure years (EY)) and ADA (n=320; 493 EY), significantly more SAE, infections and medically important infections were observed (ETA: 4.5, 5.7, 0.9; ADA: 4.7, 11.4, 0.4 per 100 EY) compared with those treated with MTX alone (n=1455; 2.907 EY; 2.6, 5.5, 0.5 per 100 EY). The risk for malignancies was not significantly increased for ETA and ADA compared with MTX (0.09, 0.27 and 0.07/100 person-years). Patients under ETA monotherapy developed more frequently incident inflammatory bowel disease (IBD) and incident uveitis (0.5 and 0.8/100 EY) than patients treated by ETA in combination with MTX (0.1 and 0.2/100 EY) or MTX alone (0.03 and 0.1/100 EY). Our data confirm the acceptable long-term tolerability of ETA and ADA in pJIA. However, whether the onset of IBD and uveitis during ETA monotherapy is a paradoxical effect or an inadequate response to therapy remains unclear and requires further investigation in this growing cohort. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Health-related quality of life among Swedish children with Juvenile Idiopathic Arthritis: parent-child discrepancies, gender differences and comparison with a European cohort.

PubMed

Lundberg, Veronica; Eriksson, Catharina

2017-04-12

This study investigates gender differences in self-reports and between parent and child reports in Health-related Quality of Life (HRQOL), measured with disease-specific and generic instruments for chronic disease. Comparison of HRQOL results in this Juvenile Idiopathic Arthritis (JIA) sample to a European cohort of children with JIA and one of children with other health conditions are also made. Fifty-three children with juvenile idiopathic arthritis (JIA), aged 8-18 years, and their parents completed the condition-specific DISABKIDS for JIA, and the DISABKIDS generic instrument for chronic conditions (DCGM-37) in a cross-sectional study. European reference data were used for comparison of child and parental reports. Child self-reports in DCGM-37 and DISABKIDS for JIA showed no gender differences. Parental and child reports of the child's HRQOL differed only in DCGM-37; this was among girls who scored their independence (p = 0.03), physical limitation (p = 0.01), social exclusion (p = 0.03), emotions (p <0.01), and general transformed score (p <0.01) higher than did their parents. Our sample of children with JIA reported more physical limitation compared to samples of European children with JIA (p = 0.01), European children with chronic conditions (p < 0.01), and their parents (p = 0.01 and p < 0.01). The Swedish children reported more problem with understanding compared to the European JIA sample (p = 0.03). Swedish parents perceived their children's independence significantly lower than did the European parents of JIA children (p < 0.01), as well as European parents of children with chronic conditions (p = 0.03). The Swedish parents also perceived their children to have significantly lower social inclusion (p < 0.05) and general transformed score (p = 0.04), in comparison to European parents of children with chronic conditions. Parent-child differences in assessment of quality of life depend on the HRQOL instrument

Comparing the importance of quality measurement themes in juvenile idiopathic inflammatory myositis between patients and families and healthcare professionals.

PubMed

Tory, Heather O; Carrasco, Ruy; Griffin, Thomas; Huber, Adam M; Kahn, Philip; Robinson, Angela Byun; Zurakowski, David; Kim, Susan

2018-04-19

A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA. The survey asked respondents to rate the importance of 19 items related to aspects of high quality care, using a Likert scale. Patients and families gave generally higher scores for importance to most of the quality measurement themes compared with healthcare professionals, with ratings of 13 of the 19 measures reaching statistical significance (p < 0.05). Of particular importance, however, was consensus between the groups on the top five most important items: quality of life, timely diagnosis, access to rheumatology, normalization of functioning/strength, and ability for self care. Despite overall differences in the rating of importance of quality indicators between patients and families and healthcare professionals, the groups agreed on the most important aspects of care. Recognizing areas of particular importance to patients and families, and overlapping in importance with providers, will promote the development of standardized quality measures with the greatest potential for improving care and outcomes for children with JIIM.

Determinants of health-related quality of life impairment in Egyptian children and adolescents with juvenile idiopathic arthritis: Sharkia Governorate.

PubMed

Abdul-Sattar, Amal B; Elewa, Enass A; El-Shahawy, Eman El-Dessoky; Waly, Eman H

2014-08-01

The aim of this study was to identify the possible determinants of impaired health-related quality of life (HRQOL) in Egyptian children and adolescents with juvenile idiopathic arthritis (JIA). Fifty-eight consecutive patients of JIA aged from 8 to 18 years underwent assessment of socio-economic and demographic characteristics; HRQOL using Pediatric Quality of Life Inventory 4.0 Generic Core Scale, disease activity using the Juvenile Arthritis Disease Activity Score based on 27 joints (JADAS-27), functional ability using the childhood health assessment questionnaire (CHAQ), pain score on visual analog scale and psychological symptoms using the Children's Depression Inventory (CDI) score. Multivariate modeling was applied to determine the factors that associated with HRQOL impairment. A total of 55 % of the patients (32 of 58) had impaired HRQOL (<78.6). In multiple regression analyses, high CHAQ scores (OR 6.0, 95 % CI 2.0-17.5, P = 0.001), pain (OR 3.1, 95 % CI 1.9-6.3, P = 0.01), stop going to school (OR 3.9, 95 % CI 2.0-7.3, P = 0.01), low socioeconomic status (OR 2.3, 95 % CI 1.09-4.7, P = 0.04) and high psychological symptoms (OR 4.2, 95 % CI 2.0-12.6, P = 0.001) were determinants for HRQOL impairment. HRQOL impairment is a significant problem in Egyptian children and adolescents with JIA. These findings underscore the critical need for monitoring of HRQOL in these patients. More attention should be given to JIA patients who stop going to school and who has low socioeconomic status.

Assessment of left atrial mechanical functions and atrial electromechanical delay in Juvenile idiopathic arthritis by tissue Doppler echocardiography.

PubMed

El Eraky, Azza Z; Handoka, Nesrin M; Ghaly, Mona Sayed; Nasef, Samah Ismail; Eldahshan, Nahed A; Ibrahim, Ahmed M; Shalaby, Sherein

2016-11-24

Juvenile idiopathic arthritis (JIA) is a systemic chronic inflammatory disease. Studies using tissue Doppler imaging (TDI) for the evaluation of cardiac functions of children with JIA are limited. Thus, this study was conducted to evaluate Left ventricular function, left atrial mechanical functions and atrial electromechanical delay in JIA. This study was carried out as a across sectional study. A total of 34 patients with active JIA and 34 controls were included. Atrial electromechanical delay and left atrial (LA) mechanical functions in addition to systolic and diastolic left ventricular (LV) functions were measured by using conventional echocardiography and TDI. Assessment of disease activity was done using Juvenile arthritis disease activity score (JADAS-27). JIA patients had abnormal atrial electromechanical coupling as established from prolonged lateral mitral annulus (PA lateral), septal mitral annulus (PA septum), inter-atrial and intra-atrial electromechanical delays compared with healthy controls. Left ventricular filling abnormalities were found characterized by a reduced E/A ratio (1.07 ± 0.56 vs. 1.48 ± 0.16, p = 0.01). E/Em was significantly higher in patients with JIA (7.58 ± 1.79 vs. 4.74 ± 1.45, p = 0.003) denoting impaired diastolic function. Left atrial mechanical functions assessment showed significantly decreased LA passive emptying fraction, increased LA active emptying fraction and LA total emptying volume in JIA patients (p = 0.01, p = 0.01, p = 0.03 respectively). Atrial electromechanical coupling intervals, and LA mechanical functions were impaired which can be considered as an early form of subclinical cardiac involvement in JIA patients. Significant diastolic functional abnormalities exist in JIA.

Usefulness of Adalimumab in the Treatment of Refractory Uveitis Associated with Juvenile Idiopathic Arthritis

PubMed Central

García-De-Vicuña, Carmen; Díaz-Llopis, Manuel; Salom, David; Bou, Rosa; Díaz-Cascajosa, Jesus; Cordero-Coma, Miguel; Ortega, Gabriela; Ortego-Centeno, Norberto; Suarez-De-Figueroa, Marta; Cruz-Martínez, Juan; Fonollosa, Alex; Blanco, Ricardo; García-Aparicio, Ángel María; Benítez-Del-Castillo, Jose M.; Antón, Jordi

2013-01-01

Purpose. To assess the efficacy and safety of adalimumab in patients with juvenile idiopathic arthritis (JIA) and associated refractory uveitis. Design. Multicenter, prospective case series. Methods. Thirty-nine patients (mean [SD] age of 11.5 [7.9] years) with JIA-associated uveitis who were either not responsive to standard immunosuppressive therapy or intolerant to it were enrolled. Patients aged 13–17 years were treated with 40 mg of adalimumab every other week for 6 months and those aged 4–12 years received 24 mg/m2 body surface. Results. Inflammation of the anterior chamber (2.02 [1.16] versus 0.42 [0.62]) and of the posterior segment (2.38 [2.97] versus 0.35 [0.71] decreased significantly between baseline and the final visit (P < 0.001). The mean (SD) macular thickness at baseline was 304.54 (125.03) μ and at the end of follow-up was 230.87 (31.12) μ (P < 0.014). Baseline immunosuppression load was 8.10 (3.99) as compared with 5.08 (3.76) at the final visit (P < 0.001). The mean dose of corticosteroids also decreased from 0.25 (0.43) to 0 (0.02) mg (P < 0.001). No significant side effects requiring discontinuation of therapy were observed. Conclusion. Adalimumab seems to be an effective and safe treatment for JIA-associated refractory uveitis and may reduce steroid requirement. PMID:24489444

Joint cartilage thickness and automated determination of bone age and bone health in juvenile idiopathic arthritis.

PubMed

Twilt, Marinka; Pradsgaard, Dan; Spannow, Anne Helene; Horlyck, Arne; Heuck, Carsten; Herlin, Troels

2017-08-10

BoneXpert is an automated method to calculate bone maturation and bone health index (BHI) in children with juvenile idiopathic arthritis (JIA). Cartilage thickness can also be seen as an indicator for bone health and arthritis damage. The objective of this study was to evaluate the relation between cartilage thickness, bone maturation and bone health in patients with JIA. Patients with JIA diagnosed according ILAR criteria included in a previous ultrasonography (US) study were eligible if hand radiographs were taken at the same time as the US examination. Of the 95 patients 67 met the inclusion criteria. Decreased cartilage thickness was seen in 27% of the examined joints. Decreased BHI was seen in half of the JIA patient, and delayed bone maturation was seen in 33% of patients. A combination of decreased BHI and bone age was seen in 1 out of 5 JIA patients. Decreased cartilage thickness in the knee, wrist and MCP joint was negatively correlated with delayed bone maturation but not with bone health index. Delayed bone maturation and decreased BHI were not related to a thinner cartilage, but a thicker cartilage. No relation with JADAS 10 was found. The rheumatologist should remain aware of delayed bone maturation and BHI in JIA patients with cartilage changes, even in the biologic era.

Methotrexate for uveitis associated with juvenile idiopathic arthritis: value and requirement for additional anti-inflammatory medication.

PubMed

Heiligenhaus, A; Mingels, A; Heinz, C; Ganser, G

2007-01-01

To study the value of methotrexate (MTX) and the requirement for additional anti-inflammatory drugs for the treatment of severe chronic iridocyclitis associated with juvenile idiopathic arthritis (JIA). Institutional study of 35 consecutive patients with JIA started on MTX as the single systemic immunosuppressive drug for the treatment of associated iridocyclitis. The clinical epidemiologic data, course of visual acuity (VA), development of complications, and the need for additional anti-inflammatory drugs were analyzed. Mean follow-up with MTX treatment was 27.6 months. Uveitic complications were present in 31 patients before MTX treatment. With MTX, quiescence of uveitis was obtained with (n=21) or without (n=4) additional topical steroids. Additional systemic immunosuppressive drugs were required in another 7 patients: cyclosporine A (n=4), azathioprine (n=1), infliximab (n=1), or etanercept (n=1). Three patients had active uveitis at the end of the follow-up period. During MTX therapy, uveitis first developed in the unaffected fellow eyes in 2 patients, and secondary glaucoma or ocular hypertension occurred in 7 patients. The VA deteriorated in 6, improved in 13, and was stable in the remaining eyes. The data suggest that MTX is very effective in controlling inflammation of uveitis in patients with JIA. However, additional topical steroids or systemic immunosuppressive drugs are often required.

Osteoporosis-pseudoglioma syndrome, a disorder affecting skeletal strength and vision, is assigned to chromosome region 11q12-13

DOE Office of Scientific and Technical Information (OSTI.GOV)

Gong, Yaoqin; Liu, Jin; Warman, M.L.

Osteoporosis-pseudoglioma syndrome (OPS) is an autosomal recessive disorder characterized by severe juvenile-onset osteoporosis and congenital or juvenile-onset blindness. The pathogenic mechanism is not known. Clinical, biochemical, and microscopic analyses suggest that OPS may be a disorder of matrix homeostasis rather than a disorder of matrix structure. Consequently, identification of the OPS gene and its protein product could provide insights regarding common osteoporotic conditions, such as postmenopausal and senile osteoporosis. As a first step toward determining the cause of OPS, we utilized a combination of traditional linkage analysis and homozygosity mapping to assign the OPS locus to chromosome region 11q12-13. Mappingmore » was accomplished by analyzing 16 DNA samples (seven affected individuals) from three different consanguineous kindreds. Studies in 10 additional families narrowed the candidate region, supported locus homogeneity, and did not detect founder effects. The OPS locus maps to a 13-cM interval between D11S1298 and D11S971 and most likely lies in a 3-cM region between GSTP1 and D11S1296. At present, no strong candidate genes colocalize with OPS. 33 refs., 2 figs., 1 tab.« less

[Secondary osteoporosis and glucocorticoid-induced osteoporosis].

PubMed

Orcel, P; Krane, S M

2000-10-01

In order to assess properly the diagnosis of osteoporosis, a short clinical investigation is required to address potential causes for bone loss. Osteoporosis used to be suspected in a patient with vertebral demineralization, but nowadays it is often diagnosed in a patient with a low bone mass on a screening dual-energy X-ray absorptiometry (DEXA). In this setting, it is important for the clinician to look for secondary osteoporosis, especially in men in whom secondary osteoporosis is more frequent than in women, before discussing any specific therapy. The major causes are longterm glucocorticoid therapy, endocrine (hypogonadism, primary hyperparathyroidism, hyperthyroidism), or digestive diseases.

Outcomes of bracing in juvenile idiopathic scoliosis until skeletal maturity or surgery.

PubMed

Khoshbin, Amir; Caspi, Liora; Law, Peggy W; Donaldson, Sandra; Stephens, Derek; da Silva, Trevor; Bradley, Catharine S; Wright, James G

2015-01-01

Retrospective comparative study. To evaluate the outcome of bracing in patients with juvenile idiopathic scoliosis (JIS) at either skeletal maturity or time of scoliosis surgery. JIS is generally thought to have poor outcomes with high rates of surgical fusion. All patients with JIS between the ages of 4 and 10 years treated with a brace at the Hospital for Sick Children (SickKids) between 1989 and 2011 were eligible. Data were collected from patient health records until either 2 years after skeletal maturity or date of surgery. The average age at diagnosis of 88 patients with JIS was 8.4 ± 1.4 years, with a female to male ratio of approximately 8:1. Pretreatment, Risser score was zero for 80 patients (91%); 72 (92%) of the females were premenarche; and primary Cobb angles ranged from 20° to 71°. Of the 88 patients, 60 (68%) had used a thoracolumbosacral orthosis exclusively; 28 (32%) patients used "other braces" (Milwaukee, Charleston, or a combination of braces), with an average treatment duration of 3.6 ± 1.9 years.As per Scoliosis Research Society definitions, a "non-curve-progression" (≤5° change) group consisted of 25 (28%) patients; and a "curve-progression" group consisted of 63 (72%) patients where the curve had progressed 6° or more.Of the 88 patients, 44 (50%) underwent surgery. The operative rate was higher for patients with curves 30° or more than those with curves 20° to 29° prior to brace treatment (37/58 [64%] vs. 7/30 [23%], respectively; P = 0.001); other braces compared with thoracolumbosacral orthosis (19/28 [68%] vs. 25/60 [42%], respectively; P = 0.02); Lenke I and III curves compared with Lenke VI curves (33/54 [61%] vs. 2/14 [14%], respectively; P = 0.007).

Treating juvenile idiopathic arthritis to target: recommendations of an international task force.

PubMed

Ravelli, Angelo; Consolaro, Alessandro; Horneff, Gerd; Laxer, Ronald M; Lovell, Daniel J; Wulffraat, Nico M; Akikusa, Jonathan D; Al-Mayouf, Sulaiman M; Antón, Jordi; Avcin, Tadej; Berard, Roberta A; Beresford, Michael W; Burgos-Vargas, Ruben; Cimaz, Rolando; De Benedetti, Fabrizio; Demirkaya, Erkan; Foell, Dirk; Itoh, Yasuhiko; Lahdenne, Pekka; Morgan, Esi M; Quartier, Pierre; Ruperto, Nicolino; Russo, Ricardo; Saad-Magalhães, Claudia; Sawhney, Sujata; Scott, Christiaan; Shenoi, Susan; Swart, Joost F; Uziel, Yosef; Vastert, Sebastiaan J; Smolen, Josef S

2018-06-01

Recent therapeutic advances in juvenile idiopathic arthritis (JIA) have made remission an achievable goal for most patients. Reaching this target leads to improved outcomes. The objective was to develop recommendations for treating JIA to target. A Steering Committee formulated a set of recommendations based on evidence derived from a systematic literature review. These were subsequently discussed, amended and voted on by an international Task Force of 30 paediatric rheumatologists in a consensus-based, Delphi-like procedure. Although the literature review did not reveal trials that compared a treat-to-target approach with another or no strategy, it provided indirect evidence regarding an optimised approach to therapy that facilitated development of recommendations. The group agreed on six overarching principles and eight recommendations. The main treatment target, which should be based on a shared decision with parents/patients, was defined as remission, with the alternative target of low disease activity. The frequency and timeline of follow-up evaluations to ensure achievement and maintenance of the target depend on JIA category and level of disease activity. Additional recommendations emphasise the importance of ensuring adequate growth and development and avoiding long-term systemic glucocorticoid administration to maintain the target. All items were agreed on by more than 80% of the members of the Task Force. A research agenda was formulated. The Task Force developed recommendations for treating JIA to target, being aware that the evidence is not strong and needs to be expanded by future research. These recommendations can inform various stakeholders about strategies to reach optimal outcomes for JIA. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

[Low bone mineral density in juvenile idiopathic arthritis: Prevalence and related factors].

PubMed

Galindo Zavala, Rocío; Núñez Cuadros, Esmeralda; Martín Pedraz, Laura; Díaz-Cordovés Rego, Gisela; Sierra Salinas, Carlos; Urda Cardona, Antonio

2017-10-01

Height adjustment is currently recommended for Z-score bone mineral density (BMD) assessed by dual energy X-ray absorptiometry. At present there are no studies that evaluate the prevalence of low BMD in paediatric patients with Juvenile Idiopathic Arthritis (JIA) in Spain following current recommendations. To evaluate low BMD in JIA in paediatric patients with JIA in Spain following the latest recommendations, as well as to assess associated factors. Observational cross-sectional study of Spanish JIA patients from 5 to 16 years-old, followed-up in a Paediatric Rheumatology Unit between July 2014 and July 2015. Anthropometric, clinical and treatment data were recorded. Dual energy X-ray absorptiometry, and bone metabolism parameters were collected, and a completed diet and exercise questionnaire was obtained. A total of 92 children participated. The population prevalence estimation of low BMD was less than 5% (95% CI). A significant positive correlation was found in the multiple linear regression analysis between the body mass index percentile (B: 0.021; P<.001) and lean mass index (B: 0.0002; P=.012), and BMD Z-score adjusted for height (Z-SAH). A significant negative correlation was found between fat mass index (B: -0.0001; P=.018) and serum type I collagen N-propeptide (B: -0,0006; P=.036) and Z-SAH. Low BMD prevalence in JIA patients in our population is low. An adequate nutritional status and the prevalence of lean over fat mass seem to promote the acquisition of bone mass. Those JIA patients with lower BMD could be subjected to an increase of bone turnover. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Long-term treatment with rituximab in severe juvenile idiopathic arthritis-associated uveitis.

PubMed

Miserocchi, Elisabetta; Modorati, Giulio; Berchicci, Luigi; Pontikaki, Irene; Meroni, Pierluigi; Gerloni, Valeria

2016-06-01

To evaluate retrospectively the long-term efficacy of rituximab in patients with severe juvenile idiopathic arthritis (JIA)-associated uveitis. Eight patients (15 eyes) with severe and longstanding JIA uveitis, who had an inadequate response in controlling uveitis to one or more biologic agents including tumour necrosis factor blockers and abatacept, received rituximab therapy. Rituximab was given at a dose of 1000 mg per infusion on days 1 and 15 and then every 6 months. Clinical responses to treatment, including decrease in uveitis activity, visual acuity changes, reduction of concomitant local and systemic corticosteroid and/or immunosuppressants, and occurrence of adverse events, were assessed. Eight patients with a mean±SD age of 22.8±5.5 years were treated. The mean ocular disease duration was 17.7 years; the mean±SD follow-up time on rituximab was 44.75±4.9 months; and the mean number of rituximab infusions received was 8.75 (range 6-12). All patients achieved complete control of uveitis, but in two patients rituximab was discontinued due to inefficacy in treating arthritis. The decrease in uveitis activity was evident 4-5 months after the first infusion. Systemic corticosteroids and immunosuppressants used in association with rituximab were discontinued in five patients at the end of follow-up. None of the patients experienced visual worsening during the follow-up. No drug-related complications were encountered. Rituximab may be a promising effective treatment option for refractory uveitis associated with JIA leading to long-term quiescence of uveitis, particularly for patients who have not previously responded to other biologic therapies. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Exercise Therapy in Juvenile Idiopathic Arthritis: A Systematic Review and Meta-Analysis.

PubMed

Kuntze, Gregor; Nesbitt, Colleen; Whittaker, Jackie L; Nettel-Aguirre, Alberto; Toomey, Clodagh; Esau, Shane; Doyle-Baker, Patricia K; Shank, Jena; Brooks, Julia; Benseler, Susanne; Emery, Carolyn A

2018-01-01

To conduct a systematic review to evaluate the efficacy of exercise interventions in improving outcomes across domains of functioning and disability in children and adolescents with juvenile idiopathic arthritis (JIA). Seven electronic databases were systematically searched up to November 16, 2016. Original data, analytic prospective design, physical therapy-led exercise intervention evaluation, children and adolescents with JIA, and assessment of functional, structural, activity, participation, or quality of life outcomes. Two authors screened search results, and discrepancies were resolved by consensus. Of 5037 potentially relevant studies, 9 randomized controlled trials and 1 cohort study were included and scored. Study quality (Downs and Black quality assessment tool) and level of evidence (Oxford Centre of Evidence-Based Medicine model) were assessed and meta-analysis conducted where appropriate. Alternatively, a descriptive summary approach was chosen. All randomized controlled trials were moderate-quality intervention studies (level 2b evidence; median Downs and Black score, 20 out of 32; range, 15-27). Interventions included aquatic, strengthening, proprioceptive, aerobic, and Pilates exercises. Pediatric activity capacity (Child Health Assessment Questionnaire) improved with exercise (mean difference, .45; 95% confidence interval, .05-.76). Furthermore, descriptive summaries indicated improved activity capacity, body function and structure (pain and muscle strength), and quality of life outcomes. Exercise therapy appears to be well tolerated and beneficial across clinically relevant outcomes in patients with JIA. The paucity of high-quality evidence and study heterogeneity limited the ability to provide conclusive, generalizing evidence for the efficacy of exercise therapy and to provide specific recommendations for clinical practice at this time. Future research evaluating exercise program implementation using validated outcomes and detailed adherence and

Adults With Idiopathic Scoliosis Diagnosed at Youth Experience Similar Physical Activity and Fracture Rate as Controls.

PubMed

Diarbakerli, Elias; Grauers, Anna; Danielsson, Aina; Gerdhem, Paul

2017-04-01

Cross-sectional. To describe physical activity level and fracture rates in adults with idiopathic scoliosis, diagnosed before maturity, and to compare with a control group. A previous study found a lower level of sporting activities in adults treated for idiopathic scoliosis compared with controls. Other studies have shown a lower bone mass in adults with idiopathic scoliosis compared with controls. One thousand two hundred seventy-eight adults (aged 18-71 yr) with idiopathic scoliosis and 214 controls (aged 18-70 yr) were included and answered the International Physical Activity Questionnaire - Short Form (IPAQ-SF) and questions about previous fractures. The three scoliosis treatment groups (untreated n = 360, brace n = 460, and surgically treated n = 458) were compared. Furthermore, a comparison based on onset (juvenile n = 169 or adolescent n = 976) was performed. Achieved weekly moderate activity level and metabolic equivalent task (MET) minutes/week were assessed for patients and controls. Statistical comparisons were made with analysis of covariance with adjustments for age, body mass index, and sex. The proportion achieving weekly moderate activity level was 962 out of 1278 for individuals with idiopathic scoliosis (75%) and 157 out of 214 (73%) for controls (P = 0.40). The scoliosis patients reported 2016 MET-minutes/week (median value) and the controls 2456 (P = 0.06). Fracture rates did not differ (P = 0.72). Fewer surgically treated individuals achieved moderate activity level (P = 0.046) compared with the untreated and the previously braced individuals. No difference was seen regarding MET-minutes/week (P = 0.86). No differences were seen between individuals with a juvenile onset compared with individuals with an adolescent onset (all P ≥ 0.05). Adults with idiopathic scoliosis have similar physical activity level and do not sustain more fractures compared with controls. Adults with surgically treated

Diagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease.

PubMed

Dong, Siwen; Bout-Tabaku, Sharon; Texter, Karen; Jaggi, Preeti

2015-05-01

To estimate the incidence of systemic-onset juvenile idiopathic arthritis (SoJIA) within 6 months after treatment for presumed Kawasaki disease (KD) (presumed patients with KD with subsequent diagnosis of SoJIA [pKD/SoJIA]) and describe presentation differences from sole KD. We identified patients treated for KD at Nationwide Children's Hospital and from the Pediatric Health Information System from 2009-2013. We then identified the subset of children, pKD/SoJIA, who received an International Classification of Diseases, Ninth Revision code for SoJIA and had it listed at least once 3 months after and within 6 months after KD diagnosis. Demographic characteristics, readmission rates, treatments, and complications were noted. A literature review was also performed to identify clinical, laboratory, and echocardiographic data of previously documented patients with KD later diagnosed with SoJIA. There were 6745 total treated patients with KD in the Pediatric Health Information System database during the study period; 10 patients were identified to have pKD/SoJIA (0.2% of cohort). Those with pKD/SoJIA were predominantly Caucasian compared with patients with KD (90% and 46.8%, respectively; P=.003). Macrophage activation syndrome was more common in patients with pKD/SoJIA than in sole patients with KD (30% and 0.30%, respectively; P<.001). Fifteen cases of pKD/SoJIA were identified by literature and chart review, 12 of whom were initially diagnosed with incomplete KD. We reported a 0.2% incidence of pKD/SoJIA, which was associated with Caucasian race, macrophage activation syndrome, and an incomplete KD phenotype. Copyright © 2015 Elsevier Inc. All rights reserved.

PADI4 and the HLA-DRB1 shared epitope in juvenile idiopathic arthritis.

PubMed

Hisa, Kaori; Yanagimachi, Masakatsu D; Naruto, Takuya; Miyamae, Takako; Kikuchi, Masako; Hara, Rhoki; Imagawa, Tomoyuki; Yokota, Shumpei; Mori, Masaaki

2017-01-01

Both genetic and environmental factors are associated with susceptibility to juvenile idiopathic arthritis (JIA). Many studies have reported that both a 'shared epitope' (SE) encoded by several HLA-DRB1 alleles and the peptidyl arginine deiminase type 4 (PADI4) gene polymorphisms are associated with susceptibility to rheumatoid arthritis (RA). However, it is uncertain whether JIA and RA share the latter genetic risk factor. Therefore, here we investigated relationships between HLA-SE and PADI4 polymorphisms with clinical subtypes of JIA. JIA patients (39 oligoarthritis, 48 RF-positive polyarthritis, 19 RF-negative polyarthritis and 82 systemic) and 188 healthy controls were genotyped for HLA-DRB1 by PCR-sequence-specific oligonucleotide probe methodology. Three PADI4 gene single nucleotide polymorphisms (SNPs), rs2240340, rs2240337 and rs1748033, were genotyped using TaqMan SNP Genotyping Assays. Frequencies of the HLA-SE were higher in RF-positive polyarticular JIA than in healthy controls. RF-positive polyarticular JIA was associated with HLA-SE (OR = 5.3, 95% CI = 2.5-11.9, pc < 0.001). No associations were found between clinical subtypes of JIA and PADI4 allele frequency. Nonetheless, rs2240337 in the PADI4 gene was significantly associated with anti-cyclic citrullinated peptide antibody (ACPA)-positivity in JIA. The A allele at rs2240337 was a significant risk factor for ACPA positivity in JIA (OR = 5.6, 95% CI = 1.71-23.7 pc = 0.03). PADI4 gene polymorphism is associated with ACPA-positivity in JIA. The association of HLA-SE with RF-positive polyarticular JIA as well as RA is confirmed in Japanese. Thus, HLA-SE and PADI4 status both influence JIA clinical manifestations.

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

PubMed Central

Kimura, Yukiko; Weiss, Jennifer E.; Haroldson, Kathryn L.; Lee, Tzielan; Punaro, Marilynn; Oliveira, Sheila; Rabinovich, Egla; Riebschleger, Meredith; Antón, Jordi; Blier, Peter R.; Gerloni, Valeria; Hazen, Melissa M; Kessler, Elizabeth; Onel, Karen; Passo, Murray H; Rennebohm, Robert M; Wallace, Carol A; Woo, Patricia; Wulffraat, Nico

2015-01-01

Objectives Systemic Juvenile Idiopathic Arthritis (sJIA) is characterized by fevers, rash and arthritis, for which IL1 and IL6 inhibitors appear effective. Pulmonary artery hypertension (PAH), interstitial lung disease (ILD) and alveolar proteinosis (AP) have been recently reported in sJIA patients with increased frequency. Our aim was to characterize and compare these cases to a larger cohort of sJIA patients. Methods sJIA patients who developed PAH, ILD and/or AP were identified through an electronic listserv, and their demographic, sJIA and pulmonary disease characteristics, and medication exposure information were collected. These features were compared to a cohort of sJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Results Patients (N=25) were significantly (p<0.05) more likely than the CARRA registry cohort (N=389) to be female, have more systemic features, and to have been exposed to an IL-1 inhibitor, tocilizumab, infliximab, corticosteroids, intravenous immunoglobulin, cyclosporine and cyclophosphamide. Eighty% were diagnosed after 2004. Twenty (80%) patients had MAS during their disease course and 15 (60%) had MAS at pulmonary diagnosis. Sixteen patients had PAH, 5 AP and 7 ILD. Seventeen (68%) patients were taking or recently (≤1 month) discontinued a biologic agent at pulmonary symptom onset; 12 (48%) were taking anti-IL1 therapy (primarily anakinra). Seventeen (68%) patients died at a mean of 8.8 months from pulmonary diagnosis. Conclusions PAH, AP and ILD are under-recognized complications of sJIA which are frequently fatal. These may be the result of severe uncontrolled systemic disease activity, and may be influenced by medication exposure. PMID:23139240

Utility of corticosteroid injection for temporomandibular arthritis in children with juvenile idiopathic arthritis.

PubMed

Arabshahi, Bita; Dewitt, Esi Morgan; Cahill, Ann Marie; Kaye, Robin D; Baskin, Kevin M; Towbin, Richard B; Cron, Randy Q

2005-11-01

To assess the effects of computed tomography (CT)-guided injection of corticosteroid into the temporomandibular joint (TMJ) in children with juvenile idiopathic arthritis (JIA) and clinical and magnetic resonance imaging (MRI) evidence of TMJ inflammation. Twenty-three children ages 4-16 years with JIA and MRI evidence of TMJ inflammation received CT-guided TMJ injections of corticosteroid (triamcinolone acetonide [n = 16] or triamcinolone hexacetonide [n = 7]). Jaw pain or dysfunction and maximal incisal opening (MIO) distance were assessed before and after injection. Fourteen patients had followup MRI studies of the TMJ 6-12 months after injection. Of the 13 patients with symptoms of jaw pain prior to corticosteroid treatment, 10 (77%) had complete resolution of pain (P < 0.05). Prior to corticosteroid injection, MIO in all 23 patients was below age-matched normal values. After injection, the MIO was improved by at least 0.5 cm in 10 patients (43%) (P = 0.0017). Patients under 6 years of age at the time of injection showed the best response, with a postinjection MIO similar to that in age-matched controls (P = 0.2267). There was involvement of 23 TMJs in the 14 patients who had followup MRI studies; resolution of effusions was observed in 11 (48%) of the TMJs. Other than short-term facial swelling in 2 patients, there were no side effects. The majority of children with symptomatic TMJ arthritis improved after intraarticular corticosteroid injection. Approximately half the patients experienced significant improvement in MIO and TMJ effusion. These data suggest that corticosteroid injection may be a useful procedure for the prevention and treatment of morbidities associated with TMJ arthritis in JIA.

Osteoporosis in Men

MedlinePlus

... Glossary FAQs Breadcrumb Home Osteoporosis Osteoporosis in Men Osteoporosis in Men Osteoporosis is a disease that causes ... as life expectancy continues to rise. What Causes Osteoporosis? Bone is constantly changing—that is, old bone ...

Clinical significance of specific autoantibodies in juvenile dermatomyositis.

PubMed

Feldman, B M; Reichlin, M; Laxer, R M; Targoff, I N; Stein, L D; Silverman, E D

1996-10-01

To determine the prevalence and clinical association of myositis specific antibodies in an unselected group of patients with juvenile dermatomyositis (DM). The sera of 42 subjects, representing an unselected group of patients from a single center, with juvenile DM and 7 others with idiopathic inflammatory myopathy (IIM) were examined for the presence of myositis specific antibodies by immunodiffusion against calf thymus extract and immunoprecipitation with HeLa extract. Of the subjects with juvenile DM, only 2 had evidence of antibodies specific to myositis (anti-Mi2). Three other patients with juvenile DM had defined autoantibodies not usually considered to be specific to myositis. Two of the 3 subjects had anti-PM-Scl; both developed features of scleroderma after the juvenile DM remitted. The 5 subjects with defined autoantibodies did not differ clinically from the remainder of the subjects with the exception of the late development of scleroderma features in 2. Fourteen other subjects with juvenile DM had unidentified bands on immunoprecipitation, which may represent as yet undiscovered myositis specific antibodies. No myositis specific antibodies were detected in any of the 7 subjects with other IIM syndromes. Based on our findings, we do not recommend routine clinical testing for these antibodies in children with typical juvenile DM. Further study of the unidentified bands seen in our subjects may lead to better understanding of the clinical groupings and etiopathogenesis of childhood myositis.

Multiplex Cytokine Analysis of Aqueous Humor in Juvenile Idiopathic Arthritis-Associated Anterior Uveitis With or Without Secondary Glaucoma.

PubMed

Bauer, Dirk; Kasper, Maren; Walscheid, Karoline; Koch, Jörg M; Müther, Philipp S; Kirchhof, Bernd; Heiligenhaus, Arnd; Heinz, Carsten

2018-01-01

Patients with juvenile idiopathic arthritis often develop chronic anterior uveitis (JIAU). JIAU patients possess a particularly high risk for developing secondary glaucoma when inflammatory inactivity has been achieved. By using multiplex bead assay analysis, we assessed levels of pro- and anti-inflammatory cytokines, chemokines, or metalloproteinases in the aqueous humor (AH) of patients with clinically inactive JIAU with (JIAUwG) or without secondary glaucoma (JIAUwoG), or from patients with senile cataract as controls. Laser-flare photometry analysis prior to surgery showed no significant differences between JIAUwG or JIAUwoG. Compared with the control group, levels of interleukin-8, matrix metalloproteinase-2, -3, -9, serum amyloid A (SAA), transforming growth factor beta-1, -2, -3 (TGFβ-1, -2, -3), and tumor necrosis factor-alpha in the AH were significantly higher in patients with clinically inactive JIAUwG or JIAUwoG. Samples from JIAwoG patients displayed significantly higher levels of SAA ( P  < 0.0116) than JIAUwG patients. JIAUwG patients showed an increased level of TGFβ-2 in AH samples compared with JIAUwoG ( P  < 0.0009). These molecules may contribute to the clinical development of glaucoma in patients with JIAU.

Juvenile hyaline fibromatosis: a case report and review of literature.

PubMed

Ribeiro, Sandra Lúcia; Guedes, Erilane L; Botan, Valeria; Barbosa, Alessandra; Freitas, Ernani J

2009-01-01

Juvenile hyaline fibromatosis (JHF) is a rare disease with autosomal recessive inheritance that occurs mainly in childhood and is characterized by the deposition of amorphous hyaline material in the skin and other organs. There are approximately 70 cases reported in the literature. Herein we describe the case of a 14-month-old boy with multiple cutaneous nodules around small and large joints, papulous skin lesions, hyperpigmented plaques and nodules in the perianal region, flexion contractures and stiffness of joints and diffuse osteoporosis. Symptoms were present since the second month of life. Histopathologic studies of joint nodulations demonstrated the presence of hyaline material, confirming the diagnosis of juvenile hyaline fibromatosis.

Intra-articular glucocorticoid injections in patients with juvenile idiopathic arthritis in a Singapore hospital.

PubMed

Leow, Olivia Min Yi; Lim, Lee Kean; Ooi, Pei Ling; Shek, Lynette Pei Chi; Ang, Elizabeth You Ning; Son, Mary Beth

2014-05-01

This study aimed to evaluate the efficacy and safety of intra-articular glucocorticoid (IAG) injections in our institution in children with juvenile idiopathic arthritis (JIA). This is a retrospective assessment of IAG injections performed by the Department of Paediatrics, National University Hospital, Singapore, from October 2009 to October 2011. A total of 26 procedures were evaluated for efficacy, considering parameters such as clinical response, changes in systemic medication, length of time between repeat injections, safety, consent-taking, pre- and post-procedural advice, compliance with aseptic technique, and post-procedural complications. A total of 26 IAG injections of triamcinolone hexacetonide were administered over 17 occasions (i.e. patient encounters) to ten patients with JIA during the study period. After the injections, clinical scoring by a paediatric rheumatologist showed overall improvement by an average of 2.62 points out of 15. Besides six patient encounters that had an increase in systemic medication on the day of the injection, five required an increase within six months post injection, two required no adjustments, and one resulted in a decrease in medications. In all, 21 injections did not require subsequent injections. The mean interval between repeat injections was 7.8 months. Cutaneous side effects were noted in three anatomically difficult joints. Medical documentation with regard to patient progress was found to be lacking. As per the recommendations of the American College of Rheumatology, we safely used IAG injections as the first-line therapy in our group of patients with oligoarticular JIA, and/or as an adjunct to systemic therapy in our patients with JIA.

The relationship between physical activity levels and pain in children with juvenile idiopathic arthritis.

PubMed

Limenis, Elizaveta; Grosbein, Haddas A; Feldman, Brian M

2014-02-01

Pain and reduced physical activity levels are common in children with juvenile idiopathic arthritis (JIA). Currently, there is no consensus about the role of physical activity in managing pain in JIA. The purpose of our study was to assess the relationship between physical activity level and pain in children ages 11 to 18 years with JIA. A random sample of 50 patients with JIA were approached by mailed questionnaires. Physical activity was determined using the Physical Activity Questionnaire (PAQ). Pain measures included the Numerical Rating Scale (pain severity), SUPER-KIDZ body diagram (number of painful areas), and the Child Activities Limitations Inventory-21 (pain interference). Generalized linear models were used to assess the relationship between physical activity and pain, as well as the roles of sex and age. The response rate was 84%. Thirty-four respondents completed the questionnaire package. The median age was 15 years. The mean PAQ score was 2.16/5. Physical activity declines with increasing age in youth with JIA (r = 0.53, p = 0.0014). Lower physical activity is associated with greater pain interference (r = 0.39, p = 0.0217) and more severe pain (r = 0.35, p = 0.0422). Children with JIA report significantly less activity than healthy children based on PAQ scores, with physical activity declining throughout adolescence. Physical activity is inversely related to pain interference and severity in children with JIA. Our findings suggest that physical activity interventions may play an important role in the management of pain in JIA.

Erythrocyte sedimentation rate as baseline predictor for the development of uveitis in children with juvenile idiopathic arthritis.

PubMed

Haasnoot, Arenda J W; van Tent-Hoeve, Maretta; Wulffraat, Nico M; Schalij-Delfos, Nicoline E; Los, Leonoor I; Armbrust, Wineke; Zuithoff, Nicolaas P A; de Boer, Joke H

2015-02-01

To analyze inflammatory parameters as possible predictors for the development of uveitis in juvenile idiopathic arthritis (JIA) patients. Further, to analyze the predictive value of demographic and clinical factors at the onset of arthritis. Retrospective cohort study. In 358 children with oligoarthritis and rheumatoid factor-negative polyarthritis, erythrocyte sedimentation rate (ESR), C-reactive protein, leukocyte count, presence of antinuclear antibodies (ANA), presence of human leukocyte antigen (HLA-)B27, age of onset of JIA, and sex were analyzed for their predictive value for the onset of uveitis. One hundred forty-seven patients (41%) were diagnosed with chronic anterior uveitis. Young age of onset, presence of ANA, and elevated ESR appeared to be predictive factors according to univariate analyses (P = .029, P = .007, and P = 5E(-4), respectively). According to multivariate analysis, young age of onset and elevated ESR appeared to be predictive after adjusting for the other relevant factors (P = .004 and P = .001, respectively). A prediction model was developed. Elevated ESR appears to be a predictor for the occurrence of uveitis in patients with JIA. Since ESR is already routinely tested in patients with recently diagnosed arthritis, its use as a biomarker can easily be implemented in daily practice. Copyright © 2015 Elsevier Inc. All rights reserved.

Methotrexate for the treatment of juvenile idiopathic arthritis: process to approval for JIA indication in Japan

PubMed Central

Naruto, Takuya; Imagawa, Tomoyuki; Murata, Takuji; Takei, Syuji; Tomiita, Minako; Itoh, Yasuhiko; Fujikawa, Satoshi; Yokota, Shumpei

2008-01-01

Methotrexate (MTX), the primary treatment for the articular-type juvenile idiopathic arthritis (JIA), is effective and brings about radiological improvement. Patient compliance is good, and it is recognized that its known side effects, namely, disruption of liver function and induction of pulmonary lesions, are unlikely to be severe at the low MTX doses that are administered. In Japan, MTX was granted approval in 1999 by the then Ministry of Health and Welfare specifically for treating rheumatoid arthritis in adult patients, allowing it be generally used in medical institutions for patients having National Health Insurance. However, in the pediatric field, its use outside the indications has so far been unavoidable, and has been left to the discretion of the physician. Finally, at the present conference, expansion of the indications of MTX for JIA was approved in Japan. It is noteworthy that this expansion of indications was achieved without requiring clinical trials on children sponsored by the pharmaceutical company: it was achieved rather by collecting necessary information through ongoing efforts (including collection and analysis of information about approval status in foreign countries, adequate evidence from the literature, implementation of a clinical use survey in Japan, etc.). It also merits attention that the maximum dose (10 mg/m2) was set on the basis of pharmacokinetic data from children, rather than relying on the dosing method and dose for adults. PMID:18815725

Methotrexate for the treatment of juvenile idiopathic arthritis: process to approval for JIA indication in Japan.

PubMed

Mori, Masaaki; Naruto, Takuya; Imagawa, Tomoyuki; Murata, Takuji; Takei, Syuji; Tomiita, Minako; Itoh, Yasuhiko; Fujikawa, Satoshi; Yokota, Shumpei

2009-01-01

Methotrexate (MTX), the primary treatment for the articular-type juvenile idiopathic arthritis (JIA), is effective and brings about radiological improvement. Patient compliance is good, and it is recognized that its known side effects, namely, disruption of liver function and induction of pulmonary lesions, are unlikely to be severe at the low MTX doses that are administered. In Japan, MTX was granted approval in 1999 by the then Ministry of Health and Welfare specifically for treating rheumatoid arthritis in adult patients, allowing it be generally used in medical institutions for patients having National Health Insurance. However, in the pediatric field, its use outside the indications has so far been unavoidable, and has been left to the discretion of the physician. Finally, at the present conference, expansion of the indications of MTX for JIA was approved in Japan. It is noteworthy that this expansion of indications was achieved without requiring clinical trials on children sponsored by the pharmaceutical company: it was achieved rather by collecting necessary information through ongoing efforts (including collection and analysis of information about approval status in foreign countries, adequate evidence from the literature, implementation of a clinical use survey in Japan, etc.). It also merits attention that the maximum dose (10 mg/m2) was set on the basis of pharmacokinetic data from children, rather than relying on the dosing method and dose for adults.

Effects of Juvenile Idiopathic Arthritis on Kinematics and Kinetics of the Lower Extremities Call for Consequences in Physical Activities Recommendations

PubMed Central

Hartmann, M.; Kreuzpointner, F.; Haefner, R.; Michels, H.; Schwirtz, A.; Haas, J. P.

2010-01-01

Juvenile idiopathic arthritis (JIA) patients (n = 36) with symmetrical polyarticular joint involvement of the lower extremities and healthy controls (n = 20) were compared concerning differences in kinematic, kinetic, and spatio-temporal parameters with 3D gait analysis. The aims of this study were to quantify the differences in gait between JIA patients and healthy controls and to provide data for more detailed sport activities recommendations. JIA-patients showed reduced walking speed and step length, strongly anterior tilted pelvis, reduced maximum hip extension, reduced knee extension during single support phase and reduced plantar flexion in push off. Additionally the roll-off procedure of the foot was slightly decelerated. The reduced push off motion in the ankle was confirmed by lower peaks in ankle moment and power. The gait of JIA-patients can be explained as a crouch-like gait with hyperflexion in hip and knee joints and less plantar flexion in the ankle. A preventive mobility workout would be recommendable to reduce these restrictions in the future. Advisable are sports with emphasis on extension in hip, knee, and ankle plantar flexion. PMID:20862334

The dental attitudes, knowledge and health practices of patients with Juvenile Idiopathic Arthritis.

PubMed

Waterhouse, P J; Thomason, J M; Fitzgerald, J F; Foster, H E; Steen, I N; Welbury, R R

2005-12-01

To investigate the dental attitudes, knowledge and dental health practices of children and adults with a previous diagnosis of Juvenile Idiopathic Arthritis (JIA). A self-completion questionnaire. Ninety-one children and 82 adults with JIA were age and gender matched with 152 healthy controls. For those below the age of 16 years, the parents' attitude, knowledge and dental health practices were investigated by the questionnaire. The adult subjects and controls completed an identical questionnaire assessing their own attitude, knowledge and dental health practices. Response rates of 84% and 75% were achieved for the subject and controls respectively. Both groups responded similarly to questions assessing perception of different medical conditions. The majority of respondents thought leukaemia was a very serious condition. Twenty-seven percent of subjects and 34% of controls felt dental decay was "slightly or not serious". Ninety percent of subjects and 93% of controls knew having sweet snacks during the day would harm teeth, but fewer were sure that eating sweet foods at mealtimes only would help reduce decay. The majority of respondents (63% and 56% respectively) did not know whether children should receive fluoride tablets but the majority of subjects in both groups had attended a dentist within the last year. Descriptive analyses and chi-squared analysis were undertaken. A p-value of < or =0.01 was taken as strong evidence of a difference between groups. The perception of health and illness by both groups was appropriate. The questions investigating dental knowledge revealed understanding of the basic messages of prevention of dental disease, but finer detail appeared less well understood. Responses concerning dental health confirmed positive attitudes towards good dental health habits. The benefits of brushing with fluoride toothpaste were known, and the majority toothbrushed daily and received dental care within the previous year.

PADI4 and the HLA-DRB1 shared epitope in juvenile idiopathic arthritis

PubMed Central

Hisa, Kaori; Yanagimachi, Masakatsu D.; Naruto, Takuya; Miyamae, Takako; Kikuchi, Masako; Hara, Rhoki; Imagawa, Tomoyuki; Yokota, Shumpei; Mori, Masaaki

2017-01-01

Objective Both genetic and environmental factors are associated with susceptibility to juvenile idiopathic arthritis (JIA). Many studies have reported that both a ‘shared epitope’ (SE) encoded by several HLA-DRB1 alleles and the peptidyl arginine deiminase type 4 (PADI4) gene polymorphisms are associated with susceptibility to rheumatoid arthritis (RA). However, it is uncertain whether JIA and RA share the latter genetic risk factor. Therefore, here we investigated relationships between HLA-SE and PADI4 polymorphisms with clinical subtypes of JIA. Methods JIA patients (39 oligoarthritis, 48 RF-positive polyarthritis, 19 RF-negative polyarthritis and 82 systemic) and 188 healthy controls were genotyped for HLA-DRB1 by PCR-sequence-specific oligonucleotide probe methodology. Three PADI4 gene single nucleotide polymorphisms (SNPs), rs2240340, rs2240337 and rs1748033, were genotyped using TaqMan SNP Genotyping Assays. Results Frequencies of the HLA-SE were higher in RF-positive polyarticular JIA than in healthy controls. RF-positive polyarticular JIA was associated with HLA-SE (OR = 5.3, 95% CI = 2.5–11.9, pc < 0.001). No associations were found between clinical subtypes of JIA and PADI4 allele frequency. Nonetheless, rs2240337 in the PADI4 gene was significantly associated with anti-cyclic citrullinated peptide antibody (ACPA)-positivity in JIA. The A allele at rs2240337 was a significant risk factor for ACPA positivity in JIA (OR = 5.6, 95% CI = 1.71–23.7 pc = 0.03). Conclusion PADI4 gene polymorphism is associated with ACPA-positivity in JIA. The association of HLA-SE with RF-positive polyarticular JIA as well as RA is confirmed in Japanese. Thus, HLA-SE and PADI4 status both influence JIA clinical manifestations. PMID:28182665

STELLATE NONHEREDITARY IDIOPATHIC FOVEOMACULAR RETINOSCHISIS ACCOMPANIED BY CONTRALATERAL PERIPHERAL RETINOSCHISIS.

PubMed

Ahmed, Daniel; Stattin, Martin; Glittenberg, Carl; Krebs, Ilse; Ansari-Shahrezaei, Siamak

2017-01-16

To present a patient with stellate nonhereditary idiopathic foveomacular retinoschisis on one eye and peripheral retinoschisis without foveal affection on the other eye. A case report with complete workup of family history and clinical examination, including multimodal imaging with optical coherence tomography and angiography, fluorescein angiography, and infrared fundus imaging. Genetic testing for gene mutation XRLS1 was performed. A white woman with unremarkable medical history presented with stellate foveal splitting of the outer plexiform layer on the right eye and peripheral splitting of the outer plexiform layer on both eyes. All known allegeable trigger factors for the existence of a hereditary or acquired foveomacular retinoschisis were ruled out either by clinical presentation or genetic testing. This led to the diagnosis of stellate nonhereditary idiopathic foveomacular retinoschisis with central involvement only present on one eye. Although peripheral schisis of the outer plexiform layer is often concomitant with central splitting in X-linked juvenile retinoschisis, this is the first known report of nonhereditary cleavage of the outer plexiform layer of the peripheral retina without central affection in a patient with documented stellate nonhereditary idiopathic foveomacular retinoschisis on the other eye. These findings suggest an accurate bilateral examination of the peripheral retina while confirming the diagnose of stellate nonhereditary idiopathic foveomacular retinoschisis.

Prevalence of HLA-B27 antigen in patients with juvenile idiopathic arthritis.

PubMed

Żuber, Zbigniew; Turowska-Heydel, Dorota; Sobczyk, Małgorzata; Chudek, Jerzy

2015-01-01

Human leukocyte antigen B27 (HLA-B27) is considered as a risk factor for development of juvenile idiopathic arthritis (JIA). The aim of this study was to analyse the prevalence of HLA-B27 antigen in JIA categories and its influence on disease onset and response to conventional therapy. The retrospective analysis included 461 unselected children with JIA hospitalized in a single reference rheumatology centre between July 2007 and June 2012. The diagnosis was based on criteria by the International League of Association for Rheumatology. HLA-B27 was determined in 387 of all patients (84%) by hybridization of the amplified, labelled product to immobilize it on the microarray probe. HLA-B27 antigen was found in 104 of 383 affected children (27.2%), 48 of 206 girls (23.3%), and 56 of 177 boys (31.6%) - most frequently in patients with enthesitis-related arthritis (71%), psoriatic arthritis (50%) and unclassified cases (86.7%). The age of JIA onset was slightly (by 1 year) but significantly different in patients with and without HLA-B27 antigen [11 (8.5-14) vs. 10 (5-13.5) years.; p < 0.001]. The use of disease-modifying antirheumatic drugs (DMARDs) and corticosteroids was more frequently clinically ineffective in HLA-B27 positive than negative patients (23.1% vs. 15.2%; p = 0.09). Patients with polyarthritis, systemic, and psoriatic arthritis more frequently received biological therapy. HLA-B27 positive patients with enthesitis-related arthritis received biological therapy more frequently than HLA-B27 negative ones (20.4% vs. 0, respectively; p = 0.09). HLA-B27 antigen is a strong risk factor for the development of enthesitis-related arthritis, and to a lesser extent for psoriatic arthritis and extended course of oligoarthritis. The presence of this antigen does not affect the disease onset but seems to predict resistance to therapy with disease-modifying drugs and corticosteroids.

Prevalence of HLA-B27 antigen in patients with juvenile idiopathic arthritis

PubMed Central

Turowska-Heydel, Dorota; Sobczyk, Małgorzata; Chudek, Jerzy

2015-01-01

Introduction Human leukocyte antigen B27 (HLA-B27) is considered as a risk factor for development of juvenile idiopathic arthritis (JIA). The aim of this study was to analyse the prevalence of HLA-B27 antigen in JIA categories and its influence on disease onset and response to conventional therapy. Material and methods The retrospective analysis included 461 unselected children with JIA hospitalized in a single reference rheumatology centre between July 2007 and June 2012. The diagnosis was based on criteria by the International League of Association for Rheumatology. HLA-B27 was determined in 387 of all patients (84%) by hybridization of the amplified, labelled product to immobilize it on the microarray probe. Results HLA-B27 antigen was found in 104 of 383 affected children (27.2%), 48 of 206 girls (23.3%), and 56 of 177 boys (31.6%) – most frequently in patients with enthesitis-related arthritis (71%), psoriatic arthritis (50%) and unclassified cases (86.7%). The age of JIA onset was slightly (by 1 year) but significantly different in patients with and without HLA-B27 antigen [11 (8.5–14) vs. 10 (5–13.5) years.; p < 0.001]. The use of disease-modifying antirheumatic drugs (DMARDs) and corticosteroids was more frequently clinically ineffective in HLA-B27 positive than negative patients (23.1% vs. 15.2%; p = 0.09). Patients with polyarthritis, systemic, and psoriatic arthritis more frequently received biological therapy. HLA-B27 positive patients with enthesitis-related arthritis received biological therapy more frequently than HLA-B27 negative ones (20.4% vs. 0, respectively; p = 0.09). Conclusions HLA-B27 antigen is a strong risk factor for the development of enthesitis-related arthritis, and to a lesser extent for psoriatic arthritis and extended course of oligoarthritis. The presence of this antigen does not affect the disease onset but seems to predict resistance to therapy with disease-modifying drugs and corticosteroids. PMID:27407238

Intra-articular glucocorticoid injections in patients with juvenile idiopathic arthritis in a Singapore hospital

PubMed Central

Leow, Olivia Min Yi; Lim, Lee Kean; Ooi, Pei Ling; Shek, Lynette Pei Chi; Ang, Elizabeth You Ning; Son, Mary Beth

2014-01-01

INTRODUCTION This study aimed to evaluate the efficacy and safety of intra-articular glucocorticoid (IAG) injections in our institution in children with juvenile idiopathic arthritis (JIA). METHODS This is a retrospective assessment of IAG injections performed by the Department of Paediatrics, National University Hospital, Singapore, from October 2009 to October 2011. A total of 26 procedures were evaluated for efficacy, considering parameters such as clinical response, changes in systemic medication, length of time between repeat injections, safety, consent-taking, pre- and post-procedural advice, compliance with aseptic technique, and post-procedural complications. RESULTS A total of 26 IAG injections of triamcinolone hexacetonide were administered over 17 occasions (i.e. patient encounters) to ten patients with JIA during the study period. After the injections, clinical scoring by a paediatric rheumatologist showed overall improvement by an average of 2.62 points out of 15. Besides six patient encounters that had an increase in systemic medication on the day of the injection, five required an increase within six months post injection, two required no adjustments, and one resulted in a decrease in medications. In all, 21 injections did not require subsequent injections. The mean interval between repeat injections was 7.8 months. Cutaneous side effects were noted in three anatomically difficult joints. Medical documentation with regard to patient progress was found to be lacking. CONCLUSION As per the recommendations of the American College of Rheumatology, we safely used IAG injections as the first-line therapy in our group of patients with oligoarticular JIA, and/or as an adjunct to systemic therapy in our patients with JIA. PMID:24862747

Osteoporosis

MedlinePlus

Osteoporosis is a condition that leads to loss of bone mass. From the outside, osteoporotic bone is ... disease. Prevention is the best measure for treating osteoporosis by eating a recommended balanced diet including foods ...

Osteoporosis

MedlinePlus

Osteoporosis is a disease that thins and weakens the bones. Your bones become fragile and break easily, ... United States, millions of people either already have osteoporosis or are at high risk due to low ...

Osteoporosis and Hispanic Women

MedlinePlus

... FAQs Breadcrumb Home Osteoporosis Osteoporosis and Hispanic Women Osteoporosis and Hispanic Women It is a common misconception ... Are Available? Resources For Your Information What Is Osteoporosis? Osteoporosis is a condition in which the bones ...

Surfing for juvenile idiopathic arthritis: perspectives on quality and content of information on the Internet.

PubMed

Stinson, Jennifer N; Tucker, Lori; Huber, Adam; Harris, Heather; Lin, Carmen; Cohen, Lindsay; Gill, Navreet; Lukas-Bretzler, Jacqueline; Proulx, Laurie; Prowten, David

2009-08-01

To determine the quality and content of English language Internet information about juvenile idiopathic arthritis (JIA) from the perspectives of consumers and healthcare professionals. Key words relevant to JIA were searched across 10 search engines. Quality of information was appraised independently by 2 health professionals, 1 young adult with JIA, and a parent using the DISCERN tool. Concordance of the website content (i.e., accuracy and completeness) with available evidence about the management of JIA was determined. Readability was determined using Flesch-Kincaid grade level and Reading Ease Score. Out of the 3000 Web pages accessed, only 58 unique sites met the inclusion criteria. Of these sites only 16 had DISCERN scores above 50% (indicating fair quality). These sites were then rated by consumers. Most sites targeted parents and none were specifically developed for youth with JIA. The overall quality of website information was fair, with a mean DISCERN quality rating score of 48.92 out of 75 (+/- 6.56, range 34.0-59.5). Overall completeness of sites was 9.07 out of 16 (+/- 2.28, range 5.25-13.25) and accuracy was 3.09 out of 4 (+/- 0.86, range 2-4), indicating a moderate level of accuracy. Average Flesch-Kincaid grade level and Reading Ease Score were 11.48 (+/- 0.74, range 10.1-12.0) and 36.36 (+/- 10.86, range 6.30-48.1), respectively, indicating that the material was difficult to read. Our study highlights the paucity of high quality Internet health information at an appropriate reading level for youth with JIA and their parents.

Acute hepatitis in three patients with systemic juvenile idiopathic arthritis taking interleukin-1 receptor antagonist.

PubMed

Canna, Scott; Frankovich, Jennifer; Higgins, Gloria; Narkewicz, Michael R; Nash, S Russell; Hollister, J Roger; Soep, Jennifer B; Dragone, Leonard L

2009-12-22

We investigated the etiology of acute hepatitis in three children with systemic Juvenile Idiopathic Arthritis (sJIA) taking Interleukin-1 receptor antagonist (IL1RA). Laboratory and clinical data for three children with sJIA diagnosed at ages 13 months to 8 years who developed acute hepatitis during treatment with IL1RA were reviewed for evidence of sJIA flare, infection, macrophage activation syndrome (MAS), malignancy, and drug reaction. In all patients, hepatitis persisted despite cessation of known hepatotoxic drugs and in absence of known infectious triggers, until discontinuation of IL1RA. Liver biopsies had mixed inflammatory infiltrates with associated hepatocellular injury suggestive of an exogenous trigger. At the time of hepatitis, laboratory data and liver biopsies were not characteristic of MAS. In two patients, transaminitis resolved within one week of discontinuing IL1RA, the third improved dramatically in one month. Although sJIA symptoms improved significantly on IL1RA, it appeared that IL1RA contributed to the development of acute hepatitis. Hepatitis possibly occurred as a result of an altered immune response to a typical childhood infection while on IL1RA. Alternatively, hepatitis could have represented an atypical presentation of MAS in patients with sJIA taking IL1RA. Further investigation is warranted to determine how anti-IL1 therapies alter immune responsiveness to exogenous triggers in patients with immune dysfunction such as sJIA. Our patients suggest that close monitoring for hepatic and other toxicities is indicated when treating with IL1RA.

Frequency of radiographic damage and progression in individual joints in children with juvenile idiopathic arthritis.

PubMed

Giancane, Gabriella; Pederzoli, Silvia; Norambuena, Ximena; Ioseliani, Maka; Sato, Juliana; Gallo, Maria Chiara; Negro, Giorgia; Pistorio, Angela; Ruperto, Nicolino; Martini, Alberto; Ravelli, Angelo

2014-01-01

To evaluate the presence and progression of radiographic joint damage, as assessed with the adapted Sharp/van der Heijde score (SHS), in individual joints in the hand and wrist in patients with juvenile idiopathic arthritis (JIA) and to compare progression of damage among different JIA categories. A total of 372 radiographs of both wrists and hands obtained at first observation and at last followup visit (after 1-10 years) in 186 children with polyarticular-course JIA were evaluated. All radiographs were scored using the adapted SHS by 2 independent readers. Radiographic assessment included evaluation of joint space narrowing (JSN) and erosions on baseline and last followup radiographs and of progression of radiographic changes from baseline to last followup radiographs. Both JSN and erosions occurred in all adapted SHS areas. Overall, radiographic damage and progression were more common in the wrist and less common in metacarpophalangeal (MCP) joints. The hamate and capitate areas appeared particularly vulnerable to cartilage loss. Erosions were identified most frequently in the hamate and capitate bones as well as in the second and third metacarpal bases. Patients with extended oligoarthritis were distinctly less susceptible to JSN in hand joints, whereas patients with polyarthritis showed a greater tendency to developing erosions in hand joints. Radiographic joint damage and progression in our patients with JIA were seen most commonly in the wrist and less commonly in MCP joints. The frequency and localization of structural abnormalities differed markedly across disease categories. Copyright © 2014 by the American College of Rheumatology.

Investigation of rheumatoid arthritis susceptibility loci in juvenile idiopathic arthritis confirms high degree of overlap.

PubMed

Hinks, Anne; Cobb, Joanna; Sudman, Marc; Eyre, Stephen; Martin, Paul; Flynn, Edward; Packham, Jonathon; Barton, Anne; Worthington, Jane; Langefeld, Carl D; Glass, David N; Thompson, Susan D; Thomson, Wendy

2012-07-01

Rheumatoid arthritis (RA) shares some similar clinical and pathological features with juvenile idiopathic arthritis (JIA); indeed, the strategy of investigating whether RA susceptibility loci also confer susceptibility to JIA has already proved highly successful in identifying novel JIA loci. A plethora of newly validated RA loci has been reported in the past year. Therefore, the aim of this study was to investigate these single nucleotide polymorphisms (SNP) to determine if they were also associated with JIA. Thirty-four SNP that showed validated association with RA and had not been investigated previously in the UK JIA cohort were genotyped in JIA cases (n=1242), healthy controls (n=4281), and data were extracted for approximately 5380 UK Caucasian controls from the Wellcome Trust Case-Control Consortium 2. Genotype and allele frequencies were compared between cases with JIA and controls using PLINK. A replication cohort of 813 JIA cases and 3058 controls from the USA was available for validation of any significant findings. Thirteen SNP showed significant association (p<0.05) with JIA and for all but one the direction of association was the same as in RA. Of the eight loci that were tested, three showed significant association in the US cohort. A novel JIA susceptibility locus was identified, CD247, which represents another JIA susceptibility gene whose protein product is important in T-cell activation and signalling. The authors have also confirmed association of the PTPN2 and IL2RA genes with JIA, both reaching genome-wide significance in the combined analysis.

Longterm Safety and Efficacy of Adalimumab and Infliximab for Uveitis Associated with Juvenile Idiopathic Arthritis.

PubMed

Cecchin, Vanessa; Zannin, Maria Elisabetta; Ferrari, Daniele; Pontikaki, Irene; Miserocchi, Elisabetta; Paroli, Maria P; Bracaglia, Claudia; Marafon, Denise Pires; Pastore, Serena; Parentin, Fulvio; Simonini, Gabriele; De Libero, Cinzia; Falcini, Fernanda; Petaccia, Antonella; Filocamo, Giovanni; De Marco, Riccardo; La Torre, Francesco; Guerriero, Silvana; Martino, Silvana; Comacchio, Francesco; Muratore, Valentina; Martini, Giorgia; Vittadello, Fabio; Zulian, Francesco

2018-04-15

Anti-TNF-α agents have significantly changed the management of juvenile idiopathic arthritis (JIA). We evaluated the safety and efficacy of adalimumab (ADA) and infliximab (IFX) for the treatment of JIA-associated uveitis in patients treated for ≥ 2 years. Patients with JIA-associated uveitis treated with IFX and ADA were managed by a standardized protocol and data were entered in the ORCHIDEA registry. At baseline, all patients were refractory to standard immunosuppressive treatment or were corticosteroid-dependent. Data recorded every 3 months were uveitis course, number/type of ocular flares and complications, drug-related adverse events (AE), and treatment switch or withdrawal. Data of patients treated for ≥ 2 years were analyzed by descriptive statistics. Up to December 2014, 154 patients with ≥ 24 months followup were included in the study. Fifty-nine patients were treated with IFX and 95 with ADA. Clinical remission, defined as the absence of flares for > 6 months on treatment, was achieved in 69 patients (44.8%), with a better remission rate for ADA (60.0%) as compared to IFX (20.3%; p < 0.001). A significant reduction of flares was observed in all patients without difference between the 2 treatment modalities. The number of new ocular complications decreased in both groups but was lower for ADA (p = 0.015). No serious AE were recorded; 16.4% of patients experienced 35 minor AE and the incidence rate was lower with ADA than with IFX. At the 2-year followup, ADA showed a better efficacy and safety profile than IFX for the treatment of refractory JIA-associated uveitis.

Evidence for Tocilizumab as a Treatment Option in Refractory Uveitis Associated with Juvenile Idiopathic Arthritis.

PubMed

Tappeiner, Christoph; Mesquida, Marina; Adán, Alfredo; Anton, Jordi; Ramanan, Athimalaipet V; Carreno, Ester; Mackensen, Friederike; Kotaniemi, Kaisu; de Boer, Joke H; Bou, Rosa; de Vicuña, Carmen García; Heiligenhaus, Arnd

2016-12-01

To report on experience using the anti-interleukin 6 receptor antibody tocilizumab (TCZ) to treat severe and therapy-refractory uveitis associated with juvenile idiopathic arthritis (JIA). Retrospective data were gathered from patients with JIA receiving TCZ treatment for uveitis. JIA and related uveitis data (disease onset, activity, structural complications, and topical and systemic antiinflammatory treatment) were evaluated at the start of TCZ (baseline) and every 3 months during TCZ therapy. A total of 17 patients (14 women) with active uveitis were included (mean age 15.3 ± 6.9 yrs, mean followup time 8.5 mos). In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, methotrexate (MTX), and other synthetic and biological disease-modifying antirheumatic drugs, including ≥ 1 tumor necrosis factor-α (TNF-α) inhibitor. Uveitis inactivity was achieved in 10 patients after a mean of 5.7 months of TCZ treatment (in 3 of them, it recurred during followup) and persisted in the remaining 7 patients. By using TCZ, systemic corticosteroids or immunosuppressives could be spared in 7 patients. Macular edema was present in 5 patients at baseline and improved in all of them under TCZ treatment. Arthritis was active in 11 patients at the initial and in 6 at the final followup visit. TCZ appears to represent a therapeutic option for severe JIA-associated uveitis that has been refractory to MTX and TNF-α inhibitors in selected patients. The present data indicate that inflammatory macular edema responds well to TCZ in patients with JIA-associated uveitis.

Abatacept in the treatment of severe, longstanding, and refractory uveitis associated with juvenile idiopathic arthritis.

PubMed

Tappeiner, Christoph; Miserocchi, Elisabetta; Bodaghi, Bahram; Kotaniemi, Kaisu; Mackensen, Friederike; Gerloni, Valeria; Quartier, Pierre; Lutz, Thomas; Heiligenhaus, Arnd

2015-04-01

Abatacept (ABA), a selective T cell costimulation modulator that binds to CD80 and CD86 on antigen-presenting cells, was investigated for its antiinflammatory effect in treating severe chronic uveitis associated with juvenile idiopathic arthritis (JIA). Our retrospective study was conducted by members of the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC). Patients with JIA who are receiving ABA treatment for active uveitis were included. In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, immunosuppressives, and at least 1 tumor necrosis factor-α inhibitor. A standardized protocol was used to document uveitis (MIWGUC) and arthritis. Baseline visit and visits at 3, 6, 9, and 12 months before and after ABA start were evaluated. Primary outcome measure was defined as achievement of uveitis inactivity; secondary outcome measures were tapering of corticosteroid and/or immunosuppressive treatment, and occurrence of complications. In all, 21 patients (16 female) with active uveitis (n = 21) and arthritis (n = 18) were included (mean age 11.8 ± 3.6 yrs). In 7 of 18 patients with active arthritis at baseline, inactivity was achieved following ABA treatment. Uveitis inactivity was achieved in 11 patients, but recurred later in 8 of them, and remained active in another 10 cases. Systemic corticosteroids or immunosuppression were tapered in 3 patients, but uveitis recurred in all of them during further followup. Ocular complications secondary to uveitis were present in 17 patients at baseline, while 3 patients developed new ocular complications during followup. A sustained response to ABA was uncommon in patients with severe and refractory uveitis.

Osteoporosis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Riggs, B.L. Melton III, L.J.

This book contains 20 chapters. Some of the titles are: Radiology of asteoporosis; Quantitative computed tomography in assessment of osteoporosis; Nuclear medicine and densitometry; Assessment of bone turnover by histormorphometry in osteoporosis; and The biochemistry of bone.

Cost of biologics in the treatment of juvenile idiopathic arthritis: a factor not to be overlooked.

PubMed

Prince, Femke H M; van Suijlekom-Smit, Lisette W A

2013-08-01

Biologics are a promising treatment option for juvenile idiopathic arthritis (JIA) but drug costs are very high compared to conventional treatment. From a socioeconomic view the additional costs of new interventions should be weighed against their incremental health benefits compared to standard care. Therefore we evaluated data on cost-effectiveness of biologics in JIA. We searched Medline, Embase, and The York Centre for Reviews and Dissemination database for relevant literature. Current data show that biologics are reducing direct and indirect healthcare costs if one excludes the costs of the drug itself. The costs of biologics are more than ten times as high as conventional drug treatment. As a result of limited data, no comparison on cost-effectiveness between biologics could be performed. Although data on long-term cost-effectiveness of biologics are lacking, the expectation is that they will be cost-effective in the long-term. The idea behind this is that biologic treatment should be administered to patients that without these drugs would incur high direct and indirect costs due to continuous severe disease resulting in irreversible disabilities. In our opinion the best cost benefit could be gained if these patients receive biologic treatment introduced early in the disease. This is in order to minimize irreversible damage to the joints and minimize need for long-term biologic therapy by early suppression of the disease. To support these hypotheses future research is needed on long-term cost-effectiveness of all biologics used in JIA.

Investigation of type 1 diabetes and coeliac disease susceptibility loci for association with juvenile idiopathic arthritis.

PubMed

Hinks, Anne; Martin, Paul; Flynn, Edward; Eyre, Steve; Packham, Jon; Barton, Anne; Worthington, Jane; Thomson, Wendy

2010-12-01

There is strong evidence suggesting that juvenile idiopathic arthritis (JIA) shares many susceptibility loci with other autoimmune diseases. To investigate variants robustly associated with type 1 diabetes (T1D) or coeliac disease (CD) for association with JIA. Sixteen single-nucleotide polymorphisms (SNPs) already identified as susceptibility loci for T1D/CD were selected for genotyping in patients with JIA (n=1054) and healthy controls (n=3129). Genotype and allele frequencies were compared using the Cochrane-Armitage trend test implemented in PLINK. One SNP in the LPP gene, rs1464510, showed significant association with JIA (p(trend)=0.002, OR=1.18, 95% CI 1.06 to 1.30). A second SNP, rs653178 in ATXN2, also showed nominal evidence for association with JIA (p(trend)=0.02, OR=1.13, 95% CI 1.02 to 1.25). The SNP, rs17810546, in IL12A showed subtype-specific association with enthesitis-related arthritis (ERA) subtype (p(trend)=0.005, OR=1.88, 95% CI 1.2 to 2.94). Evidence for a novel JIA susceptibility locus, LPP, is presented. Association at the SH2B3/ATXN2 locus, previously reported to be associated with JIA in a US series, also supports this region as contributing to JIA susceptibility. In addition, a subtype-specific association of IL12A with ERA is identified. All findings will require validation in independent JIA cohorts.

Clinical features of children with enthesitis-related juvenile idiopathic arthritis / juvenile spondyloarthritis followed in a French tertiary care pediatric rheumatology centre.

PubMed

Goirand, Maxime; Breton, Sylvain; Chevallier, Frédéric; Duong, Ngoc-Phoi; Uettwiller, Florence; Melki, Isabelle; Mouy, Richard; Wouters, Carine; Bader-Meunier, Brigitte; Job-Deslandre, Chantal; Quartier, Pierre

2018-04-02

Childhood-onset spondyloarthropathies usually start with enthesitis and peripheral arthritis. However, axial disease may develop afterward. Patients are most often classified, following revised (Edmonton 2011) ILAR criteria, as enthesitis-related arthritis, psoriatic arthritis, or unclassified juvenile idiopathic arthritis, particularly in cases of psoriasis in the patient or a first-degree relative. In adults, peripheral spondyloarthritis is classified by ASAS criteria. We retrospectively studied patients with childhood-onset spondyloarthropathies followed for more than one year in our referral centre. We did not exclude patients with a personal or familial history of psoriasis. We included 114 patients followed between January 2008 and December 2015 for a median of 2.5 years (IQR = 2.3). Sixty-nine per-cent of patients fulfilled the revised ILAR classification criteria for enthesitis-related arthritis, and 92% the ASAS criteria for peripheral spondyolarthritis (p <  0.001). Axial disease and sacroiliitis were rare at disease onset. However, they appeared during follow-up in 63% and 47% of cases respectively, after a median disease duration of 2.6 (IC 95% [2.2-4.4]) and 5.3 years (IC 95% [4.1-7.7]), respectively. Multivariable analysis showed that familial history of spondyloarthritis was associated with the presence of sacroiliitis and active disease at the latest follow-up (OR = 3.61 [1.5-8.7], p <  0.01 and 2.98 [1.2-7.3], p = 0.02, respectively). Axial involvement developed in most patients within five years. Revised Edmonton criteria were less sensitive than ASAS criteria to classify patients as having childhood-onset spondyloarthropathies. The main risk factor for both sacroiliitis and persistent active disease was a familial history of spondyloarthritis.

[Postmenopausal osteoporosis].

PubMed

László, Adám

2004-01-04

Due to its incidence and clinical consequences osteoporosis followed by vertebral, hip, and forearm fractures represents an outstanding problem of nowadays' health care. Because of its high mortality rate hip fractures are of special interest. The number of fractures caused by postmenopausal osteoporosis increases with age. Costs of examinations and treatment of women with postmenopausal osteoporosis and fractures are also increasing and represent a significant amount all over the world. Organization of Osteoporosis Centres in Hungary was founded in 1995 and has been since functioning, however, only the one-sixth of osteoporotic patients are treated. Several risk factors are known in the pathogenesis of osteoporosis, first of all the lack of sufficient calcium and vitamin D intake, age, genetic factors, and circumstances known to predispose falling. Estrogen deficiency is the most likely cause of postmenopausal osteoporosis. Osteodensitometry by DEXA is the most important method to evaluate osteoporosis, since decrease in bone mineral density strongly correlates with fracture incidence. Physical, radiologic, and laboratory examination are also required at the first visit and during follow-up. The quantity of bone can hardly be influenced after the 35th year of age, thus prevention of osteoporosis has special significance: appropriate calcium and vitamin D supplementation, weight-bearing sports and physical activity can prevent fractures. According to the results from studies fulfilling the criteria of evidence-based medicine, first choice treatment of osteoporosis involves hormone replacement therapy, bisphosphonates, the tissue specific tibolone, raloxifen and calcitonin. Calcium and vitamin D supplementation are always necessary to be added to any antiporotic treatment. Other combinations of different antiporotic drugs are useless and make the treatment more expensive. Other treatments like massage, physiotherapy, hip-protecting pants, etc. as well as

Development of a national audit tool for juvenile idiopathic arthritis: a BSPAR project funded by the Health Care Quality Improvement Partnership

PubMed Central

McErlane, Flora; Foster, Helen E; Armitt, Gillian; Bailey, Kathryn; Cobb, Joanna; Davidson, Joyce E; Douglas, Sharon; Fell, Andrew; Friswell, Mark; Pilkington, Clarissa; Strike, Helen; Smith, Nicola; Thomson, Wendy; Cleary, Gavin

2018-01-01

Abstract Objective Timely access to holistic multidisciplinary care is the core principle underpinning management of juvenile idiopathic arthritis (JIA). Data collected in national clinical audit programmes fundamentally aim to improve health outcomes of disease, ensuring clinical care is equitable, safe and patient-centred. The aim of this study was to develop a tool for national audit of JIA in the UK. Methods A staged and consultative methodology was used across a broad group of relevant stakeholders to develop a national audit tool, with reference to pre-existing standards of care for JIA. The tool comprises key service delivery quality measures assessed against two aspects of impact, namely disease-related outcome measures and patient/carer reported outcome and experience measures. Results Eleven service-related quality measures were identified, including those that map to current standards for commissioning of JIA clinical services in the UK. The three-variable Juvenile Arthritis Disease Activity Score and presence/absence of sacro-iliitis in patients with enthesitis-related arthritis were identified as the primary disease-related outcome measures, with presence/absence of uveitis a secondary outcome. Novel patient/carer reported outcomes and patient/carer reported experience measures were developed and face validity confirmed by relevant patient/carer groups. Conclusion A tool for national audit of JIA has been developed with the aim of benchmarking current clinical practice and setting future standards and targets for improvement. Staged implementation of this national audit tool should facilitate investigation of variability in levels of care and drive quality improvement. This will require engagement from patients and carers, clinical teams and commissioners of JIA services. PMID:29069424

Development of a national audit tool for juvenile idiopathic arthritis: a BSPAR project funded by the Health Care Quality Improvement Partnership.

PubMed

McErlane, Flora; Foster, Helen E; Armitt, Gillian; Bailey, Kathryn; Cobb, Joanna; Davidson, Joyce E; Douglas, Sharon; Fell, Andrew; Friswell, Mark; Pilkington, Clarissa; Strike, Helen; Smith, Nicola; Thomson, Wendy; Cleary, Gavin

2018-01-01

Timely access to holistic multidisciplinary care is the core principle underpinning management of juvenile idiopathic arthritis (JIA). Data collected in national clinical audit programmes fundamentally aim to improve health outcomes of disease, ensuring clinical care is equitable, safe and patient-centred. The aim of this study was to develop a tool for national audit of JIA in the UK. A staged and consultative methodology was used across a broad group of relevant stakeholders to develop a national audit tool, with reference to pre-existing standards of care for JIA. The tool comprises key service delivery quality measures assessed against two aspects of impact, namely disease-related outcome measures and patient/carer reported outcome and experience measures. Eleven service-related quality measures were identified, including those that map to current standards for commissioning of JIA clinical services in the UK. The three-variable Juvenile Arthritis Disease Activity Score and presence/absence of sacro-iliitis in patients with enthesitis-related arthritis were identified as the primary disease-related outcome measures, with presence/absence of uveitis a secondary outcome. Novel patient/carer reported outcomes and patient/carer reported experience measures were developed and face validity confirmed by relevant patient/carer groups. A tool for national audit of JIA has been developed with the aim of benchmarking current clinical practice and setting future standards and targets for improvement. Staged implementation of this national audit tool should facilitate investigation of variability in levels of care and drive quality improvement. This will require engagement from patients and carers, clinical teams and commissioners of JIA services. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology.

Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis and systemic lupus erythematosus.

PubMed

Aytaç, Selin; Batu, Ezgi Deniz; Ünal, Şule; Bilginer, Yelda; Çetin, Mualla; Tuncer, Murat; Gümrük, Fatma; Özen, Seza

2016-10-01

Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years. More SJIA patients had MAS at disease onset than SLE patients (53.6 vs. 16.7 %). Fever, high C-reactive protein and hyperferritinemia were present in all MAS episodes. Rash was less (p = 0.03), and fatigue was more frequent (p = 0.042) in SLE than SJIA patients. All received corticosteroids. Cyclosporine was given in 74.2 % of SJIA-MAS; 66.7 % of SLE-MAS episodes. Intravenous immunoglobulin, anakinra, or etoposide was administered during 67.7; 41.9; 32.3 % of SJIA-MAS and 33.3; 33.3; 50 % of SLE-MAS episodes, respectively. Plasmapheresis was performed during 41.9 % of SJIA-MAS and 33.3 % of SLE-MAS episodes. The mortality rate was 11.8 % (n = 4;3 SJIA, 1 SLE). Hepatosplenomegaly was more frequent (p = 0.005), and plasmapheresis was performed more frequently (p = 0.021) in the patients who died compared to the cured patients. The median duration between symptom onset and admission to our hospital was longer among the patients who died (16.5 vs. 7 days; p = 0.049). Our patients' characteristics were similar to the reported cases, but our mortality rate is slightly higher probably due to late referral to our center. Early diagnosis and effective treatment are crucial to prevent mortality.

Juvenile idiopathic arthritis in adulthood: fulfilment of classification criteria for adult rheumatic diseases, long-term outcomes and predictors of inactive disease, functional status and damage.

PubMed

Oliveira-Ramos, Filipa; Eusébio, Mónica; M Martins, Fernando; Mourão, Ana Filipa; Furtado, Carolina; Campanilho-Marques, Raquel; Cordeiro, Inês; Ferreira, Joana; Cerqueira, Marcos; Figueira, Ricardo; Brito, Iva; Canhão, Helena; Santos, Maria José; Melo-Gomes, José A; Fonseca, João Eurico

2016-01-01

To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. Patients with JIA registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index-articular (JADI-A) and Juvenile Arthritis Damage Index-extra-articular (JADI-E) damage index and disease activity were analysed. 426 patients were included. Most of patients with systemic JIA fulfilled criteria for Adult Still's disease. 95.6% of the patients with rheumatoid factor (RF)-positive polyarthritis and 57.1% of the patients with RF-negative polyarthritis matched criteria for rheumatoid arthritis (RA). 38.9% of the patients with extended oligoarthritis were classified as RA while 34.8% of the patients with persistent oligoarthritis were classified as spondyloarthritis. Patients with enthesitis-related arthritis fulfilled criteria for spondyloarthritis in 94.7%. Patients with psoriatic arthritis maintained this classification. Patients with inactive disease had lower disease duration, lower diagnosis delay and corticosteroids exposure. Longer disease duration was associated with higher HAQ, JADI-A and JADI-E. Higher JADI-A was also associated with biological treatment and retirement due to JIA disability and higher JADI-E with corticosteroids exposure. Younger age at disease onset was predictive of higher HAQ, JADI-A and JADI-E and decreased the chance of inactive disease. Most of the included patients fulfilled classification criteria for adult rheumatic diseases, maintain active disease and have functional impairment. Younger age at disease onset was predictive of higher disability and decreased the

Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature.

PubMed

Zhang, Pingping; Li, Xiaofeng; Li, Yating; Wang, Jing; Zeng, Huasong; Zeng, Xiaofeng

2015-01-01

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome based on changes in clinical imaging, and it has been reported to mainly occur in adults. However, it has been recently discovered that RPLS is also prevalent in infant patients, particularly in those using glucocorticoids, immunosuppressant medications and cytotoxic drugs. The current study presents a 5-year-old male with a previous diagnosis of systemic-onset juvenile idiopathic arthritis (SoJIA) and macrophage-activation syndrome who developed posterior reversible encephalopathy syndrome during treatment with glucocorticoids, disease-modifying antirheumatic drugs and biological agent (etanercept) therapy. After ~5 days of treatment, the patient made a complete clinical recovery; the magnetic resonance imaging reviewed 2 weeks later showed that the previous hyper-intensity signal had disappeared and the multiple lesions in the brain had been completely absorbed. The case report shows that, conforming to recent literature, SoJIA in infants should be considered a risk factor for developing RPLS. The clinical manifestations of the disease are multiple, but usually reversible, and the patients mostly have a good prognosis. Rapid diagnosis and treatment is essential as early treatment may prevent progression to irreversible brain damage. By increasing the awareness of RPLS, the patient care may improve and further insight may be gained.

Physical Examination Tools Used to Identify Swollen and Tender Lower Limb Joints in Juvenile Idiopathic Arthritis: A Scoping Review.

PubMed

Fellas, Antoni; Singh-Grewal, Davinder; Santos, Derek; Coda, Andrea

2018-01-01

Juvenile idiopathic arthritis (JIA) is the most common form of rheumatic disease in childhood and adolescents, affecting between 16 and 150 per 100,000 young persons below the age of 16. The lower limb is commonly affected in JIA, with joint swelling and tenderness often observed as a result of active synovitis. The objective of this scoping review is to identify the existence of physical examination (PE) tools to identify and record swollen and tender lower limb joints in children with JIA. Two reviewers individually screened the eligibility of titles and abstracts retrieved from the following online databases: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, and CINAHL. Studies that proposed and validated a comprehensive lower limb PE tool were included in this scoping review. After removal of duplicates, 1232 citations were retrieved, in which twelve were identified as potentially eligible. No studies met the set criteria for inclusion. Further research is needed in developing and validating specific PE tools for clinicians such as podiatrists and other allied health professionals involved in the management of pathological lower limb joints in children diagnosed with JIA. These lower limb PE tools may be useful in conjunction with existing disease activity scores to optimise screening of the lower extremity and monitoring the efficacy of targeted interventions.

Postmenopausal osteoporosis.

PubMed

Diab, Dima L; Watts, Nelson B

2013-12-01

The aim of this study is to provide a thorough updated review of the diagnosis and treatment of postmenopausal osteoporosis. There have been several important findings in the field of postmenopausal osteoporosis over the past 1-2 years. Fewer morphometric vertebral fractures were found in women treated for 6 years with zoledronic acid compared with those who stopped treatment after 3 years. Longer duration of bisphosphonate therapy is associated with a higher risk of atypical femur fractures. Combination therapy with teriparatide and denosumab appears to increase bone mineral density to a greater extent than either therapy alone in postmenopausal women at high risk for fracture. There are several novel therapies under investigation for the treatment of osteoporosis, which are in various stages of development. Nonadherence to osteoporosis therapies continues to be a major problem in clinical practice. There are numerous effective pharmacologic treatment options for postmenopausal osteoporosis. Bisphosphonate drug holidays continue to be an area of significant debate.

Orthodontic and dentofacial orthopedic management of juvenile idiopathic arthritis: a systematic review of the literature.

PubMed

von Bremen, J; Ruf, S

2011-08-01

To systematically review the literature published on orthodontic treatment principles in patients with juvenile idiopathic arthritis (JIA). Several electronic databases (PubMed, Medpilot, Web of Science, and DIMDI) and orthodontic and rheumatologic literature were systematically searched for studies published until May 2010. The articles were rated by two independent reviewers and included after three selection steps (title-abstract-full text). Articles had to be studies performed on ≥ 5 patients with a disease onset before the age of 16. The selection process resulted in the inclusion of three publications on dentofacial orthopedics and six on combined surgical orthodontic therapy. The three studies on dentofacial orthopedics aimed to improve the mandibular retrusion by means of removable functional appliances (activator). Whereas these orthodontic approaches comprised relatively large and homogeneous patient samples (14, 22, and 72 subjects, aged 6-16), the surgical studies were basically case series with a large age span of the patients (5-12 subjects, aged 10-44). In these surgical treatment approaches, orthodontics was limited to pre-surgical leveling and post-surgical finishing, while the skeletal discrepancy was treated surgically by a variety of techniques (costochondral grafts, bilateral sagittal spilt osteotomy, Le Fort I, and genioplasty). The treatment goals of both approaches were improvement of esthetics and function and/or pain reduction, and both approaches showed satisfactory results. Because of the heterogeneity of the subject material and the low level of evidence of the papers, it is difficult to draw any conclusions on the orthodontic/dentofacial orthopedic management of JIA. It appears as if removable functional appliances may be beneficial in adolescent patients with JIA. © 2011 John Wiley & Sons A/S.

Fundamental movement skills, physical fitness and physical activity among Australian children with juvenile idiopathic arthritis.

PubMed

Hulsegge, Gerben; Henschke, Nicholas; McKay, Damien; Chaitow, Jeffrey; West, Kerry; Broderick, Carolyn; Singh-Grewal, Davinder

2015-04-01

To describe fundamental movement skills (FMS), physical fitness and level of physical activity among Australian children with juvenile idiopathic arthritis (JIA) and compare this with healthy peers. Children aged 6-16 years with JIA were recruited from hospital rheumatology clinics and private rheumatology rooms in Sydney, Australia. All children attended an assessment day, where FMS were assessed by a senior paediatric physiotherapist, physical fitness was assessed using the multistage 20-metre shuttle run test, and physical activity and physical and psychosocial well-being were assessed with questionnaires. These results were compared with age- and gender-matched peers from the NSW Schools Physical Activity and Nutrition Survey and the Health of Young Victorians Study using logistic regression analysis. Twenty-eight children with JIA participated in this study. There were no differences in the proportion of children who had mastered FMS between children with JIA and their healthy peers (P > 0.05). However, there was a trend for children with JIA to have poorer physical fitness and be less physically active than healthy peers. Parents of children with JIA indicated more physical and psychosocial impairments among their children and themselves compared with parents of healthy children (P < 0.05). This is the first study in Australia to compare FMS, physical activity and fitness in children with JIA and their peers. While older children with JIA appear to have poorer physical fitness and physical activity levels than their peers, there is no difference in FMS. © 2014 The Authors. Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Osteoporosis in Men

MedlinePlus

Osteoporosis in Men A Patient’s Guide In osteoporosis, bones become weak and are more likely to fracture (break). It is a “ ... osteoporosis or osteopenia (mildly low bone mass) are men. The lifetime risk of having a fracture due ...

When a patient suspected with juvenile idiopathic arthritis turns out to be diagnosed with an infectious disease - a review of Lyme arthritis in children.

PubMed

Orczyk, Krzysztof; Świdrowska-Jaros, Joanna; Smolewska, Elżbieta

2017-05-08

The Lyme arthritis is a common manifestation of infection with Borrelia burgdorferi spirochete. Despite its infectious background, the inflammation clinically and histopatologically resembles juvenile idiopathic arthritis. As it affects a considerable number of Lyme disease patients, it should be routinely considered in differential diagnosis. Development of arthritis is partially dependent on spirochetal factors, including the ribosomal spacer type and the sequence of outer surface protein C. Immunological background involves Th1-related response, but IL-17 provides an additional route of developing arthritis. Autoimmune mechanisms may lead to antibiotic-refractory arthritis. The current diagnostic standard is based on a 2-step testing: ELISA screening and immunoblot confirmation. Other suggested methods contain modified two-tier test with C6 ELISA instead of immunoblot. An initial 28-day course of oral antibiotics (doxycycline, cefuroxime axetil or amoxicillin) is a recommended treatment. Severe cases require further anti-inflammatory management. Precise investigation of new diagnostic and therapeutic approaches is advisable.

Long-term safety and efficacy of abatacept in children with juvenile idiopathic arthritis.

PubMed

Ruperto, Nicolino; Lovell, Daniel J; Quartier, Pierre; Paz, Eliana; Rubio-Pérez, Nadina; Silva, Clovis A; Abud-Mendoza, Carlos; Burgos-Vargas, Ruben; Gerloni, Valeria; Melo-Gomes, Jose A; Saad-Magalhães, Claudia; Chavez-Corrales, J; Huemer, Christian; Kivitz, Alan; Blanco, Francisco J; Foeldvari, Ivan; Hofer, Michael; Horneff, Gerd; Huppertz, Hans-Iko; Job-Deslandre, Chantal; Loy, Anna; Minden, Kirsten; Punaro, Marilynn; Nunez, Alejandro Flores; Sigal, Leonard H; Block, Alan J; Nys, Marleen; Martini, Alberto; Giannini, Edward H

2010-06-01

We previously documented that abatacept was effective and safe in patients with juvenile idiopathic arthritis (JIA) who had not previously achieved a satisfactory clinical response with disease-modifying antirheumatic drugs or tumor necrosis factor blockade. Here, we report results from the long-term extension (LTE) phase of that study. This report describes the long-term, open-label extension phase of a double-blind, randomized, controlled withdrawal trial in 190 patients with JIA ages 6-17 years. Children were treated with 10 mg/kg abatacept administered intravenously every 4 weeks, with or without methotrexate. Efficacy results were based on data derived from the 153 patients who entered the open-label LTE phase and reflect >or=21 months (589 days) of treatment. Safety results include all available open-label data as of May 7, 2008. Of the 190 enrolled patients, 153 entered the LTE. By day 589, 90%, 88%, 75%, 57%, and 39% of patients treated with abatacept during the double-blind and LTE phases achieved responses according to the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30), Pedi 50, Pedi 70, Pedi 90, and Pedi 100 criteria for improvement, respectively. Similar response rates were observed by day 589 among patients previously treated with placebo. Among patients who had not achieved an ACR Pedi 30 response at the end of the open-label lead-in phase and who proceeded directly into the LTE, 73%, 64%, 46%, 18%, and 5% achieved ACR Pedi 30, Pedi 50, Pedi 70, Pedi 90, and Pedi 100 responses, respectively, by day 589 of the LTE. No cases of tuberculosis and no malignancies were reported during the LTE. Pneumonia developed in 3 patients, and multiple sclerosis developed in 1 patient. Abatacept provided clinically significant and durable efficacy in patients with JIA, including those who did not initially achieve an ACR Pedi 30 response during the initial 4-month open-label lead-in phase.

[Secondary osteoporosis in gynecology].

PubMed

Taguchi, Y; Gorai, I

1998-06-01

Several diseases and medications are known to induce secondary osteoporosis. Among them, same situations are related to gynecological field. They include Turner's syndrome, anorexia nervosa, ovarian dysfunction, oophorectomy, GnRH agonist therapy, and osteoporosis associated with pregnancy. We briefly describe these secondary osteoporosis in this article as follows. Several studies have found osteoporosis to be a common complication of Turner's syndrome and hormone replacement therapy has been used as a possible management; in anorexic patient, low body weight, prolonged amenorrhea, early onset of anorexia nervosa, and hypercortisolism have been reported to be risks for bone demineralization; since oophorectomy which is a common intervention in gynecology leads osteoporosis, it is important to prevent osteoporosis caused by surgery as well as postmenopausal osteoporosis; GnRH agonist, which induces estrogen deficient state and affect bone mass, is commonly used as a management for endometriosis and leiomyoma of uterus; associated with pregnancy, post-pregnancy spinal osteoporosis and transient osteoporosis of the hip are clinically considered to be important and heparin therapy and magnesium sulfate therapy are commonly employed during pregnancy, affecting calcium homeostasis.

Harnessing interactive technologies to improve health outcomes in juvenile idiopathic arthritis.

PubMed

Coda, Andrea; Sculley, Dean; Santos, Derek; Girones, Xavier; Brosseau, Lucie; Smith, Derek R; Burns, Joshua; Rome, Keith; Munro, Jane; Singh-Grewal, Davinder

2017-05-16

Children and adolescents with Juvenile Idiopathic Arthritis (JIA) typically have reduced physical activity level and impaired aerobic and anaerobic exercise capacity when compared to their non-JIA counterparts. Low intensity exercise regimens appear to be safe in children with JIA and may results in improvements in overall physical function. Poor adherence to paediatric rheumatology treatment may lead to negative clinical outcomes and possibly increased disease activity. This includes symptoms such as pain, fatigue, quality of life, longer term outcomes including joint damage, as well as increase of healthcare associated costs. Low adherence to medications such as methotrexate and biological-drugs remains a significant issue for paediatric rheumatologists, with alarming reports that less than half of the children with JIA are compliant to drug-therapy. The recent advances in interactive technology resulting in a variety of wearable user-friendly smart devices may become a key solution to address important questions in JIA clinical management. Fully understanding the impact that arthritis and treatment complications have upon individual children and their families has long been a challenge for clinicians. Modern interactive technologies can be customised and accessed directly in the hands or wrists of children with JIA. These secured networks could be accessible 'live' at anytime and anywhere by the child, parents and clinicians. Multidisciplinary teams in paediatric rheumatology may benefit from adopting these technologies to better understand domains such as patient biological parameters, symptoms progression, adherence to drug-therapy, quality of life, and participation in physical activities. Most importantly the use of smart devices technologies may also facilitate more timely clinical decisions, improve self-management and parents awareness in the progression of their child's disease. Paediatric rheumatology research could also benefit from the use of these

Relapse rate of uveitis post-methotrexate treatment in juvenile idiopathic arthritis.

PubMed

Kalinina Ayuso, Viera; van de Winkel, Evelyne Leonce; Rothova, Aniki; de Boer, Joke Helena

2011-02-01

To evaluate the efficacy of methotrexate (MTX) and the effect of its withdrawal on relapse rate of uveitis associated with juvenile idiopathic arthritis (JIA). Retrospective case series. Data of 22 pediatric JIA patients who were being treated with MTX for active uveitis were studied retrospectively. Relapse rate after the withdrawal of MTX was established. Anterior chamber (AC) inflammation, topical steroid use during the first year of MTX treatment, and associations of relapses after the withdrawal were evaluated statistically. Duration of MTX treatment and its withdrawal was determined individually in collaboration with a rheumatologist with an intention to continue the treatment for at least 1 year and to withdraw in case of inactivity of uveitis and arthritis. Inactivity of uveitis was defined as the presence of ≤0.5+ cells in the AC. Eighteen patients (18/22; 82%) showed improvement of their uveitis with a significant decrease in activity of AC inflammation after a minimal period of 3 months of MTX treatment. A topical steroid-sparing effect was observed when MTX was administered for a period of 3 to 9 months. MTX was discontinued because of inactive uveitis in 13 patients. In 9 patients (8/13; 69%) a relapse of uveitis was observed after a mean time of 7.5 months (± SD 7.3). Six patients (6/13; 46%) had a relapse within the first year after the withdrawal. Relapse-free survival after withdrawal of MTX was significantly longer in patients who had been treated with MTX for more than 3 years (P = .009), children who were older than 8 years at the moment of withdrawal (P = .003), and patients who had an inactivity of uveitis of longer than 2 years before withdrawal of MTX (P = .033). Longer inactivity under MTX therapy was independently protective for relapses after the withdrawal (hazard ratio = 0.07; 95% confidence interval 0.01-0.86; P = .038), which means that 1-year increase of duration of inactive uveitis before the withdrawal of MTX results in a

Osteoporosis (image)

MedlinePlus

Osteoporosis is a condition characterized by progressive loss of bone density, thinning of bone tissue and increased vulnerability to fractures. Osteoporosis may result from disease, dietary or hormonal deficiency ...

[Determination of 25(OH)D serum levels in children with systemic lupus erythematosus and juvenile idiopathic arthritis].

PubMed

Rosiles, Vanessa Hernández; Salazar, Carolina Duarte; Velazquez, Rocío Maldonado; Ruiz, Rodolfo Rivas; Clark, Patricia

It is well recognized that vitamin D has a direct effect in bone and muscle and has been associated as well with some rheumatologic diseases. Reports in children are scarce. The aim of this study was to determine the concentration level of 25(OH)D in a group of patients with systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA) and compare them with healthy controls. Vitamin D (25(OH)D) was measured with isotope-dilution liquid chromatography-tandem mass spectrometry (ID-LC-MS/MS), PTH with immunoradiometric assay (IRMA), calcium, phosphorus and alkaline phosphatase by colorimetric assay in 37 patients with SLE, 37 patients with JIA and 79 healthy controls. Mean 25(OH)D concentration levels were as follows: SLE 18.9±7.92ng/ml, JIA 21.97±5.55ng/ml and 23.6±3.07ng/ml in healthy controls. There was a significant difference between SLE patients vs. healthy controls (p <0.05); 29.7% of SLE patients, 35.1% of JIA patients and 31.6% of healthy controls had deficient levels of vitamin D. One third of the total sample of children in this study had deficient levels of vitamin D. Patients with SLE presented a significant difference compared with healthy controls. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

Genome-Wide Association Meta-Analysis Reveals Novel Juvenile Idiopathic Arthritis Susceptibility Loci.

PubMed

McIntosh, Laura A; Marion, Miranda C; Sudman, Marc; Comeau, Mary E; Becker, Mara L; Bohnsack, John F; Fingerlin, Tasha E; Griffin, Thomas A; Haas, J Peter; Lovell, Daniel J; Maier, Lisa A; Nigrovic, Peter A; Prahalad, Sampath; Punaro, Marilynn; Rosé, Carlos D; Wallace, Carol A; Wise, Carol A; Moncrieffe, Halima; Howard, Timothy D; Langefeld, Carl D; Thompson, Susan D

2017-11-01

Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease and has a strong genomic component. To date, JIA genetic association studies have had limited sample sizes, used heterogeneous patient populations, or included only candidate regions. The aim of this study was to identify new associations between JIA patients with oligoarticular disease and those with IgM rheumatoid factor (RF)-negative polyarticular disease, which are clinically similar and the most prevalent JIA disease subtypes. Three cohorts comprising 2,751 patients with oligoarticular or RF-negative polyarticular JIA were genotyped using the Affymetrix Genome-Wide SNP Array 6.0 or the Illumina HumanCoreExome-12+ Array. Overall, 15,886 local and out-of-study controls, typed on these platforms or the Illumina HumanOmni2.5, were used for association analyses. High-quality single-nucleotide polymorphisms (SNPs) were used for imputation to 1000 Genomes prior to SNP association analysis. Meta-analysis showed evidence of association (P < 1 × 10 -6 ) at 9 regions: PRR9_LOR (P = 5.12 × 10 -8 ), ILDR1_CD86 (P = 6.73 × 10 -8 ), WDFY4 (P = 1.79 × 10 -7 ), PTH1R (P = 1.87 × 10 -7 ), RNF215 (P = 3.09 × 10 -7 ), AHI1_LINC00271 (P = 3.48 × 10 -7 ), JAK1 (P = 4.18 × 10 -7 ), LINC00951 (P = 5.80 × 10 -7 ), and HBP1 (P = 7.29 × 10 -7 ). Of these, PRR9_LOR, ILDR1_CD86, RNF215, LINC00951, and HBP1 were shown, for the first time, to be autoimmune disease susceptibility loci. Furthermore, associated SNPs included cis expression quantitative trait loci for WDFY4, CCDC12, MTP18, SF3A1, AHI1, COG5, HBP1, and GPR22. This study provides evidence of both unique JIA risk loci and risk loci overlapping between JIA and other autoimmune diseases. These newly associated SNPs are shown to influence gene expression, and their bounding regions tie into molecular pathways of immunologic relevance. Thus, they likely represent regions that contribute to the pathology of oligoarticular JIA and RF

Juvenile idiopathic arthritis activity and function ability: deleterious effects in periodontal disease?

PubMed

Pugliese, Camila; van der Vinne, Roberta T A; Campos, Lucia M A; Guardieiro, Priscila R; Saviolli, Cynthia; Bonfá, Eloisa; Pereira, Rosa M R; Viana, Vilma S; Borba, Eduardo F; Silva, Clovis A

2016-01-01

The impact of juvenile idiopathic arthritis (JIA) in periodontal diseases is controversial probably due to gender and age heterogeneity. We therefore evaluated a homogeneous female post-pubertal JIA population for these conditions. Thirty-five JIA patients and 35 gender/age comparable healthy controls were evaluated according to demographic data, complete periodontal evaluation, fasting lipoproteins, and anti-lipoprotein lipase antibodies. JIA scores, laboratorial tests, X-rays, and treatment were also assessed. Current age was similar in JIA patients and controls (11.90 ± 2.0 vs. 12.50 ± 3.0 years, p = 0.289). Complete periodontal assessments revealed that gingival index, dental plaque, gingival bleeding, and clinical dental attachment indices were alike in JIA patients and controls (p > 0.05), except for gingival enlargement in former group (p < 0.0001). Further analysis of patients with and without gingivitis revealed that cyclosporine use was more often observed in JIA patients with gingivitis (37 vs. 0%, p = 0.01), whereas no differences were evidenced in demographic, JIA scores, inflammatory markers, and lipid profile in both groups. Of note, two parameters of periodontal assessment were correlated with JIA scores [gingival index (GI) and Childhood Health Assessment Questionnaire (CHAQ) (r s  = +0.402, p = 0.020)] and plaque index (PI) and visual analog scale (VAS) physician (r s  = +0.430, p = 0.013). In addition, evaluation of dental assessment demonstrated that JIA activity scores had positive correlation with decayed, missing, and filled teeth (DMF-T) and junvenile athritis disease activity score (JADAS) (r s  = +0.364,p = 0.037), VAS physician (r s  = +0.401,p = 0.021) and VAS patient (r s  = +0.364,p = 0.037). We demonstrated, using rigorous criteria, that periodontal and dental condition in JIA is similar to controls. In spite of that, the finding of a correlation with disease parameters

Osteoporosis and Asian American Women

MedlinePlus

... Breadcrumb Home Osteoporosis Osteoporosis and Asian American Women Osteoporosis and Asian American Women Asian American women are ... Are Available? Resources For Your Information What Is Osteoporosis? Osteoporosis is a condition in which the bones ...

Secondary osteoporosis.

PubMed

Boyle, I T

1993-10-01

Osteoporosis with attendant increased fracture risk is a common complication of many other diseases. Indeed, almost all chronic diseases make some impact on life-style, usually by restricting physical activity and hence reducing the anabolic effect of exercise and gravitational strains on the skeleton. Restricted appetite and modified gastrointestinal tract function is another commonplace finding that has an impact on bone nutrition and synthesis, as on other systems. Sex hormone status is of particular importance for the maintenance of the normal skeleton, and the postmenopausal woman is at particular risk for most causes of secondary osteoporosis. In dealing with secondary osteoporosis in the hypo-oestrogenic woman, the question of giving hormone replacement therapy in addition to other disease-specific therapy should always be considered, as, for example, in a young amenorrhoeic woman with Crohn's disease. Similarly, in hypogonadal men the administration of testosterone is useful for bone conservation. The wider availability of bone densitometry ought to make us more aware of the presence of osteoporosis in the many disease states discussed above. This is particularly important as the life span of such patients is now increased by improved management of the underlying disease process in many instances. Even in steroid-induced osteoporosis--one of the commonest and most severe forms of osteoporosis--we now have some effective therapy in the form of the bisphosphonates and other anti-bone-resorbing drug classes. The possibility of prophylaxis against secondary osteoporosis has therefore become a possibility, although the very long-term effects of such drug regimens are still unknown. In some situations, such as thyrotoxicosis, Cushing's syndrome and immobilization, spontaneous resolution of at least part of the osteoporosis is possible after cure of the underlying problem. The shorter the existence of the basic problem, the more successful the restoration of the

Secondary osteoporosis.

PubMed

Gennari, C; Martini, G; Nuti, R

1998-06-01

Generalized osteoporosis currently represents a heterogeneous group of conditions with many different causes and pathogenetic mechanisms, that often are variably associated. The term "secondary" is applied to all patients with osteoporosis in whom the identifiable causal factors are other than menopause and aging. In this heterogeneous group of conditions, produced by many different pathogenetic mechanisms, a negative bone balance may be variably associated with low, normal or increased bone remodeling states. A consistent group of secondary osteoporosis is related to endocrinological or iatrogenic causes. Exogenous hypercortisolism may be considered an important risk factor for secondary osteoporosis in the community, and probably glucocorticoid-induced osteoporosis is the most common type of secondary osteoporosis. Supraphysiological doses of corticosteroids cause two abnormalities in bone metabolism: a relative increase in bone resorption, and a relative reduction in bone formation. Bone loss, mostly of trabecular bone, with its resultant fractures is the most incapacitating consequence of osteoporosis. The estimated incidence of fractures in patients prescribed corticosteroid is 30% to 50%. Osteoporosis is considered one of the potentially serious side effects of heparin therapy. The occurrence of heparin-induced osteoporosis appeared to be strictly related to the length of treatment (over 4-5 months), and the dosage (15,000 U or more daily), but the pathogenesis is poorly understood. It has been suggested that heparin could cause an increase in bone resorption by increasing the number of differentiated osteoclasts, and by enhancing the activity of individual osteoclasts. Hyperthyroidism is frequently associated with loss of trabecular and cortical bone; the enhanced bone turnover that develops in thyrotoxicosis is characterized by an increase in the number of osteoclasts and resorption sites, and an increase in the ratio of resorptive to formative bone

Male osteoporosis.

PubMed

Rocchietti March, M; Pisani, D; Aliberti, G

2009-12-01

Osteoporosis is a worldwide major public health problem, defined as "a systemic skeletal disease characterized by low bone mass and microarchitectural deterioration of bone tissue with a consequent increase in bone fragility and susceptibility to fractures". Osteoporosis is diagnosed by bone mineral density measurement (T-score of -2.5 or below) also in men. However, most of the studies carried out in the last decade focused on pathogenesis, diagnosis and treatment of osteoporosis in women. In spite of this, recent epidemiological and observational studies have shown that osteoporosis in men is an increasingly important clinical issue. In part because the world population is aging, it is likely that the total number of hip fractures in men in 2025 will be similar to current estimates in women. Furthermore 25-33% of men in some populations will sustain osteoporotic fractures in their lifetime. Nevertheless, male osteoporosis is still underdiagnosed and further studies are required to clarify the pathogenesis and find out the right therapy. Prevention and early diagnosis are, nowadays, the best ways of treatment.

Prevalence of juvenile idiopathic arthritis in children aged 6 to 12 years in Embu das Artes, state of Sao Paulo, Brazil.

PubMed

Yamashita, Edson; Terreri, Maria Teresa R A; Hilário, Maria Odete E; Len, Claudio A

2013-01-01

The aim of the study was to study the prevalence of juvenile idiopathic arthritis (JIA) in school children in the city of Embu das Artes in São Paulo State. 2880 school children from seven public schools, aged between 6 and 12 years, were evaluated (clinical findings) by a pediatric rheumatologist. A board certified Pediatric Rheumatologist evaluated the subjects with suspected inflammatory arthropathy. Children with higher suspicion were referred to a specialized service. One hundred and forty-one children have presented abnormalities on examination of musculoskeletal system, with isolated pain on palpation the most common finding in the first evaluation (60.9%), with improvement in almost all cases in the second examination. Most of the abnormalities were related to recent injuries or congenital malformations. Six children have clinical findings suggestive of chronic arthropathy and were referred to a specialized pediatric rheumatology clinic. Of these, a 12 year-old girl fulfilled the criteria for JIA. The other diagnoses were aseptic necrosis of the hip (P = 1) of and post-trauma synovitis (P = 4). The prevalence of JIA in children aged between 6 and 12 years was 1/2.880 (or 0.34/1.000).

Impaired osteoblast function in osteoporosis: comparison between calcium balance and dynamic histomorphometry.

PubMed

Arlot, M; Edouard, C; Meunier, P J; Neer, R M; Reeve, J

1984-09-01

Osteoblast function was investigated in 27 patients with idiopathic osteoporosis. Transiliac bone biopsy specimens were taken after double labelling with tetracycline, and metabolic calcium balance was studied almost simultaneously. Many of the patients showed poor double labelling of their otherwise unremarkable trabecular osteoid, suggesting impaired formation of bone at many of these surfaces. This phenomenon was not accompanied by increased width of osteoid seams (as seen in osteomalacia), indicating that formation of the matrix and its mineralisation were in equilibrium. For the first time, highly significant positive correlations (p less than 0.01) were found between indices of bone formation, determined by labelling with tetracycline, and calcium balance. Thus some patients with osteoporosis who are rapidly losing bone have low rates of formation of trabecular bone both by individual osteoblasts and in relation to available bone surfaces. As histological indices of bone resorption also independently correlated strongly and inversely (p less than 0.01) with calcium balance the rate of initiation of new basic multicellular units by osteoclastic resorption of trabecular surfaces (or the depth of resorption at these surfaces) also appears to be an important determinant of mineral balance. The mechanisms that regulate the effective life span of mature osteoblasts require further investigation, particularly as some promising treatments that can increase trabecular bone volume in osteoporosis, such as parathyroid peptide hPTH (1-34) and sodium fluoride, must work through a reversal of osteoblastic depression.

Infections in children and adolescents with juvenile idiopathic arthritis and inflammatory bowel disease treated with tumor necrosis factor-α inhibitors: systematic review of the literature.

PubMed

Toussi, Sima S; Pan, Nancy; Walters, Heather M; Walsh, Thomas J

2013-11-01

Tumor necrosis factor alpha (TNF-α) inhibitors are increasingly administered to children and adolescents with juvenile idiopathic arthritis (JIA) and pediatric inflammatory bowel disease (pIBD). Adult studies indicate that TNF-α inhibitors lead to an increased risk of serious infections compared to other disease-modifying antirheumatic drugs. We report herein a systematic literature review detailing the epidemiology and types of infections reported in children with JIA and pIBD treated with TNF-α inhibitors. The most frequently reported infections were mild and characterized as viral in etiology. Severe bacterial and fungal infections also occurred, but were less common and possibly associated with intrinsic risk factors and concurrent immunosuppressive therapy. Few pediatric patients developed Mycobacterium tuberculosis, likely due to effective screening. There were 8 infectious fatalities in children treated with TNF-α inhibitors. Overall, although rare, serious infections occur in immunocompromised children and adolescents with JIA and pIBD receiving TNF-α inhibitors.

Infections in Children and Adolescents With Juvenile Idiopathic Arthritis and Inflammatory Bowel Disease Treated With Tumor Necrosis Factor–α Inhibitors: Systematic Review of the Literature

PubMed Central

Toussi, Sima S.; Pan, Nancy; Walters, Heather M.; Walsh, Thomas J.

2013-01-01

Tumor necrosis factor alpha (TNF-α) inhibitors are increasingly administered to children and adolescents with juvenile idiopathic arthritis (JIA) and pediatric inflammatory bowel disease (pIBD). Adult studies indicate that TNF-α inhibitors lead to an increased risk of serious infections compared to other disease-modifying antirheumatic drugs. We report herein a systematic literature review detailing the epidemiology and types of infections reported in children with JIA and pIBD treated with TNF-α inhibitors. The most frequently reported infections were mild and characterized as viral in etiology. Severe bacterial and fungal infections also occurred, but were less common and possibly associated with intrinsic risk factors and concurrent immunosuppressive therapy. Few pediatric patients developed Mycobacterium tuberculosis, likely due to effective screening. There were 8 infectious fatalities in children treated with TNF-α inhibitors. Overall, although rare, serious infections occur in immunocompromised children and adolescents with JIA and pIBD receiving TNF-α inhibitors. PMID:23899685

Daily Sleep Patterns, Sleep Quality, and Sleep Hygiene Among Parent–Child Dyads of Young Children Newly Diagnosed With Juvenile Idiopathic Arthritis and Typically Developing Children

PubMed Central

Chen, Maida Lynn; Cain, Kevin C.; Ringold, Sarah; Wallace, Carol A.; Ward, Teresa M.

2016-01-01

Objectives Describe daily sleep patterns, sleep quality, and sleep hygiene in 2–5-year-old children newly diagnosed with juvenile idiopathic arthritis (JIA) and their parents in comparison with typically developing (TD) children and parents. Methods Participants (13 JIA, 16 TD parent–child dyads) wore actigraphs for 10 days. Parents completed sleep diaries and sleep hygiene survey. Results Children with JIA had significantly less total sleep time, lower sleep efficiency (SE), and longer naps than TD children. Parents of children with JIA had significantly earlier bedtimes, more wake after sleep onset (WASO) and lower SE than TD parents. Parent–child SE and WASO were interrelated in JIA dyads. Sleep hygiene practices were inconsistent in both groups of children. Conclusions Inadequate amounts of sleep and poor sleep quality were common in parent–child dyads. Early interventions to improve sleep duration and promote sleep hygiene practices may alleviate future sleep problems and improve parent and child well-being. PMID:26994855

Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis.

PubMed

Bennett, Tellen D; Fluchel, Mark; Hersh, Aimee O; Hayward, Kristen N; Hersh, Adam L; Brogan, Thomas V; Srivastava, Rajendu; Stone, Bryan L; Korgenski, E Kent; Mundorff, Michael B; Casper, T Charles; Bratton, Susan L

2012-12-01

To describe patient demographics, interventions, and outcomes in hospitalized children with macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE) or juvenile idiopathic arthritis (JIA). We performed a retrospective cohort study using data recorded in the Pediatric Health Information System (PHIS) database from October 1, 2006 to September 30, 2010. Participants had International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for MAS and either SLE or JIA. The primary outcome was hospital mortality (for the index admission). Secondary outcomes included intensive care unit (ICU) admission, critical care interventions, and medication use. A total of 121 children at 28 children's hospitals met the inclusion criteria, including 19 children with SLE and 102 children with JIA. The index admission mortality rate was 7% (8 of 121 patients). ICU admission (33%), mechanical ventilation (26%), and inotrope/vasopressor therapy (26%) were common. Compared to children with JIA, those with SLE had a similar mortality rate (6% versus 11%, respectively; exact P = 0.6). More patients with SLE than those with JIA received ICU care (63% versus 27%; P = 0.002), received mechanical ventilation (53% versus 21%; P = 0.003), and had cardiovascular dysfunction (47% versus 23% received inotrope/vasopressor therapy; P = 0.02). Children with SLE and those with JIA received cyclosporine at similar rates, but more children with SLE received cyclophosphamide and mycophenolate mofetil, and more children with JIA received interleukin-1 antagonists. Organ system dysfunction is common in children with rheumatic diseases complicated by MAS, and more organ system support is required in children with underlying SLE than in children with JIA. Current treatment of pediatric MAS varies based on the underlying rheumatic disease. Copyright © 2012 by the American College of Rheumatology.

Patient characteristics associated with response to NSAID monotherapy in children with systemic juvenile idiopathic arthritis.

PubMed

Sura, Anjali; Failing, Christopher; Sturza, Julie; Stannard, Jasmine; Riebschleger, Meredith

2018-01-05

Systemic juvenile idiopathic arthritis (sJIA) is an auto-inflammatory disease characterized by fever, arthritis, and ≥1 of rash, generalized lymphadenopathy, hepato/splenomegaly, and serositis. Non-steroidal anti-inflammatory drugs (NSAIDs) are among the initial treatments of sJIA, but there is currently no evidence indicating which children should undergo a trial of NSAID monotherapy and which should not. Our objective is to identify presentation characteristics which are associated with response and lack of response to a trial of NSAID monotherapy. This is a retrospective single-center cohort study of children diagnosed with sJIA from 2000 to 2014. Patient demographics and disease characteristics were investigated to identify predictors of response to NSAID monotherapy. Eighty-seven children were newly diagnosed with sJIA 2000-2014. Thirteen of the 51 children who received NSAID monotherapy achieved clinically inactive disease (CID) without other medications. Age at presentation (≤8 years old), initial joint count (≤5), and C-reactive protein (CRP) (≤13 mg/dL) at diagnosis were associated with achievement of CID on NSAIDs alone. Physicians were less likely to trial NSAID monotherapy if the patient had either serositis or macrophage activation syndrome (MAS) at diagnosis. Ultimate achievement of CID and time to CID were not significantly affected by whether the patient received a trial of NSAID monotherapy. While a subset of children with sJIA can achieve CID with NSAID monotherapy, we recommend against a trial in patients who are >8 years old, with >5 joints involved, or with CRP > 13 mg/dL. Patients who undergo a trial of NSAID monotherapy should follow up within 2-4 weeks to evaluate for possible need for drug escalation. Clinical trials are necessary to confirm these findings.

Distinct expression pattern of IFN-alpha and TNF-alpha in juvenile idiopathic arthritis synovial tissue.

PubMed

Gattorno, M; Chicha, L; Gregorio, A; Ferlito, F; Rossi, F; Jarrossay, D; Lanzavecchia, A; Martini, A; Manz, M G

2007-04-01

Recent laboratory and clinical data suggest that two prototype autoimmune diseases, systemic lupus erythematosus and rheumatoid arthritis are mainly driven by distinct cytokines, interferon (IFN)-alpha and tumour necrosis factor (TNF)-alpha, respectively. We here investigated the presence and characteristics of natural type I IFN-producing cells (IPCs), as well as IFN-alpha and TNF-alpha expression at sites of inflammation in juvenile idiopathic arthritis (JIA). Peripheral blood (PB) and synovial fluid (SF) mononuclear cells (MNCs) (n = 25 each) from JIA patients with active disease were studied. IPCs were identified as BCDA-2(+)CD123(+)HLA-DR(+)CD45RA(+) cells, and dendritic cells (DCs) as CD11c(+)CD14(-/low)lin(-) cells by flow cytometry. IPCs and DCs were analysed for Toll-like receptor-7 and -9 mRNA expression by real-time polymerase chain reaction. IFN-alpha was measured by enzyme-linked immunosorbent assay in serum, SF and in supernatants of influenza virus-infected, cultured IPCs. Synovial tissues of n = 6 additional JIA patients were analysed by immunohistochemistry using mAbs against CD123, IFN-alpha, TNF-alpha, CD3, CD19 and CD138. IPCs were enriched in SF MNCs compared with PB MNCs in all JIA patients. Influenza-induced, but no spontaneous IFN-alpha release was detected from SF IPCs, and serum and SF IFN-alpha levels were not elevated. Nonetheless, in synovial tissue IFN-alpha producing cells accumulated at inflammatory lymph-follicular-like structures, while TNF-alpha producing cells were mostly found at the lining and sublining layers. These data suggest that besides TNF-alpha-expressing cells, IFN-alpha-producing IPCs are involved in initiation, maintenance or regulation of the inflammatory response in JIA.

Measurement of blood calprotectin (MRP-8/MRP-14) levels in patients with juvenile idiopathic arthritis.

PubMed

Bojko, Jaryna

2017-01-01

The aim of the investigation was to compare blood calprotectin (MRP8/14, S100A 8/9) levels in patients with systemic-onset, polyarticular, RF-negative and oligoarticular subtypes of juvenile idiopathic arthritis (JIA), and to explore links between blood calprotectin levels and clinical and laboratory markers of JIA activity. Measurement of calprotectin in blood serum was performed in 160 patients with JIA followed up at Lviv Regional Council Public Institution "Western-Ukrainian Specialised Children's Medical Centre". Seventeen patients with systemic-onset JIA (sJIA) and 49 patients with other JIA subtypes (RF-negative polyarthritis and oligoarthritis) in the active phase of the disease were included in this study. Determination of calprotectin levels in blood serum was performed using EK-MRP8/14 Buhlmann Calprotectin reagents (Buhlmann, Switzerland) by the ELISA method. The results of the investigations showed that blood calprotectin levels were higher in patients with systemic-onset subtype of the disease (median 13,800 ng/ml), and differed significantly from levels in healthy children (median 1,800 ng/ml, p = 0.00002), levels in patients with articular subtypes of JIA (median 2,700 ng/ml, p = 0.000008), and patients with RF-negative polyarthritis (median 3,800 ng/ml, p = 0.003226) and oligoarthritis (median 2,500 ng/ml, p = 0.000009). The highest blood calprotectin levels were found in patients with newly diagnosed sJIA, the median being 32,500 ng/ml (range: 13,800-177,000 ng/ml). Direct correlations were found between blood calprotectin and JADAS 27 activity score ( p = 0.000009), ESR ( p = 0.000079) and CRP ( p = 0.000058). Blood calprotectin level is one of the measures that can be used to confirm the diagnosis of sJIA and to monitor the disease activity and therapy effectiveness.

5-Aminoimidazole-4-carboxamide ribonucleotide-transformylase and inosine-triphosphate-pyrophosphatase genes variants predict remission rate during methotrexate therapy in patients with juvenile idiopathic arthritis.

PubMed

Pastore, Serena; Stocco, Gabriele; Moressa, Valentina; Zandonà, Luigi; Favretto, Diego; Malusà, Noelia; Decorti, Giuliana; Lepore, Loredana; Ventura, Alessandro

2015-04-01

For children with juvenile idiopathic arthritis (JIA) who fail to respond to methotrexate, the delay in identifying the optimal treatment at an early stage of disease can lead to long-term joint damage. Recent studies indicate that relevant variants to predict methotrexate response in JIA are those in 5-aminoimidazole-4-carboxamide ribonucleotide-transformylase (ATIC), inosine-triphosphate-pyrophosphatase (ITPA) and solute-liquid-carrier-19A1 genes. The purpose of the study was, therefore, to explore the role of these candidate genetic factors on methotrexate response in an Italian cohort of children with JIA. Clinical response to methotrexate was evaluated as clinical remission stable for a 6-month period, as ACRPed score and as change in Juvenile Arthritis Disease score. The most relevant SNPs for each gene considered were assayed on patients' DNA. ITPA activity was measured in patients' erythrocytes. Sixty-nine patients with JIA were analyzed: 52.2 % responded to therapy (ACRPed70 score), while 37.7 % reached clinical remission stable for 6 months. ATIC rs2372536 GG genotype was associated with improved clinical remission (adjusted p value = 0.0090). For ITPA, rs1127354 A variant was associated with reduced clinical remission: (adjusted p value = 0.028); this association was present even for patients with wild-type ITPA and low ITPA activity. These preliminary results indicate that genotyping of ATIC rs2372536 and ITPA rs1127354 variants or measuring ITPA activity could be useful to predict methotrexate response in children with JIA after validation by further prospective studies on a larger patient cohort.

Association of HLA-A*02:06 and HLA-DRB1*04:05 with clinical subtypes of juvenile idiopathic arthritis.

PubMed

Yanagimachi, Masakatsu; Miyamae, Takako; Naruto, Takuya; Hara, Takuma; Kikuchi, Masako; Hara, Ryoki; Imagawa, Tomoyuki; Mori, Masaaki; Kaneko, Tetsuji; Goto, Hiroaki; Morita, Satoshi; Mizuki, Nobuhisa; Kimura, Akinori; Yokota, Shumpei

2011-03-01

Juvenile idiopathic arthritis (JIA) is one of the most common forms of pediatric chronic arthritis. JIA is a clinically heterogeneous disease. Therefore, the genetic background of JIA may also be heterogeneous. The aim of this study was to investigate associations between human leukocyte antigen (HLA) and susceptibility to JIA and/or uveitis, which is one of the most devastating complications of JIA. A total of 106 Japanese articular JIA patients (67 with polyarthritis and 39 with oligoarthritis) and 678 healthy controls were genotyped for HLA-A, -B and -DRB1 by PCR-sequence-specific oligonucleotide probe methodology. HLA-A(*)02:06 was the risk factor for JIA accompanied by uveitis after adjustment for clinical factors (corrected P-value < 0.001, odds ratio (OR) 11.7, 95% confidence interval (CI) 3.2-43.0). On the other hand, HLA-DRB1(*)04:05 was associated with polyarticular JIA (corrected P-value < 0.001, OR 2.9, 95% CI 1.7-4.8). We found an association of HLA-A(*)02:06 with susceptibility to JIA accompanied by uveitis, which might be considered a separate clinical JIA entity. We also found an association between HLA-DRB1(*)04:05 and polyarticular JIA. Thus, clinical subtypes of JIA can be classified by the presence of the specific HLA alleles, HLA-A(*)02:06 and DRB1(*)04:05.

Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans for new-onset polyarticular juvenile idiopathic arthritis.

PubMed

Ringold, Sarah; Weiss, Pamela F; Colbert, Robert A; DeWitt, Esi Morgan; Lee, Tzielan; Onel, Karen; Prahalad, Sampath; Schneider, Rayfel; Shenoi, Susan; Vehe, Richard K; Kimura, Yukiko

2014-07-01

There is no standardized approach to the initial treatment of polyarticular juvenile idiopathic arthritis (JIA) among pediatric rheumatologists. Understanding the comparative effectiveness of the diverse therapeutic options available will result in better health outcomes for polyarticular JIA. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans (CTPs) for use in clinical practice to facilitate such studies. A case-based survey was administered to CARRA members to identify the common treatment approaches for new-onset polyarticular JIA. Two face-to-face consensus conferences employed modified nominal group technique to identify treatment strategies, operational case definition, end points, and data elements to be collected. A core workgroup reviewed the relevant literature, refined plans, and developed medication dosing and monitoring recommendations. The initial case-based survey identified significant variability among treatment approaches for new-onset polyarticular JIA. We developed 3 CTPs based on treatment strategies for the first 12 months of therapy, as well as case definitions and clinical and laboratory monitoring schedules. The CTPs include a step-up plan (nonbiologic disease-modifying antirheumatic drug [DMARD] followed by a biologic DMARD), an early combination plan (nonbiologic and biologic DMARD combined within a month of treatment initiation), and a biologic only plan. This approach was approved by 96% of the CARRA JIA Research Committee members attending the 2013 CARRA face-to-face meeting. Three standardized CTPs were developed for new-onset polyarticular JIA. Coupled with data collection at defined intervals, use of these CTPs will enable the study of their comparative effectiveness in an observational setting to optimize initial management of polyarticular JIA. Copyright © 2014 by the American College of Rheumatology.

The RAndomized Placebo Phase Study Of Rilonacept in the Treatment of Systemic Juvenile Idiopathic Arthritis (RAPPORT)

PubMed Central

Ilowite, Norman T.; Prather, Kristi; Lokhnygina, Yuliya; Schanberg, Laura E.; Elder, Melissa; Milojevic, Diana; Verbsky, James W.; Spalding, Steven J.; Kimura, Yukiko; Imundo, Lisa F.; Punaro, Marilynn G.; Sherry, David D.; Tarvin, Stacey E.; Zemel, Lawrence S.; Birmingham, James D.; Gottlieb, Beth S.; Miller, Michael L.; O'Neil, Kathleen; Ruth, Natasha M.; Wallace, Carol A.; Singer, Nora G.; Sandborg, Christy I.

2015-01-01

Background Interleukin-1 plays a pivotal role in in the pathogenesis of systemic juvenile idiopathic arthritis (sJIA). We assessed the efficacy and safety of rilonacept (IL-1 trap), an IL-1 inhibitor, in a randomized, double-blind, placebo-controlled trial. Methods An initial 4-week double-blind placebo phase was incorporated into a 24-week randomized multi-center design, followed by an open label phase. We randomized 71 children with at least 2 active joints 1:1 to 2 arms of the study. Patients in the rilonacept arm received rilonacept (4.4mg/kg loading dose followed by 2.2mg/kg weekly, subcutaneously) from day 0; patients in the placebo arm received placebo for 4 weeks followed by a loading dose of rilonacept at week 4 followed by weekly maintenance doses. The primary endpoint was time to response, using adapted JIA ACR30 response criteria coupled with absence of fever and taper of systemic corticosteroids using pre-specified criteria. Results Time to response was shorter in the rilonacept arm than in the placebo arm (Chi-square 7.235, P=.007). Secondary analysis showed 20/35 (57%) of patients in the rilonacept arm responded at week 4 compared to 9/33 (27%) in the placebo arm (P=.016) using the same response criteria. Exacerbation of sJIA (4) was the most common SAE. More patients in the rilonacept arm had elevated liver transaminases, including more than three times the upper limits of normal, as compared to those in the placebo arm. Adverse events were similar in the two arms of the study. Conclusions Rilonacept was generally well tolerated and demonstrated efficacy in active sJIA. PMID:24839206

Blueberry Improves the Therapeutic Effect of Etanercept on Patients with Juvenile Idiopathic Arthritis: Phase III Study.

PubMed

Zhong, Yingjie; Wang, Ye; Guo, Jun; Chu, Haifeng; Gao, Yong; Pang, Limin

2015-11-01

Juvenile idiopathic arthritis (JIA) is the most common arthritis in the adolescents under the age of 16. Etanercept, an inhibitor of tumor necrosis factor, is often used to treat JIA despite its significant side effects. Homeopathic remedies, such as blueberries, have anti-inflammatory properties with fewer unwanted effects and should be considered as a primary treatment. We aimed to explore the efficacy and safety of combination therapy of blueberry and etanercept for JIA. Two hundred and one JIA patients were selected, and randomly and evenly assigned to three groups: ETA (50 mg of etanercept twice weekly), ETABJ (matched etanercept and 50 ml blueberry juice daily) and ETAPJ (matched etanercept and placebo juice). The severity of JIA was measured using American College of Rheumatology scales (ACR) 20, 50 and 70. The levels of pro-inflammatory cytokines, interleukin-1 (IL1) alpha and IL1 beta, and interleukin-1 receptor antagonist (IL1RA) were measured by qRT-PCR and ELISA. After a 6-month follow-up, the ACR20, ACR50 and ACR70 in an ETABJ group were higher than those in other two groups (P < 0.05), suggesting clinically meaningful improvement in JIA. Meanwhile, the symptoms and side effects were reduced significantly or absent in an ETABJ group, including mental diseases, retrobulbar optic neuritis, gaining weight, infection, cutaneous vasculitis, diarrhea, uveitis and pancytopenia. Blueberries reduced the levels of IL1 alpha and beta, and increased the level of IL1RA. Thus, a combination therapy of blueberry and etanercept can reduce the severity of JIA and should be developed as a new method for JIA therapy.

Intra-articular corticoid injection induces circulating glucocorticoid bioactivity and systemic immune activation in juvenile idiopathic arthritis.

PubMed

Rintamäki, H; Tamm, K; Vaarala, O; Sidoroff, M; Honkanen, V; Raivio, T; Jänne, Oa; Kolho, K-L

2011-01-01

To study the systemic effects of intra-articular (IA) glucocorticoid (GC) injections in juvenile idiopathic arthritis (JIA). The study group comprised 21 JIA patients being treated with IA methylprednisolone [MP (n = 15) or MP plus triamcinolone hexacetonide (THA) (n = 6)] prescribed on clinical indications. The systemic effect of MP was assessed by measuring circulating glucocorticoid bioactivity (GBA) with a recombinant cell transactivation assay 7 and 24 h after the IA injections, and after 2 months. The systemic immunological responses were studied with a novel assay for testing patient serum-induced changes in the secretion of interferon (IFN)-γ and interleukin (IL)-5 from target cells. Administration of IA GC induced serum GBA (p = 0.001) and suppressed circulating cortisol levels (p = 0.002) 7 h after the injection. Serum withdrawn 24 h after the IA injection induced less IL-5 secretion from mitogen-activated target cells when compared with pre-treatment sera (p = 0.036). This decrease in target cell T helper (Th)2 response (IL-5) was MP dose related (r = -0.550, p = 0.018). High IL-5 secretion from target cells prior to the IA injections was associated with good clinical outcome at 2 months, seen as a low number of active (p = 0.044) and restricted joints (p = 0.049). IA GC injections have systemic effects that are reflected in the serum as an immediate elevation of GBA, a decrease of endogenous cortisol as well as a suppressive effect of patient serum on target cell IL-5 secretion. These systemic effects may play a role in the attenuation of disease activity.

Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative.

PubMed

Constantin, Tamas; Foeldvari, Ivan; Anton, Jordi; de Boer, Joke; Czitrom-Guillaume, Severine; Edelsten, Clive; Gepstein, Raz; Heiligenhaus, Arnd; Pilkington, Clarissa A; Simonini, Gabriele; Uziel, Yosef; Vastert, Sebastian J; Wulffraat, Nico M; Haasnoot, Anne-Mieke; Walscheid, Karoline; Pálinkás, Annamária; Pattani, Reshma; Györgyi, Zoltán; Kozma, Richárd; Boom, Victor; Ponyi, Andrea; Ravelli, Angelo; Ramanan, Athimalaipet V

2018-03-28

In 2012, a European initiative called S ingle Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and uveitis is possibly its most devastating extra-articular manifestation. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment practices differ widely, within and between nations. To provide recommendations for the diagnosis and treatment of JIA-associated uveitis. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of nine experienced paediatric rheumatologists and three experts in ophthalmology from Europe. Recommendations derived from a validated systematic literature review were evaluated by an Expert Committee and subsequently discussed at two consensus meetings using nominal group techniques. Recommendations were accepted if >80% agreement was reached (including all three ophthalmologists). In total, 22 recommendations were accepted (with >80% agreement among experts): 3 on diagnosis, 5 on disease activity measurements, 12 on treatment and 2 on future recommendations. The SHARE initiative aims to identify best practices for treatment of patients suffering from JIA-associated uveitis. Within this remit, recommendations for the diagnosis and treatment of JIA-associated uveitis have been formulated by an evidence-informed consensus process to suggest a standard of care for JIA-associated uveitis patients throughout Europe. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Clinical and MRI outcome of cervical spine lesions in children with juvenile idiopathic arthritis treated with anti-TNFα drugs early in disease course.

PubMed

Ključevšek, Damjana; Emeršič, Nina; Toplak, Nataša; Avčin, Tadej

2017-05-15

The purpose of the study was to evaluate the clinical and magnetic resonance imaging (MRI) outcome of cervical spine arthritis in children with juvenile idiopathic arthritis (JIA), who received anti-TNFα early in the course of cervical spine arthritis. Medical charts and imaging of JIA patients with cervical spine involvement were reviewed in this retrospective study. Data, including age at disease onset, JIA type, disease activity, treatment and clinical outcome were collected. Initial and followup MRI examinations of cervical spine were performed according to the hospital protocol to evaluate the presence of inflammation and potential chronic/late changes. Fifteen JIA patients with MRI proved cervical spine inflammation (11 girls, 4 boys, median age 6.3y) were included in the study: 9 had polyarthritis, 3 extended oligoarthritis, 2 persistent oligoarthritis and 1 juvenile psoriatic arthritis. All children were initially treated with high-dose steroids and methotrexate. In addition, 11 patients were treated with anti-TNFα drug within 3 months, and 3 patients within 7 months of cervical spine involvement confirmed by MRI. Mean observation time was 2.9y, mean duration of anti-TNFα treatment was 2.2y. Last MRI showed no active inflammation in 12/15 children, allowing to stop biological treatment in 3 patients, and in 3/15 significant reduction of inflammation. Mild chronic changes were found on MRI in 3 children. Early treatment with anti-TNFα drugs resulted in significantly reduced inflammation or complete remission of cervical spine arthritis proved by MRI, and prevented the development of serious chronic/late changes. Repeated MRI examinations are suggested in the follow-up of JIA patients with cervical spine arthritis.

Pilot study comparing the Childhood Arthritis & Rheumatology Research Alliance (CARRA) systemic Juvenile Idiopathic Arthritis Consensus Treatment Plans.

PubMed

Kimura, Yukiko; Grevich, Sriharsha; Beukelman, Timothy; Morgan, Esi; Nigrovic, Peter A; Mieszkalski, Kelly; Graham, T Brent; Ibarra, Maria; Ilowite, Norman; Klein-Gitelman, Marisa; Onel, Karen; Prahalad, Sampath; Punaro, Marilynn; Ringold, Sarah; Toib, Dana; Van Mater, Heather; Weiss, Jennifer E; Weiss, Pamela F; Schanberg, Laura E

2017-04-11

To assess the feasibility of studying the comparative effectiveness of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans (CTPs) for systemic Juvenile Idiopathic Arthritis (JIA) using an observational registry. Untreated systemic JIA patients enrolled in the CARRA Registry were begun on one of 4 CTPs chosen by the treating physician and patient/family (glucocorticoid [GC] alone; methotrexate [MTX] ± GC; IL1 inhibitor [IL1i] ± GC; IL6 inhibitor [IL6i] ± GC). The primary outcome of clinical inactive disease (CID) without current GC use was assessed at 9 months. clinicaltrials.gov NCT01697254; first registered 9/28/12 (retrospectively enrolled). Thirty patients were enrolled at 13 sites; eight patients were started on a non-biologic CTP (2 GC, 6 MTX) and 22 patients on a biologic CTP (12 IL1i, 10 IL6i) at disease onset. Demographic and disease features were similar between CTP groups. CTP choice appeared to segregate by site preference. CID off GC was achieved by 37% (11 of 30) including 11/22 (50%) starting a biologic CTP compared to 0/8 starting a non-biologic CTP (p = 0.014). There were four serious adverse events: two infections, one appendicitis and one macrophage activation syndrome. The CARRA systemic JIA CTP pilot study demonstrated successful implementation of CTPs using the CARRA registry infrastructure. Having demonstrated feasibility, a larger study using CTP response to better determine the relative effectiveness of treatments for new-onset systemic JIA is now underway.

The effect of positive family history of autoimmunity in juvenile idiopathic arthritis characteristics; a case control study.

PubMed

Khani, Mehdi; Ziaee, Vahid; Moradinejad, Mohamad-Hassan; Parvaneh, Nima

2013-10-01

To compare Juvenile Idiopathic Arthritis (JIA) patients with and without family history of autoimmune disease with respect to clinical features and laboratory data. Sixteen JIA patients with family history of autoimmune disease were identified during study, 32 patients were chosen for comparative group from referred patients to the rheumatology clinic according to the date of referral. Two groups were compared with respect to age of onset, sex, subtype, disease activity, duration of active disease and laboratory variables. The age of onset was significantly lower in JIA patients with family history of autoimmunity (4.7 years vs. 7.0 years; P=0.02), polyarthicular subtype was more frequent in patients with positive family history (50% vs.25%; P=0.04) most of JIA patients with positive family history were in the active phase at the time of study (64% vs 25%; P=0.02) and had a longer duration of active disease (21.0 months vs 12.3 months; P=0.04). Patients with positive family history had more positive ANA (43.5%% vs 12.5%; P=0.01) and also more positive ADA (75% vs 20.8%; P=0.002). Two groups were similar according to sex, and other laboratory variables. JIA patients with family history of autoimmune disease seem to have a more severe disease than patients without such family history, they are younger at the onset, and have mostly poyarthicular subtype. They also have more ANA and ADA positivity. These findings are different from familial JIA case-control studies according to active disease duration, subtype, and ANA positivity.

Association of IRF5 polymorphisms with susceptibility to macrophage activation syndrome in patients with juvenile idiopathic arthritis.

PubMed

Yanagimachi, Masakatsu; Naruto, Takuya; Miyamae, Takako; Hara, Takuma; Kikuchi, Masako; Hara, Ryoki; Imagawa, Tomoyuki; Mori, Masaaki; Sato, Hidenori; Goto, Hiroaki; Yokota, Shumpei

2011-04-01

Systemic-onset juvenile idiopathic arthritis (systemic JIA) and macrophage activation syndrome (MAS), the most devastating complication of systemic JIA, are characterized by abnormal levels of proinflammatory cytokines. Interferon regulatory factor 5 (IRF5) is a member of the IRF family of transcription factors, and acts as a master transcription factor in the activation of genes encoding proinflammatory cytokines. Polymorphisms in the IRF5 gene have been associated with susceptibility to autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. Our aim was to assess associations of IRF5 gene polymorphisms with susceptibility to systemic JIA and MAS. Three IRF5 single-nucleotide polymorphisms (rs729302, rs2004640, and rs2280714) were genotyped using TaqMan assays in 81 patients with systemic JIA (33 with MAS, 48 without) and 190 controls. There were no associations of the IRF5 gene polymorphisms or haplotypes under study with susceptibility to systemic JIA. There was a significant association of the rs2004640 T allele with MAS susceptibility (OR 4.11; 95% CI 1.84, 9.16; p = 0.001). The IRF5 haplotype (rs729302 A, rs2004640 T, and rs2280714 T), which was reported as conferring an increased risk of SLE, was significantly associated with MAS susceptibility in patients with systemic JIA (OR 4.61; 95% CI 1.73, 12.3; p < 0.001). IRF5 gene polymorphism is a genetic factor influencing susceptibility to MAS in patients with systemic JIA, and IRF5 contributes to the pathogenesis of MAS in these patients.

Ultrasound-guided steroid tendon sheath injections in juvenile idiopathic arthritis: a 10-year single-center retrospective study.

PubMed

Peters, Shannon E; Laxer, Ronald M; Connolly, Bairbre L; Parra, Dimitri A

2017-04-11

The aims of this study were to: (a) Identify tendon sheaths most commonly treated with steroid injections in a pediatric patient population with Juvenile Idiopathic Arthritis (JIA); (b) Describe technical aspects of the procedure; (c) Characterize sonographic appearance of tenosynovitis in JIA; (d) Assess agreement between clinical request and sites injected. This was a 10 year single-center retrospective study (May 2006-April 2016) of patients with JIA referred by Rheumatology for ultrasound-guided tendon sheath injections. Patient demographics, clinical referral information, sonographic appearance of the tendon sheaths and technical aspects of the procedure were analyzed. There were 308 procedures of 244 patients (75% female, mean age 9.6 years) who underwent a total of 926 tendon sheath injections. Ankle tendons were most commonly injected (84.9%), specifically the tendon sheaths of tibialis posterior (22.3%), peroneus longus (20%) and brevis (19.7%). The majority of treated sites (91.9%) showed peritendinous fluid and sheath thickening on ultrasound. There were 2 minor intra-procedure complications without sequelae. A good agreement between clinical request and sites injected was observed. Ultrasound-guided tendon sheath injections with steroids are used frequently to treat patients with JIA. It is a safe intervention with a high technical success rate. The ankle region, specifically the medial compartment, is the site most commonly injected in this group of patients. The most common sonographic finding is peritendinous fluid and sheath thickening. These findings might assist clinicians and radiologists to characterize and more effectively manage tenosynovitis in patients with JIA.

Changes in body mass index in children with juvenile idiopathic arthritis treated with tumor necrosis factor inhibitors.

PubMed

Shafferman, Ashley; Fontaine, Kevin R; Cron, Randy Q; Beukelman, Timothy

2014-01-01

To evaluate changes in body mass index (BMI) among cohorts of children with juvenile idiopathic arthritis (JIA) with and without tumor necrosis factor (TNF) inhibitor therapy. We performed a retrospective chart review of children with JIA who newly initiated TNF inhibitor therapy and had at least 1 year of subsequent followup (TNF cohort). We also included children with JIA and at least 1 year of followup without any TNF inhibitor therapy (comparator cohort). Children with systemic arthritis were excluded. Age and sex specific BMI z-scores and their corresponding categories (normal, overweight, obese) were determined. We compared changes from a baseline visit to the last followup visit using t-test for BMI z-scores and chi square and Kruskal-Wallis tests for BMI categories. The TNF cohort had 167 patients; the comparator cohort had 37. The median study followup was 2.8 and 2.2 years, respectively. The cohorts had similar age, sex, race, weight, and height distributions. The TNF cohort had a statistically significant increase in BMI z-score from baseline (+0.15; p = 0.02) that was not significantly different from the increase (+0.09) observed in the comparator cohort (p = 0.5). There was no significant change in the proportions of overweight and obese children in the TNF cohort compared to baseline (p = 0.6) or compared to the change in the comparator cohort (p = 0.2). Over more than 2 years of followup, we did not observe a significant increase in BMI among children with JIA receiving TNF inhibitor compared to those not receiving it.

Health Beliefs about Osteoporosis and Osteoporosis Screening in Older Women and Men

ERIC Educational Resources Information Center

Nayak, Smita; Roberts, Mark S.; Chang, Chung-Chou H.; Greenspan, Susan L.

2010-01-01

Objective: To examine older adults' beliefs about osteoporosis and osteoporosis screening to identify barriers to screening. Design: Cross-sectional mailed survey. Setting: Western Pennsylvania. Methods: Surveys were mailed to 1,830 women and men aged 60 years and older. The survey assessed socio-demographic characteristics, osteoporosis and…

Magnetic Resonance Imaging (MRI) of the Knee as an Outcome Measure in Juvenile Idiopathic Arthritis: An OMERACT Reliability Study on MRI Scales.

PubMed

Hemke, Robert; Tzaribachev, Nikolay; Nusman, Charlotte M; van Rossum, Marion A J; Maas, Mario; Doria, Andrea S

2017-08-01

There is increasing evidence that early therapeutic intervention improves longterm joint outcome in juvenile idiopathic arthritis (JIA). Given the existence of highly effective treatments, there is an urgent need for reliable and accurate measures of disease activity and joint damage in JIA. Our objective was to assess the reliability of 2 magnetic resonance imaging (MRI) scoring methods: the Juvenile Arthritis MRI Scoring (JAMRIS) system and the International Prophylaxis Study Group (IPSG) consensus score, for evaluating disease status of the knee in patients with JIA. Four international readers independently scored an MRI dataset of 25 JIA patients with clinical knee involvement. Synovial thickening, joint effusion, bone marrow changes, cartilage lesions, bone erosions, and subchondral cysts were scored using the JAMRIS and IPSG systems. Further, synovial enhancement, infrapatellar fat pad heterogeneity, tendinopathy, and enthesopathy were scored. Interreader reliability was analyzed by using the generalized κ, ICC, and the smallest detectable difference (SDD). ICC regarding interreader reliability ranged from 0.33 (95% CI 0.12-0.52, SDD = 0.29) for enthesopathy up to 0.95 (95% CI 0.92-0.97, SDD = 3.19) for synovial thickening. Good interreader reliability was found concerning joint effusion (ICC 0.93, 95% CI 0.89-0.95, SDD = 0.51), synovial enhancement (ICC 0.90, 95% CI 0.85-0.94, SDD = 9.85), and bone marrow changes (ICC 0.87, 95% CI 0.80-0.92, SDD = 10.94). Moderate to substantial reliability was found concerning cartilage lesions and bone erosions (ICC 0.55-0.72, SDD 1.41-13.65). The preliminary results are promising for most of the scored JAMRIS and IPSG items. However, further refinement of the scoring system is warranted for unsatisfactorily reliable items such as bone erosions, cartilage lesions, and enthesopathy.

2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups.

PubMed

Lundberg, Ingrid E; Tjärnlund, Anna; Bottai, Matteo; Werth, Victoria P; Pilkington, Clarissa; Visser, Marianne de; Alfredsson, Lars; Amato, Anthony A; Barohn, Richard J; Liang, Matthew H; Singh, Jasvinder A; Aggarwal, Rohit; Arnardottir, Snjolaug; Chinoy, Hector; Cooper, Robert G; Dankó, Katalin; Dimachkie, Mazen M; Feldman, Brian M; Torre, Ignacio Garcia-De La; Gordon, Patrick; Hayashi, Taichi; Katz, James D; Kohsaka, Hitoshi; Lachenbruch, Peter A; Lang, Bianca A; Li, Yuhui; Oddis, Chester V; Olesinska, Marzena; Reed, Ann M; Rutkowska-Sak, Lidia; Sanner, Helga; Selva-O'Callaghan, Albert; Song, Yeong-Wook; Vencovsky, Jiri; Ytterberg, Steven R; Miller, Frederick W; Rider, Lisa G

2017-12-01

To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology and paediatric clinics worldwide. Several statistical methods were used to derive the classification criteria. Based on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived. Each item is assigned a weighted score. The total score corresponds to a probability of having IIM. Subclassification is performed using a classification tree. A probability cut-off of 55%, corresponding to a score of 5.5 (6.7 with muscle biopsy) 'probable IIM', had best sensitivity/specificity (87%/82% without biopsies, 93%/88% with biopsies) and is recommended as a minimum to classify a patient as having IIM. A probability of ≥90%, corresponding to a score of ≥7.5 (≥8.7 with muscle biopsy), corresponds to 'definite IIM'. A probability of <50%, corresponding to a score of <5.3 (<6.5 with muscle biopsy), rules out IIM, leaving a probability of ≥50 to <55% as 'possible IIM'. The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology and paediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of 'definite', 'probable' and 'possible' IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

[New insights of myositis-specific and -associated autoantibodies in juvenile and adult type myositis].

PubMed

Váncsa, Andrea; Dankó, Katalin

2016-07-01

Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179-1184.

Tumor necrosis factor-α -308 A/G gene polymorphism in children with juvenile idiopathic arthritis: relation to disease activity, damage, and functional status.

PubMed

El Gazzar, Iman I; Fathy, Hanan M; Gheita, Tamer A; Nour El-Din, Abeer M; Rasheed, Enas Abdel; Bassyouni, Rasha H; Kenawy, Sanaa A

2017-08-01

The study aims to evaluate the clinical significance of serum levels of tumor necrosis factor alpha (TNF-α) and -308 A/G promoter polymorphism in juvenile idiopathic arthritis (JIA) patients and find any association to the subsets, clinical and laboratory features, disease activity, and damage as well as functional disability. Forty-eight JIA children and 30 controls were included in the present study. Juvenile arthritis disease activity score in 27 joints (JADAS-27) was calculated, juvenile arthritis damage index (JADI) was assessed, and Childhood Health Assessment Questionnaire (CHAQ) measured the functional status. Serum TNF-α was assayed by ELISA and gene (-308) promoter polymorphism was determined by polymerase chain reaction. The 48 JIA children (mean age 11.5 ± 2.8 years) were 13 systemic, 17 oligoarticular, and 18 polyarticular onset. The serum TNF-α was significantly higher in patients (90.4 ± 6.3 ng/ml) compared to control (3.5 ± 2.6 ng/ml) (p < 0.0001) with a tendency to be higher in the polyarticular subtype. All controls had TNF-α -308 GG alleles. The frequency of GG genotype tended to be higher in systemic onset compared to oligoarticular and polyarticular subtypes. The serum TNF-α significantly correlated with JADAS-27 (r = 0.32, p = 0.03) and CHAQ (r = 0.37, p = 0.01) and negatively with the presence of GG alleles (r = -0.48, p = 0.001). The GG alleles were significantly negatively associated with C-reactive protein (r = -0.32, p = 0.03) with a tendency to negatively correlate with JADAS-27, CHAQ, and JADI-extrarticular (r = -0.28, p = 0.06; r = -0.25, p = 0.09 and r = -0.25, p = 0.09, respectively). There is evidence of a possible influence of the -308 SNP promoter position on the production of TNF-α, the severity of JIA which may consequently influence the response to anti-TNF-α treatment.

Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

PubMed

Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

2010-09-01

To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

The CARRA Registry

ClinicalTrials.gov

2015-11-16

Juvenile Idiopathic Arthritis; Systemic Lupus Erythematosus; Mixed Connective Tissue Disease; Juvenile Ankylosing Spondylitis; Juvenile Dermatomyositis; Localized Scleroderma; Systemic Sclerosis; Vasculitis; Sarcoid; Fibromyalgia, Primary; Auto-inflammatory Disease; Idiopathic Uveitis Idiopathic

Transitional care in clinical networks for young people with juvenile idiopathic arthritis: current situation and challenges.

PubMed

Cruikshank, Mary; Foster, Helen E; Stewart, Jane; Davidson, Joyce E; Rapley, Tim

2016-04-01

Clinical networks for paediatric and adolescent rheumatology are evolving, and their effect and role in the transition process between paediatric and adult services are unknown. We therefore explored the experiences of those involved to try and understand this further. Health professionals, young people with juvenile idiopathic arthritis and their families were recruited via five national health service paediatric and adolescent rheumatology specialist centres and networks across the UK. Seventy participants took part in focus groups and one-to-one interviews. Data was analysed using coding, memoing and mapping techniques to identify features of transitional services across the sector. Variation and inequities in transitional care exist. Although transition services in networks are evolving, development has lagged behind other areas with network establishment focusing more on access to paediatric rheumatology multidisciplinary teams. Challenges include workforce shortfalls, differences in service priorities, standards and healthcare infrastructures, and managing the legacy of historic encounters. Providing equitable high-quality clinically effective services for transition across the UK has a long way to go. There is a call from within the sector for more protected time, staff and resources to develop transition roles and services, as well as streamlining of local referral pathways between paediatric and adult healthcare services. In addition, there is a need to support professionals in developing their understanding of transitional care in clinical networks, particularly around service design, organisational change and the interpersonal skills required for collaborative working. Key messages • Transitional care in clinical networks requires collaborative working and an effective interface with paediatric and adult rheumatology.• Professional centrism and historic encounters may affect collaborative relationships within clinical networks.• Education

Ocular morbidities of juvenile idiopathic arthritis-associated uveitis in adulthood: results from a tertiary center study.

PubMed

Oray, Merih; Khachatryan, Naira; Ebrahimiadib, Nazanin; Abu Samra, Khawla; Lee, Stacey; Foster, C Stephen

2016-09-01

To describe the clinical and visual outcomes of juvenile idiopathic arthritis (JIA)-associated uveitis in adults and to examine risk factors for ongoing inflammation in adulthood. Medical records were reviewed for patients with JIA-associated uveitis who were >16 years old at the final visit (the last visit prior to data collection). In total, 135 eyes of 77 patients (70 female, 7 male) were included. The mean age of patients at the final visit was 29.72 ± 11.27 years. The number of eyes with visual acuity of ≤20/50 and ≤20/200 at the final visit was 37 (28 %) and 20 (15 %), respectively; at least one ocular complication was present in 72 % of eyes. Band keratopathy was the most frequent complication (42 %), followed by cataract (25 %), posterior synechiae (22 %), maculopathy (22 %), ocular hypertension (13 %), and hypotony (5 %). At the final visit, patients who were >16 years of age at presentation to the Massachusetts Eye Research and Surgery Institution had more ocular complications and a greater degree of vision loss than patients who were ≤16 years of age. Ongoing inflammation at the final visit was noted in 40 patients (52 %). The presence of posterior synechiae, hypotony, cataract at presentation, and a history of cataract surgery prior to presentation were predictive of ongoing inflammation in adulthood in univariate analysis. The presence of hypotony and posterior synechiae at the initial visit were predictive factors in multivariate analysis. JIA-associated uveitis may be associated with ongoing inflammation, ocular complications, and severe visual impairment in adulthood. The presence of posterior synechiae and hypotony at the initial visit is predictive of ongoing inflammation.

Effects of Pilates exercises on health-related quality of life in individuals with juvenile idiopathic arthritis.

PubMed

Mendonça, Tânia M; Terreri, Maria T; Silva, Carlos H; Neto, Morun Bernardino; Pinto, Rogério M; Natour, Jamil; Len, Claudio A

2013-11-01

To determine the effects of Pilates exercises on health-related quality of life (HRQOL) in individuals with juvenile idiopathic arthritis (JIA). Randomized, prospective, single-blind trial. Outpatient clinic of pediatric rheumatology and the rehabilitation department. Children (N=50) with JIA according to the International League of Associations for Rheumatology criteria. Participants were randomly assigned into 2 groups. In group I (n=25), the participants were given a conventional exercise program for 6 months. Patients in group II (n=25) participated in a Pilates exercise program for 6 months. The primary outcome measure was HRQOL, as measured by the Pediatric Quality of Life Inventory version 4.0 (PedsQL 4.0). The secondary outcome measures provided an estimate of the clinical relevance of the primary outcome results and included joint pain intensity (according to a 10-cm visual analog scale), disability (according to the Childhood Health Assessment Questionnaire), joint status (using the Pediatric Escola Paulista de Medicina Range of Motion Scale), and the total PedsQL 4.0 score. All participants completed the study. The scores of the PedsQL 4.0 differed significantly between groups, indicating that Pilates exercises increased these scores when compared with the conventional exercise program. Group II participants showed significant improvements in the 10-cm visual analog scale-joint pain, Childhood Health Assessment Questionnaire, and Pediatric Escola Paulista de Medicina Range of Motion Scale. The use of Pilates exercises had a positive physical and psychosocial impact on HRQOL in individuals with JIA. Future multicenter studies with a follow-up beyond the period of treatment using more objective parameters will be useful to support the results of the present study. Copyright © 2013 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Protective role of HLA-DRB1*11 against juvenile idiopathic arthritis living in North Eastern Iran.

PubMed

Rezaieyazdi, Zahra; Kochakzadeh, Morteza; Hatef, Mohammad Reza; Esmaily, Habibollah; Malek, Abdolreza; Valizadeh, Narges; Tabaei, Samira; Rafatpanah, Houshang

2018-06-01

Juvenile idiopathic arthritis (JIA) is one of the most common chronic rheumatic diseases in children. The complex nature of this immune-mediated disease owes itself to several predisposing genes and environmental factors affecting its pathogenesis. Conducted in Iran, this study was originally intended to investigate every possible association between HLA DRB1 alleles and a susceptibility to JIA. In this case-control study, 45 patients with a definite diagnosis of JIA based on International League against Rheumatism (ILAR) criteria were compared against 46 healthy controls. DNA samples taken from both groups were analyzed using PCR-sequence specific primers (PCR-SSP) method. Data analysis including parametric and nonparametric test and multivariate analysis was undertaken using the SPSS 11.5 software. A P-value< 0.05 was regarded as statistically significant. Mean ages in case group and healthy controls were 14.64±6.21 and 13.73±6.39, respectively with no significant difference between the two groups ( P =0.515). Sex difference between JIA group and healthy controls was also not significant ( P =0.068). The frequency of HLA-DRB1*01 was found the most frequent HLA-RB1 in our patients (33.3%). No significant statistical correlation between various HLA-DRB1 alleles and clinical subtypes of the disease could be established from the data. HLA-DRB1*11 was shown to raise protection to JIA ( P =0.035, OR=2.755, 95% CI=0.963-8.055) in northeastern Iran. In addition, we found that HLA-RB1*09 is nominally associated with an increased risk of JIA ( P =0.56, OR=2, 05, 95% CI=0.18-23.63). HLA-DRB1*11 was shown to raise protection to JIA in northeastern Iran. The disparity of findings in other ethnicities prompts further investigations with larger sample sizes.

Fever as an Initial Manifestation of Enthesitis-Related Arthritis Subtype of Juvenile Idiopathic Arthritis: Retrospective Study

PubMed Central

Guo, Ruru; Cao, Lanfang; Kong, Xianming; Liu, Xuesong; Xue, Haiyan; Shen, Lijuan; Li, Xiaoli

2015-01-01

Objective We wished to determine the prevalence of fever as one of the first symptoms of the enthesitis-related arthritis (ERA) subtype of juvenile idiopathic arthritis. Also, we wished to ascertain if ERA patients with fever at disease onset differed from those without fever. Methods Consecutive cases of ERA were diagnosed and followed in a retrospective observational study from 1998 to 2013. Information about clinical/laboratory data, medications, magnetic resonance imaging (MRI), and disease activity during the study period was also recorded. Results A total of 146 consecutive ERA patients were assessed. Among them, 52 patients (35.6%) had fever as one of the first symptoms at disease onset. Compared with ERA patients without fever at disease onset, patients with fever had significantly more painful joints (3.5 vs. 2.8), more swollen joints (1.1 vs. 0.8), and more enthesitis (1.0 vs. 0.4) (p<0.05 for all comparisons). Patients with fever had significantly higher mean values of erythrocyte sedimentation rate, C-reactive protein, platelet count, and child health assessment questionnaire (CHAQ) scores (40.8 vs. 26.4 mm/h; 20.7 vs. 9.7 mg/dL; 353.2×109/L vs. 275.6×109/L; 1.0 vs. 0.8, respectively; all p<0.05). During two-year follow-up, CHAQ score, number of flares, as well as the number of patients treated with oral non-steroidal anti-inflammatory drugs, corticosteroids and combination therapy with disease-modifying anti-rheumatic drugs, were significantly higher in ERA patients with fever. Conclusions Fever was a frequent manifestation of ERA. ERA patients with fever had more active disease at disease onset and poorer outcomes than ERA patients without fever. PMID:26030261

What Is Osteoporosis?

MedlinePlus

... Series of Publications for the Public What Is Osteoporosis? Fast Facts: An Easy-to-Read Series of ... in the hip, spine, and wrist. Who Gets Osteoporosis? In the United States, millions of people either ...

Decreased bone density in carriers and patients of an Israeli family with the osteoporosis-pseudoglioma syndrome.

PubMed

Lev, Dorit; Binson, Inga; Foldes, A Joseph; Watemberg, Nathan; Lerman-Sagie, Tally

2003-06-01

The osteoporosis-pseudoglioma syndrome is a rare autosomal recessive disorder characterized by severe juvenile-onset osteoporosis and congenital or early-onset blindness. Other manifestations include muscular hypotonia, ligamentous laxity, mild mental retardation and seizures. The gene responsible was recently identified to be the low density lipoprotein receptor-related family member LRP5 on chromosome 11q11-12. To measure bone density in two siblings with the OPPG syndrome as well as in their family members (parents and siblings). Bone mineral density was determined in the lumbar spine (antero-posterior), femoral neck, two-thirds distal forearm (> 95% cortical bone) and ultradistal forearm (predominantly trabecular bone) by dual-energy X-ray absorptiometry. The studies revealed osteoporotic changes both in the patients and the carriers. The findings demonstrate that OPPG carriers have reduced bone mass, which is a risk factor for development of early osteoporotic changes.

Circulating complexes between tumour necrosis factor-alpha and etanercept predict long-term efficacy of etanercept in juvenile idiopathic arthritis.

PubMed

Kahn, Robin; Berthold, Elisabet; Gullstrand, Birgitta; Schmidt, Tobias; Kahn, Fredrik; Geborek, Pierre; Saxne, Tore; Bengtsson, Anders A; Månsson, Bengt

2016-04-01

The relationship between tumour necrosis factor-alpha (TNF-α) and drug survival had not been studied in juvenile idiopathic arthritis (JIA), and there were no laboratory tests to predict the long-term efficacy of biological drugs for JIA. We studied whether serum levels of TNF-α, free or bound to etanercept, could predict long-term efficacy of etanercept in children with JIA. We included 41 biologic-naïve patients with JIA who started treatment with etanercept at Skåne University Hospital between 1999 and 2010. Serum taken at the start of treatment and at the six-week follow-up were analysed for TNF-α and the long-term efficacy of etanercept was assessed using the drug survival time. Levels of TNF-α increased significantly at the six-week follow-up, and this was almost exclusively comprised of TNF-α in complex with etanercept. The increase in TNF-α showed a dose-dependent correlation to long-term drug survival (p < 0.01). Increasing levels of circulating TNF-α at treatment initiation predicted long-term efficacy of etanercept in children with JIA, which may have been due to different pathophysiological mechanisms of inflammation. Our result may provide a helpful clinical tool, as high levels of circulating TNF-α/etanercept complexes could be used as a marker for the long-term efficacy of etanercept. ©2015 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.

Orofacial symptoms related to temporomandibular joint arthritis in juvenile idiopathic arthritis: smallest detectable difference in self-reported pain intensity.

PubMed

Stoustrup, Peter; Kristensen, Kasper D; Verna, Carlalberta; Küseler, Annelise; Herlin, Troels; Pedersen, Thomas K

2012-12-01

Temporomandibular joint (TMJ) inflammation in patients with juvenile idiopathic arthritis (JIA) may lead to mandibular growth disturbances and interfere with optimal joint and muscle function. Orofacial symptoms are common clinical findings in relation to TMJ arthritis in adolescence. Knowledge about their clinical manifestation is important for TMJ arthritis diagnosis, treatment choice, and outcome evaluation. The aim of our prospective observational study was to evaluate and describe the frequency, the main complaints, and the localization of TMJ arthritis-related orofacial symptoms. The smallest detectable differences (SDD) for minimal, average, and maximal pain were estimated. Thirty-three patients with JIA and arthritis-related orofacial symptoms in relation to 55 affected TMJ were included in our questionnaire study (mean age 14.11 yrs). Calculation of the SDD was based on a duplicate assessment 45 min after the first questionnaire was completed. The majority of the patients had common orofacial symptoms during mastication and maximal mouth opening procedures. Persistent orofacial symptoms were rare. The TMJ area in combination with the masseter muscle region was the orofacial region where symptoms were most common. The SDD for minimal, average, and maximal pain were between 10 and 14 mm on a visual analog scale. Our study offers new knowledge about TMJ arthritis-related orofacial symptoms that may aid diagnosis and clinical decision-making. We suggest that TMJ arthritis-related orofacial symptoms could be understood as products of the primary TMJ inflammation in combination with secondary myogenic and functional issues.

The Effect of Positive Family History of Autoimmunity in Juvenile Idiopathic Arthritis Characteristics; a Case Control Study

PubMed Central

Khani, Mehdi; Ziaee, Vahid; Moradinejad, Mohamad-Hassan; Parvaneh, Nima

2013-01-01

Objective To compare Juvenile Idiopathic Arthritis (JIA) patients with and without family history of autoimmune disease with respect to clinical features and laboratory data. Methods Sixteen JIA patients with family history of autoimmune disease were identified during study, 32 patients were chosen for comparative group from referred patients to the rheumatology clinic according to the date of referral. Two groups were compared with respect to age of onset, sex, subtype, disease activity, duration of active disease and laboratory variables. Findings The age of onset was significantly lower in JIA patients with family history of autoimmunity (4.7 years vs. 7.0 years; P=0.02), polyarthicular subtype was more frequent in patients with positive family history (50% vs.25%; P=0.04) most of JIA patients with positive family history were in the active phase at the time of study (64% vs 25%; P=0.02) and had a longer duration of active disease (21.0 months vs 12.3 months; P=0.04). Patients with positive family history had more positive ANA (43.5%% vs 12.5%; P=0.01) and also more positive ADA (75% vs 20.8%; P=0.002). Two groups were similar according to sex, and other laboratory variables. Conclusion JIA patients with family history of autoimmune disease seem to have a more severe disease than patients without such family history, they are younger at the onset, and have mostly poyarthicular subtype. They also have more ANA and ADA positivity. These findings are different from familial JIA case-control studies according to active disease duration, subtype, and ANA positivity. PMID:24800019

Social/economic costs and health-related quality of life in patients with juvenile idiopathic arthritis in Europe.

PubMed

Kuhlmann, A; Schmidt, T; Treskova, M; López-Bastida, J; Linertová, R; Oliva-Moreno, J; Serrano-Aguilar, P; Posada-de-la-Paz, M; Kanavos, P; Taruscio, D; Schieppati, A; Iskrov, G; Péntek, M; Delgado, C; von der Schulenburg, J M; Persson, U; Chevreul, K; Fattore, G

2016-04-01

The aim of this study was to determine the economic burden from a societal perspective and the health-related quality of life (HRQOL) of patients with juvenile idiopathic arthritis (JIA) in Europe. We conducted a cross-sectional study of patients with JIA from Germany, Italy, Spain, France, the United Kingdom, Bulgaria, and Sweden. Data on demographic characteristics, healthcare resource utilization, informal care, labor productivity losses, and HRQOL were collected from the questionnaires completed by patients or their caregivers. HRQOL was measured with the EuroQol 5-domain (EQ-5D-5L) questionnaire. A total of 162 patients (67 Germany, 34 Sweden, 33 Italy, 23 United Kingdom, 4 France, and 1 Bulgaria) completed the questionnaire. Excluding Bulgarian results, due to small sample size, country-specific annual health care costs ranged from €18,913 to €36,396 (reference year: 2012). Estimated direct healthcare costs ranged from €11,068 to €22,138; direct non-healthcare costs ranged from €7837 to €14,155 and labor productivity losses ranged from €0 to €8715. Costs are also shown to differ between children and adults. The mean EQ-5D index score for JIA patients was estimated at between 0.44 and 0.88, and the mean EQ-5D visual analogue scale score was estimated at between 62 and 79. JIA patients incur considerable societal costs and experience substantial deterioration in HRQOL in some countries. Compared with previous studies, our results show a remarkable increase in annual healthcare costs for JIA patients. Reasons for the increase are the inclusion of non-professional caregiver costs, a wider use of biologics, and longer hospital stays.

Limited value of cyclosporine A for the treatment of patients with uveitis associated with juvenile idiopathic arthritis.

PubMed

Tappeiner, C; Roesel, M; Heinz, C; Michels, H; Ganser, G; Heiligenhaus, A

2009-05-01

Juvenile idiopathic arthritis (JIA) is often associated with severe chronic anterior uveitis (CAU), and immunosuppressive therapy may be required. In this study, the value of cyclosporine A (CsA) as monotherapy or as combination therapy for treating uveitis was studied in a large cohort of JIA children. Multicentre retrospective study including 82 JIA children (girls n=60) suffering from unilateral or bilateral (n=55) CAU. The indication for CsA was active uveitis, although patients were on topical or systemic corticosteroids, MTX, or other immunosuppressive drugs. Inactivity of uveitis during the entire treatment period (mean 3.9 years) was obtained with CsA monotherapy in 6 of 25 (24%) patients, but more often when CsA was combined with the immunosuppressives (35/72 patients; 48.6%, P=0.037), or MTX (18/37 patients, 48.6%, P=0.065), which had already been given. With CsA (mean dosage 2.9 mg/kg), systemic immunosuppressive drugs and steroids could be reduced by >or=50% (n=19) or topical steroids reduced to

American College of Rheumatology provisional criteria for defining clinical inactive disease in select categories of juvenile idiopathic arthritis.

PubMed

Wallace, Carol A; Giannini, Edward H; Huang, Bin; Itert, Lukasz; Ruperto, Nicolino

2011-07-01

To prospectively validate the preliminary criteria for clinical inactive disease (CID) in patients with select categories of juvenile idiopathic arthritis (JIA). We used the process for development of classification and response criteria recommended by the American College of Rheumatology Quality of Care Committee. Patient-visit profiles were extracted from the phase III randomized controlled trial of infliximab in polyarticular-course JIA (i.e., patients considered to resemble those with select categories of JIA) and sent to an international group of expert physician raters. Using the physician ratings as the gold standard, the sensitivity and specificity were calculated using the preliminary criteria. Modifications to the criteria were made, and these were sent to a larger group of pediatric rheumatologists to determine quantitative, face, and content validity. Variables weighted heaviest by physicians when making their judgment were the number of joints with active arthritis, erythrocyte sedimentation rate (ESR), physician's global assessment, and duration of morning stiffness. Three modifications were made: the definition of uveitis, the definition of abnormal ESR, and the addition of morning stiffness. These changes did not alter the accuracy of the preliminary set. The modified criteria, termed the "criteria for CID in select categories of JIA," have excellent feasibility and face, content, criterion, and discriminant validity to detect CID in select categories of JIA. The small changes made to the preliminary criteria set did not alter the area under the receiver operating characteristic curve (0.954) or accuracy (91%), but have increased face and content validity. Copyright © 2011 by the American College of Rheumatology.

Triamcinolone acetonide and hexacetonide intra-articular treatment of symmetrical joints in juvenile idiopathic arthritis: a double-blind trial.

PubMed

Zulian, F; Martini, G; Gobber, D; Plebani, M; Zacchello, F; Manners, P

2004-10-01

Pharmacokinetic studies have shown that the biological effect of triamcinolone acetonide (TA) is equivalent to that of triamcinolone hexacetonide (TH), if used at double the dosage. In this study we compared the efficacy of intra-articular TA at a dose twice that of TH in symmetrically involved joints, in children with juvenile idiopathic arthritis (JIA). Children with active arthritis and a similar degree of inflammation in two symmetrical joints were enrolled in the study. The symmetry was assessed by both clinical examination and synovial fluid analysis. The dose given was 1 mg/kg up to 40 mg of TH or 2.0 mg/kg up to 80 mg of TA. The identity of injected compound was blinded to the patient and to the physician. Thirty-seven patients, 30 female, seven male, with JIA, entered the study. A total of 86 joints were injected. Twenty-one (53.8%) of the joints injected with TA relapsed first compared with only six (15.4%) of the joints injected with TH. In three (7.7%) relapse occurred simultaneously. Nine (23%) were still in remission after 24-month follow-up. The percentage of joints with lasting remission was higher with TH than with TA (80 vs 47.5% after 12 months and 63.6 vs 32.4% after 24 months, respectively; log rank test P = 0.003). Even when TA is given at higher doses, TH is more effective and should be considered the drug of choice for intra-articular treatment of JIA.

A randomised controlled trial of the clinical effectiveness, safety and cost-effectiveness of adalimumab in combination with methotrexate for the treatment of juvenile idiopathic arthritis associated uveitis (SYCAMORE Trial).

PubMed

Ramanan, Athimalaipet V; Dick, Andrew D; Benton, Diana; Compeyrot-Lacassagne, Sandrine; Dawoud, Dalia; Hardwick, Ben; Hickey, Helen; Hughes, Dyfrig; Jones, Ashley; Woo, Patricia; Edelsten, Clive; Beresford, Michael W

2014-01-09

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Children with JIA are at risk of inflammation of the uvea in the eye (uveitis). Overall, 20% to 25% of paediatric uveitis is associated with JIA. Major risk factors for development of uveitis in JIA are oligoarticular pattern of arthritis, an age at onset of arthritis of less than seven years of age, and antinuclear antibody positivity. In the initial stages of mild to moderate inflammation the uveitis is asymptomatic. This has led to current practice of screening all children with JIA for uveitis. Approximately 12% to 38% of patients with JIA develop uveitis in seven years following onset of arthritis. In 30% to 50% of children with JIA-associated uveitis structural complications are present at diagnosis. Furthermore about 50% to 75% of those with severe uveitis will eventually develop visual impairment secondary to ocular complications such as cataract and glaucoma. Defining the severity of inflammation and structural complications in uveitis patients is now possible following Standardised Uveitis Nomenclature (SUN) guidelines, and modified to incorporate the consensus of end point and outcome criteria into the design of randomised trials. Despite current screening and therapeutic options (pre-biologics) 10% to 15% of children with JIA-associated uveitis may develop bilateral visual impairment and certified legally blind. To date, there remains no controlled trial evidence of benefits of biologic therapy. This study will randomise 154 patients aged 2 to 18 years with active JIA-associated uveitis (despite methotrexate (MTX) treatment for at least 12 weeks). All participants will be treated for 18 months, with follow up of 3 years from randomisation (continuing on MTX throughout). All participants will receive a stable dose of MTX and in addition either adalimumab (20 mg/0.8 ml for patients<30 kg or 40 mg/0.8 ml for patients weighing 30 kg or more, subcutaneous (s/c) injection

Osteoporosis: An Overview.

ERIC Educational Resources Information Center

Johnston, C. Conrad; Slemenda, Charles

1987-01-01

An overview of osteoporosis, its types, causes, diagnosis, and treatment is presented. Risk factors and bone mass measurement are also discussed. This article serves as an introduction to a symposium on osteoporosis containing five other articles in this issue. (MT)

Growth Outcomes After GH Therapy of Patients Given Long-Term Corticosteroids for Juvenile Idiopathic Arthritis.

PubMed

David, Hélène; Aupiais, Camille; Louveau, Baptiste; Quartier, Pierre; Jacqz-Aigrain, Evelyne; Carel, Jean-Claude; Simon, Dominique

2017-12-01

Growth hormone (GH) therapy may improve statural growth outcomes in patients with severe juvenile idiopathic arthritis (JIA). To evaluate the effect of GH treatment on adult height and to identify determinants of growth outcomes in JIA. Data from 58 patients with JIA, including 53 receiving GH, enrolled in three prospective clinical trials between 1997 and 2002 were analyzed. GH (0.056 mg/kg/d [interquartile range (IQR), 0.050 to 0.062]) for a median duration of 6.5 years (IQR, 4.7 to 7.9 years). Factors associated with a favorable growth outcome (adult height - target height ≤ -1.5 standard deviations) were identified by multivariate logistic regression. Adult height was available for 48 patients 8.6 years after GH initiation (IQR, 6.0 to 10.2 years). Height standard deviation score (SDS) increased from -2.9 (IQR, -4.4 to -1.6) at baseline to -1.7 (IQR, -3.9 to -0.1) in adulthood (P < 0.001). Median adult height was below target height [SDS, -0.2 (IQR, -1.4 to 0.4); P < 0.001]. Corrected adult height SDS was -1.3 (IQR, -3.0 to -0.2). Growth outcome was favorable in 24 (52.2%) patients. Significant independent determinants of growth outcome were age at GH initiation [adjusted odds ratio (aOR), 0.68 per additional year; 95% confidence interval (CI), 0.47 to 0.99], height at GH initiation (aOR, 2.6 per additional SDS; 95% CI, 1.15 to 5.9), and mean C-reactive protein levels during follow up (aOR, 0.51 per additional 10 mg/L; 95% CI, 0.28 to 0.92). Long-term GH treatment significantly increased growth in patients with JIA but did not fully restore the genetic growth potential. The response showed marked interindividual variability and was weaker in patients with severe inflammation. Copyright © 2017 Endocrine Society

[The spa and health resort- based treatment of back pain syndrome in the adolescents presenting with juvenile idiopathic scoliosis].

PubMed

Kravtsova, E Yu; Murav'ev, S V; Kravtsov, Yu I

The relevance of the problem arises from the lack of substantiation for the inclusion of transcutaneous spinal direct current stimulation (tSDCS) in the comprehensive spa and health resort-based treatment of back pain syndrome in the adolescents presenting with juvenile idiopathic scoliosis. The objective of the present study was to demonstrate the effectiveness of transcutaneous spinal direct current stimulation for the comprehensive spa and health resort-based treatment of back pain syndrome in the adolescents presenting with juvenile idiopathic scoliosis. A total of 18 patients with scoliosis forming the study group 1 received the traditional comprehensive spa and health resort-based treatment. The course of transcutaneous spinal direct current stimulation was prescribed to 38 other patients (comprising group 2) in addition to the standard procedures. Another control group was comprised of 15 practically healthy adolescents having no signs of spinal deformations. The visual analog scale for pain, the McGill questionnaire, the scale for the assessment of the situational and personal uneasiness levels (Spilberger Ch.D., Khanin Yu.L.), and the Beck and Tsung depression scales were used, beside the routine clinical methods. Statistical data processing was carried out with the use of the Statistica 6.0 software package. In the group of patients treated with the use of transcutaneous spinal direct current stimulation, regression of pain syndrome was well apparent. In the boys with the severity of pain estimated at 2 points based on the visual analog scale who received the standard course of the spa and health resort-based treatment, the pain rank index and the index of the number of the selected descriptors decreased significantly but nonetheless remained higher than in the patients treated by means of tSDCS as a component of the combined therapy (p=0.039). Simultaneously, the significantly lower level of situational (Q1=25.00; Me=36.50; Q3=45.00; p=0.036) and

Prevalence of osteoporosis in Australian women: Geelong Osteoporosis Study.

PubMed

Henry, M J; Pasco, J A; Nicholson, G C; Seeman, E; Kotowicz, M A

2000-01-01

To evaluate the prevalence of osteoporosis at various sites among Australian women, cross-sectional bone mineral density (BMD) data for adult females was obtained from an age-stratified population-based sample (n = 1494; 20-94 yr) drawn at random from the Barwon Statistical Division, a population characteristic of Australia. Age- and weight- (and for three sites, height) matched reference ranges for BMD at the lumbar spine, proximal femur, forearm, and total body were developed using regression techniques. The cutoff BMD level for osteoporosis at the PA spine was 0. 917g/cm(2) and 0.713 g/cm(2) at the femoral neck according to the World Health Organization (WHO) guidelines. The upper cutoff level for osteopenia was 1.128 g/cm(2) at the PA spine and 0.913g/cm(2) for the femoral neck. The proportion of Australian women categorized as having osteoporosis at the PA spine, femoral neck, or midforearm ranged from 0.9% among those aged 40-44 yr to 87.0% for those older than 79 yr. This study provides reference data representative of the Australian female population. A large proportion of elderly Australian women has osteoporosis according to the WHO guidelines.

Androgens and osteoporosis.

PubMed

Ebeling, Peter R

2010-06-01

The review is timely given recent advances regarding mechanisms of androgen action on bone cells and in humans. Osteoporosis in men is an important public health problem. An improved understanding of the role of androgens in the pathophysiology of bone loss will lead to new treatments. Androgen receptors are present in most bone cells. Testosterone acts on bone both directly via the androgen receptor and indirectly, following aromatization, via the oestrogen receptor. During skeletal modelling, ERalpha is critical for longitudinal bone growth. For periosteal growth and bone expansion, androgen receptor activation has a positive effect, whereas ERalpha activation is inhibitory. During skeletal remodelling, both receptor pathways generate similar and additive effects on bone.Androgen deficiency is a common secondary cause of osteoporosis in men and should be treated with testosterone, particularly in symptomatic men. However, lack of efficacy data for testosterone in osteoporosis means it is less useful as a first-line treatment in men with age-related declines in testosterone and osteoporosis, when other agents such as bisphosphonates and parathyroid hormone are effective. Randomized, placebo-controlled trials of testosterone therapy in men with age-related declines in testosterone and osteoporosis are needed, and should carefully evaluate potential risks, as well as its efficacy in reducing fractures and other health benefits.

The Ontario Osteoporosis Strategy: implementation of a population-based osteoporosis action plan in Canada

PubMed Central

Hawker, G.; Cameron, C.; Canavan, J.; Beaton, D.; Bogoch, E.; Jain, R.; Papaioannou, A.

2016-01-01

In the last decade, there have been a number of action plans published to highlight the importance of preventing osteoporosis and related fractures. In the province of Ontario Canada, the Ministry of Health provided funding for the Ontario Osteoporosis Strategy. The goal is to reduce morbidity, mortality, and costs from osteoporosis and related fractures through an integrated and comprehensive approach aimed at health promotion and disease management. This paper describes the components of the Ontario Osteoporosis Strategy and progress on implementation efforts as of March 2009. There are five main components: health promotion; bone mineral density testing, access, and quality; postfracture care; professional education; and research and evaluation. Responsibility for implementation of the initiatives within the components is shared across a number of professional and patient organizations and academic teaching hospitals with osteoporosis researchers. The lessons learned from each phase of the development, implementation, and evaluation of the Ontario Osteoporosis Strategy provides a tremendous opportunity to inform other jurisdictions embarking on implementing similar large-scale bone health initiatives. PMID:20309525

The Ontario Osteoporosis Strategy: implementation of a population-based osteoporosis action plan in Canada.

PubMed

Jaglal, S B; Hawker, G; Cameron, C; Canavan, J; Beaton, D; Bogoch, E; Jain, R; Papaioannou, A

2010-06-01

In the last decade, there have been a number of action plans published to highlight the importance of preventing osteoporosis and related fractures. In the province of Ontario Canada, the Ministry of Health provided funding for the Ontario Osteoporosis Strategy. The goal is to reduce morbidity, mortality, and costs from osteoporosis and related fractures through an integrated and comprehensive approach aimed at health promotion and disease management. This paper describes the components of the Ontario Osteoporosis Strategy and progress on implementation efforts as of March 2009. There are five main components: health promotion; bone mineral density testing, access, and quality; postfracture care; professional education; and research and evaluation. Responsibility for implementation of the initiatives within the components is shared across a number of professional and patient organizations and academic teaching hospitals with osteoporosis researchers. The lessons learned from each phase of the development, implementation, and evaluation of the Ontario Osteoporosis Strategy provides a tremendous opportunity to inform other jurisdictions embarking on implementing similar large-scale bone health initiatives.

Idiopathic anaphylaxis.

PubMed

Fenny, Nana; Grammer, Leslie C

2015-05-01

Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

Ottawa Panel Evidence-Based Clinical Practice Guidelines for Foot Care in the Management of Juvenile Idiopathic Arthritis.

PubMed

Brosseau, Lucie; Toupin-April, Karine; Wells, George; Smith, Christine A; Pugh, Arlanna G; Stinson, Jennifer N; Duffy, Ciarán M; Gifford, Wendy; Moher, David; Sherrington, Catherine; Cavallo, Sabrina; De Angelis, Gino; Loew, Laurianne; Rahman, Prinon; Marcotte, Rachel; Taki, Jade; Bisaillon, Jacinthe; King, Judy; Coda, Andrea; Hendry, Gordon J; Gauvreau, Julie; Hayles, Martin; Hayles, Kay; Feldman, Brian; Kenny, Glen P; Li, Jing Xian; Briggs, Andrew M; Martini, Rose; Feldman, Debbie Ehrmann; Maltais, Désirée B; Tupper, Susan; Bigford, Sarah; Bisch, Marg

2016-07-01

To create evidence-based guidelines evaluating foot care interventions for the management of juvenile idiopathic arthritis (JIA). An electronic literature search of the following databases from database inception to May 2015 was conducted: MEDLINE (Ovid), EMBASE (Ovid), Cochrane CENTRAL, and clinicaltrials.gov. The Ottawa Panel selection criteria targeted studies that assessed foot care or foot orthotic interventions for the management of JIA in those aged 0 to ≤18 years. The Physiotherapy Evidence Database scale was used to evaluate study quality, of which only high-quality studies were included (score, ≥5). A total of 362 records were screened, resulting in 3 full-text articles and 1 additional citation containing supplementary information included for the analysis. Two reviewers independently extracted study data (intervention, comparator, outcome, time period, study design) from the included studies by using standardized data extraction forms. Directed by Cochrane Collaboration methodology, the statistical analysis produced figures and graphs representing the strength of intervention outcomes and their corresponding grades (A, B, C+, C, C-, D+, D, D-). Clinical significance was achieved when an improvement of ≥30% between the intervention and control groups was present, whereas P>.05 indicated statistical significance. An expert panel Delphi consensus (≥80%) was required for the endorsement of recommendations. All included studies were of high quality and analyzed the effects of multidisciplinary foot care, customized foot orthotics, and shoe inserts for the management of JIA. Custom-made foot orthotics and prefabricated shoe inserts displayed the greatest improvement in pain intensity, activity limitation, foot pain, and disability reduction (grades A, C+). The use of customized foot orthotics and prefabricated shoe inserts seems to be a good choice for managing foot pain and function in JIA. Copyright © 2016 American Congress of Rehabilitation

Plasma miR-26a as a Diagnostic Biomarker Regulates Cytokine Expression in Systemic Juvenile Idiopathic Arthritis.

PubMed

Sun, Juan; Feng, Miao; Wu, Fengqi; Ma, Xiaolin; Lu, Jie; Kang, Min; Liu, Zhewei

2016-08-01

We sought to identify specific microRNA (miRNA) for systemic juvenile idiopathic arthritis (sJIA) and to determine the involvement of these miRNA in regulating the expression of cytokines. Microarray profiling was performed to identify differentially expressed miRNA in sJIA plasma. Levels of candidate miRNA and mRNA were assessed by real-time PCR, and cytokines were measured by ELISA. Dual-luciferase reporter assay was used to validate the direct interaction between miR-26a and interleukin 6 (IL-6). Forty-eight miRNA were differentially expressed in the plasma of patients with sJIA compared with healthy controls (HC). Five miRNA were selected for further validation. The expression level of miR-26a was exclusively elevated in the plasma of patients with sJIA as compared with 4 rheumatic diseases and 2 subtypes of JIA (oligoarticular and polyarticular). The levels of IL-6, IL-1β, and tumor necrosis factor-α in the plasma of patients with sJIA were increased, and only IL-6 presented a positive correlation with miR-26a (r = 0.539, p < 0.0001). After stimulation with IL-6, miR-26a expression was upregulated in THP-1 cells, while the supernatant level of IL-6 was downregulated by transfection of miR-26a mimics. Consistently, direct target relationship between miR-26a and IL-6 was confirmed. This study demonstrates that miR-26a is expressed specifically and highly in sJIA plasma and suggests that miR-26a may regulate the levels of cytokines in sJIA. Our findings highlight miR-26a as a potential biomarker for the diagnosis as well as differential diagnosis of sJIA.

Incidence and Prevalence of Juvenile Idiopathic Arthritis Among Children in a Managed Care Population, 1996–2009

PubMed Central

Harrold, Leslie R.; Salman, Craig; Shoor, Stanford; Curtis, Jeffrey R.; Asgari, Maryam M.; Gelfand, Joel M.; Wu, Jashin J.; Herrinton, Lisa J.

2017-01-01

Objective Few studies based in well-defined North American populations have examined the occurrence of juvenile idiopathic arthritis (JIA), and none has been based in an ethnically diverse population. We used computerized healthcare information from the Kaiser Permanente Northern California membership to validate JIA diagnoses and estimate the incidence and prevalence of the disease in this well-characterized population. Methods We identified children aged ≤ 15 years with ≥ 1 relevant International Classification of Diseases, 9th edition, diagnosis code of 696.0, 714, or 720 in computerized clinical encounter data during 1996–2009. In a random sample, we then reviewed the medical records to confirm the diagnosis and diagnosis date and to identify the best-performing case-finding algorithms. Finally, we used the case-finding algorithms to estimate the incidence rate and point prevalence of JIA. Results A diagnosis of JIA was confirmed in 69% of individuals with at least 1 relevant code. Forty-five percent were newly diagnosed during the study period. The age- and sex-standardized incidence rate of JIA per 100,000 person-years was 11.9 (95% CI 10.9–12.9). It was 16.4 (95% CI 14.6–18.1) in girls and 7.7 (95% CI 6.5–8.9) in boys. The peak incidence rate occurred in children aged 11–15 years. The prevalence of JIA per 100,000 persons was 44.7 (95% CI 39.1–50.2) on December 31, 2009. Conclusion The incidence rate of JIA observed in the Kaiser Permanente population, 1996–2009, was similar to that reported in Rochester, Minnesota, USA, but 2 to 3 times higher than Canadian estimates. PMID:23588938

Associations between interleukin-10 polymorphisms and susceptibility to juvenile idiopathic arthritis: a systematic review and meta-analysis.

PubMed

Harsini, Sara; Saghazadeh, Amene; Nedjat, Saharnaz; Rezaei, Nima

2018-03-01

Cytokine genes, including interleukin-10 (IL-10), are known to play important roles in the pathogenesis of juvenile idiopathic arthritis (JIA). This study aims to determine whether the IL-10 polymorphisms confer susceptibility to JIA. A meta-analysis was performed on the associations between the IL-10 -1082 G/A, -592 C/A, and -819 C/T polymorphisms and JIA. A total number of 7 studies involving 1,785 patients and 6,142 controls were considered in the meta-analysis. Meta-analysis of the IL-10 -592 C/A and -819 C/T polymorphisms showed no association with JIA in the study participants, or in Caucasian or Middle Eastern participants. Meta-analysis of the IL-10 -1082 A allele in all study participants, Caucasian and Middle Eastern, showed significant associations with RA (overall ORs were 1.17, 1.15, and 1.41, respectively). Meta-analysis of the AA versus GG genotype of the IL-10 -1082 G/A polymorphism revealed significant associations with JIA (OR = 3.66, 95% CI = 1.44-9.29, P = 0.006) in participants from Middle Eastern countries. Additionally, meta-analysis of the GG versus AA+GA genotypes of the IL-10 -1082 G/A polymorphism revealed the GG genotype as the protective factor against JIA in the Middle Eastern subgroup (OR = 0.44, 95% CI = 0.20-0.94, P = 0,04). Moreover, meta-analysis of the IL-10 -1082 A allele in 4 studies on Hardy-Weinberg equilibrium showed a significant association with JIA (OR = 1.17, 95% CI = 1.07-1.28, P = 0.0009). No association was found between the IL-10 (-1082, -819, -592) ACC, ATA, and GCC haplotypes and JIA. These results suggest that the IL-10 -1082 G/A polymorphism confers susceptibility to JIA.

Room for improvement: patient, parent, and practitioners' perceptions of foot problems and foot care in juvenile idiopathic arthritis.

PubMed

Hendry, Gordon J; Turner, Debbie E; Lorgelly, Paula K; Woodburn, James

2012-11-01

To explore the perceived impact of disease-related foot problems and foot care in juvenile idiopathic arthritis (JIA) from the perspectives of patients, parents, pediatric rheumatologists, and health professionals. A qualitative study using an interpretative phenomenological approach. Outpatients department, public health service children's hospital. Patients (N=15; 4 adult patients, 2 parents of children with JIA, 3 pediatric rheumatologists, and 6 health professionals) from 2 National Health Service rheumatology centers (1 pediatric and 1 adult). Not applicable. Qualitative outcomes were participants' perceptions elicited using semistructured interviews (telephone or face-to-face) and focus groups using an interpretative phenomenological approach. A data-driven inductive approach to coding and theme development was adopted for transcript analysis. Participants volunteered to take part in a total of 7 interviews and 2 focus groups. The analysis revealed 6 key themes related to the impact of foot problems and perceptions of foot care from respective groups. These were the following: (1) pain, (2) mobility impairment, (3) reduced ability to perform activities of daily living, (4) footwear difficulties, (5) poor referral pathways/delayed access to care, and (6) lack of evidence in support of conservative foot care. Several areas for development of foot care services were identified including a need for improved referral pathways, shorter waiting times for initial consultations, greater attention to patient compliance, and a need for better evidence in support of customized foot orthoses. Several key foot health-related outcomes were identified, which may be of importance for measuring therapeutic response to foot-related interventions. Copyright © 2012 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report

PubMed Central

Bottai, Matteo; Tjärnlund, Anna; Santoni, Giola; Werth, Victoria P; Pilkington, Clarissa; de Visser, Marianne; Alfredsson, Lars; Amato, Anthony A; Barohn, Richard J; Liang, Matthew H; Aggarwal, Rohit; Arnardottir, Snjolaug; Chinoy, Hector; Cooper, Robert G; Danko, Katalin; Dimachkie, Mazen M; Feldman, Brian M; García-De La Torre, Ignacio; Gordon, Patrick; Hayashi, Taichi; Katz, James D; Kohsaka, Hitoshi; Lachenbruch, Peter A; Lang, Bianca A; Li, Yuhui; Oddis, Chester V; Olesinka, Marzena; Reed, Ann M; Rutkowska-Sak, Lidia; Sanner, Helga; Selva-O’Callaghan, Albert; Wook Song, Yeong; Ytterberg, Steven R; Miller, Frederick W; Rider, Lisa G; Lundberg, Ingrid E; Amoruso, Maria

2017-01-01

Objective To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs) and their major subgroups. Methods An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for inclusion in the criteria. Rheumatology, dermatology, neurology and paediatric clinics worldwide collected data on 976 IIM cases (74% adults, 26% children) and 624 non-IIM comparator cases with mimicking conditions (82% adults, 18% children). The participating clinicians classified each case as IIM or non-IIM. Generally, the classification of any given patient was based on few variables, leaving remaining variables unmeasured. We investigated the strength of the association between all variables and between these and the disease status as determined by the physician. We considered three approaches: (1) a probability-score approach, (2) a sum-of-items approach criteria and (3) a classification-tree approach. Results The approaches yielded several candidate models that were scrutinised with respect to statistical performance and clinical relevance. The probability-score approach showed superior statistical performance and clinical practicability and was therefore preferred over the others. We developed a classification tree for subclassification of patients with IIM. A calculator for electronic devices, such as computers and smartphones, facilitates the use of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria. Conclusions The new EULAR/ACR classification criteria provide a patient’s probability of having IIM for use in clinical and research settings. The probability is based on a score obtained by summing the weights associated with a set of criteria items. PMID:29177080

Fatigue in children with juvenile idiopathic arthritis: reliability of the "Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale".

PubMed

Paulo, Luciana Tudech S P; Len, Claudio A; Hilario, Maria Odete E; Pedroso, Soraya A; Vitalle, Maria Sylvia S; Terreri, Maria Teresa

2015-01-01

The aim of the study was (1) to translate the "Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale" (PedsQL-Fatigue) into Brazilian Portuguese language and culture and evaluate its reliability and (2) to measure fatigue among patients with juvenile idiopathic arthritis (JIA): (1) Translation of the PedsQL-Fatigue by two bilingual researchers; (2) Backtranslation into English assessed by the authors of the original version; (3) Pilot study with five patients followed in the Pediatric Rheumatology Outpatient Clinic and their parents; and (4) Field study and assessment of measurement properties (internal consistency, reproducibility, and construct validity). In this stage, the scale was administered to 67 patients with JIA and 63 healthy individuals, aged from 2 to 18 years old, matched by age (from 2 to 4, 5 to 7, 8 to 12, and from 13 to 18 years old). Cronbach's alpha coefficient ranged from 0.6 to 0.8 for children and parents, indicating the instrument's good internal consistency. The scale's construct validity was confirmed by a satisfactory Spearman's coefficient between the PedsQL-Fatigue and the generic PedsQL 4.0 (0.840 for the children and 0.742 for the parents). Reproducibility was also adequate (0.764 for the children and 0.938 for the parents). No differences were found between the scores obtained by the JIA group and control group, though lower scores were observed among patients with clinically active JIA when compared to those without clinical activity. The PedsQL-Fatigue is a valid and reliable tool, and that can be used to measure fatigue among patients with JIA.

[Osteoporosis and stress].

PubMed

Kumano, Hiroaki

2005-09-01

There may be three ways of relationship between stress and osteoporosis. The first is that stress induces some physiological changes leading to osteoporosis. The second is that stress induces behavioral distortion of eating, drinking, exercise, and sleep habits, which leads to osteoporosis. The third is that osteoporosis, on the other hand, brings about anxiety, depression, loss of social roles, and social isolation, which leads to stress. The susceptible sex and age groups are postmenopausal women and young women. The abrupt decrease of estrogen in postmenopausal women promotes reabsorption of bone, and it was also reported that the increase of interleukin-6 (IL-6) that is downstream of estrogen was related to the production of osteoclast and to the development of disability of the aged. Regarding the association with stress, while it was reported that depression or depressive states directly increased inflammation-induced cytokines including IL-6, it was also pointed out that stress-induced easy infectious may produce chronic infection, which indirectly increases inflammation-induced cytokines. Anorexia Nervosa that is assumed to be associated with adolescent developmental stress is noteworthy in young women. Amenorrhea is always present in this disease, and in addition to bone reabsorption associated with estrogen deficiency, the decrease of bone formation associated with malnutrition may be related to the development of osteoporosis.

Treatment of osteoporosis in men

PubMed Central

Kaufman, J.-M.; Reginster, J.-Y.; Boonen, S.; Brandi, M. L.; Cooper, C.; Dere, W.; Devogelaer, J.-P.; Diez-Perez, A.; Kanis, J. A.; McCloskey, E.; Mitlak, B.; Orwoll, E.; Ringe, J.D.; Weryha, G.; Rizzoli, R.

2013-01-01

Summary Aspects of osteoporosis in men, such as screening and identification strategies, definitions of diagnosis and intervention thresholds, and treatment options (both approved and in the pipeline) are discussed. Introduction Awareness of osteoporosis in men is improving, although it remains under-diagnosed and under-treated. A European Society for Clinical and Economic Aspects of Osteoporosis and Osteoarthritis (ESCEO) workshop was convened to discuss osteoporosis in men and to provide a report by a panel of experts (the authors). Methods A debate with an expert panel on preselected topics was conducted. Results and Conclusions Although additional fracture data are needed to endorse the clinical care of osteoporosis in men, consensus views were reached on diagnostic criteria and intervention thresholds. Empirical data in men display similarities with data acquired in women, despite pathophysiological differences, which may not be clinically relevant. Men should receive treatment at a similar 10-year fracture probability as in women. The design of mixed studies may reduce the lag between comparable treatments for osteoporosis in women becoming available in men. PMID:23201268

Genetics of osteoporosis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Urano, Tomohiko; Inoue, Satoshi, E-mail: INOUE-GER@h.u-tokyo.ac.jp; Department of Anti-Aging Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo 113-8655

Highlights: • Single-nucleotide polymorphisms (SNPs) associated with osteoporosis were identified. • SNPs mapped close to or within VDR and ESR1 are associated with bone mineral density. • WNT signaling pathway plays a pivotal role in regulating bone mineral density. • Genetic studies will be useful for identification of new therapeutic targets. - Abstract: Osteoporosis is a skeletal disease characterized by low bone mineral density (BMD) and microarchitectural deterioration of bone tissue, which increases susceptibility to fractures. BMD is a complex quantitative trait with normal distribution and seems to be genetically controlled (in 50–90% of the cases), according to studies onmore » twins and families. Over the last 20 years, candidate gene approach and genome-wide association studies (GWAS) have identified single-nucleotide polymorphisms (SNPs) that are associated with low BMD, osteoporosis, and osteoporotic fractures. These SNPs have been mapped close to or within genes including those encoding nuclear receptors and WNT-β-catenin signaling proteins. Understanding the genetics of osteoporosis will help identify novel candidates for diagnostic and therapeutic targets.« less

Osteoporosis: primary prevention in the community.

PubMed

Loh, K Y; Shong, H K

2007-10-01

The incidence of osteoporosis is increasing worldwide. It has great impact on the life of the elderly population. The most significant medical consequence of osteoporosis is fragility fracture which without proper treatment will cause severe medical and psychosocial complications. The overall cost in managing osteoporosis and its related fractures is escalating. Using bone densitometry to measure bone mineral density is useful in the diagnosis of osteoporosis but it is costly and not feasible in the community. Drugs such as estrogen replacement, raloxifene and calcitonin are effective in prevention and treatment of osteoporosis but they are also expensive. Identifying modifiable risk factors such as smoking, lack of exercise, low dietary calcium and vitamin D intake and healthy life style remain strategy in the primary prevention of osteoporosis in the community.

Osteoporosis in Inflammatory Bowel Disease

PubMed Central

Ali, Tauseef; Lam, David; Bronze, Michael S.; Humphrey, Mary Beth

2010-01-01

Osteoporosis commonly afflicts patients with inflammatory bowel disease, and many factors link the 2 states together. A literature review was conducted about the pathophysiology of osteoporosis in relation to inflammatory bowel disease. Screening guidelines for osteoporosis in general as well as those directed at patients with inflammatory bowel disease are reviewed, as are currently available treatment options. The purpose of this article is to increase physician awareness about osteopenia and osteoporosis occurring in patients with inflammatory bowel disease and to provide basic, clinically relevant information about the pathophysiology and guidelines to help them treat these patients in a cost-effective manner. PMID:19559158

Motor system hyperconnectivity in juvenile myoclonic epilepsy: a cognitive functional magnetic resonance imaging study.

PubMed

Vollmar, Christian; O'Muircheartaigh, Jonathan; Barker, Gareth J; Symms, Mark R; Thompson, Pamela; Kumari, Veena; Duncan, John S; Janz, Dieter; Richardson, Mark P; Koepp, Matthias J

2011-06-01

Juvenile myoclonic epilepsy is the most frequent idiopathic generalized epilepsy syndrome. It is characterized by predominant myoclonic jerks of upper limbs, often provoked by cognitive activities, and typically responsive to treatment with sodium valproate. Neurophysiological, neuropsychological and imaging studies in juvenile myoclonic epilepsy have consistently pointed towards subtle abnormalities in the medial frontal lobes. Using functional magnetic resonance imaging with an executive frontal lobe paradigm, we investigated cortical activation patterns and interaction between cortical regions in 30 patients with juvenile myoclonic epilepsy and 26 healthy controls. With increasing cognitive demand, patients showed increasing coactivation of the primary motor cortex and supplementary motor area. This effect was stronger in patients still suffering from seizures, and was not seen in healthy controls. Patients with juvenile myoclonic epilepsy showed increased functional connectivity between the motor system and frontoparietal cognitive networks. Furthermore, we found impaired deactivation of the default mode network during cognitive tasks with persistent activation in medial frontal and central regions in patients. Coactivation in the motor cortex and supplementary motor area with increasing cognitive load and increased functional coupling between the motor system and cognitive networks provide an explanation how cognitive effort can cause myoclonic jerks in juvenile myoclonic epilepsy. The supplementary motor area represents the anatomical link between these two functional systems, and our findings may be the functional correlate of previously described structural abnormalities in the medial frontal lobe in juvenile myoclonic epilepsy.

Idiopathic Ophthalmodynia and Idiopathic Rhinalgia: A Prospective Series of 16 New Cases.

PubMed

Pareja, Juan A; Montojo, Teresa; Guerrero, Ángel L; Álvarez, Mónica; Porta-Etessam, Jesús; Cuadrado, María L

2015-01-01

Idiopathic ophthalmodynia and idiopathic rhinalgia were described a few years ago. These conditions seem specific pain syndromes with a distinctive location in the eye or in the nose. We aimed to present a new prospective series in order to verify the consistency of these syndromes. We performed a descriptive study of all patients referred to our regional neurologic clinics from 2010 to 2014 because of facial pain exclusively felt in the eye or in the nose fulfilling the proposed diagnostic criteria for idiopathic ophthalmodynia and idiopathic rhinalgia. There were 9 patients with idiopathic ophthalmodynia and 7 patients with idiopathic rhinalgia, with a clear female preponderance, and a mean age at onset in the fifth decade. The pain was usually moderate and the temporal pattern was generally chronic. Only one patient reported accompaniments (hypersensitivity to the light and to the flow of air in the symptomatic eye). Preventive treatment with amitriptyline, pregabalin, or gabapentin was partially or totally effective. The clinical features of this new series parallels those of the original description, thus indicating that both idiopathic ophthalmodynia and idiopathic rhinalgia have clear-cut clinical pictures with excellent consistency both inter- and intra-individually. © 2015 American Headache Society.

[Secondary osteoporosis].

PubMed

Lafita, J; Pineda, J; Fuentes, C; Martínez, J P

2003-01-01

Secondary osteoporosis is caused by pathologies or medications, differing from the bone loss explainable by the post-menopausal stage or by ageing. The possible pathologies that can condition the loss of bone mass are very varied: endocrinological, digestive, genetic, haematological, rheumatic, post-transplant, pharmacological and a wide miscellaneous group. This article essentially reviews the endocrinological causes, with special emphasis on the more controversial aspects, followed by a clinical approach for the systematic diagnosis of these pathologies, which are frequent in cases initially labelled as primary osteoporosis

[Epidemiology of osteoporosis].

PubMed

Grazio, Simeon

2006-01-01

Osteoporosis represents a major and increasing public health problem with the aging of population. Major clinical consequences and economic burden of the disease are fractures. Many risk factors are associated with the fractures including low bone mass, hormonal disorders, personal and family history of fractures, low body weight, use of certain drugs (e.g. glucocorticoids), cigarette smoking, elevated intake of alchohol, low physical activity, insufficient level of vitamin D and low intake of calcium. This epidemiological review describes frequency, importance of risk factors and impact of osteoporosis and osteoporotic fractures. Objective measures of bone mineral density along with clinical assessment of risk factors can help identify patients who will benefit from prevention and intervention efforts and eventually reduce the morbidity and mortality associated with osteoporosis-related fractures.

A randomised controlled trial of the clinical effectiveness, safety and cost-effectiveness of adalimumab in combination with methotrexate for the treatment of juvenile idiopathic arthritis associated uveitis (SYCAMORE Trial)

PubMed Central

2014-01-01

Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Children with JIA are at risk of inflammation of the uvea in the eye (uveitis). Overall, 20% to 25% of paediatric uveitis is associated with JIA. Major risk factors for development of uveitis in JIA are oligoarticular pattern of arthritis, an age at onset of arthritis of less than seven years of age, and antinuclear antibody positivity. In the initial stages of mild to moderate inflammation the uveitis is asymptomatic. This has led to current practice of screening all children with JIA for uveitis. Approximately 12% to 38% of patients with JIA develop uveitis in seven years following onset of arthritis. In 30% to 50% of children with JIA-associated uveitis structural complications are present at diagnosis. Furthermore about 50% to 75% of those with severe uveitis will eventually develop visual impairment secondary to ocular complications such as cataract and glaucoma. Defining the severity of inflammation and structural complications in uveitis patients is now possible following Standardised Uveitis Nomenclature (SUN) guidelines, and modified to incorporate the consensus of end point and outcome criteria into the design of randomised trials. Despite current screening and therapeutic options (pre-biologics) 10% to 15% of children with JIA-associated uveitis may develop bilateral visual impairment and certified legally blind. To date, there remains no controlled trial evidence of benefits of biologic therapy. Methods/design This study will randomise 154 patients aged 2 to 18 years with active JIA-associated uveitis (despite methotrexate (MTX) treatment for at least 12 weeks). All participants will be treated for 18 months, with follow up of 3 years from randomisation (continuing on MTX throughout). All participants will receive a stable dose of MTX and in addition either adalimumab (20 mg/0.8 ml for patients <30 kg or 40 mg/0.8 ml for patients weighing 30 kg or more

Treatment of primary osteoporosis in men

PubMed Central

Giusti, Andrea; Bianchi, Gerolamo

2015-01-01

With the aging of the population worldwide, osteoporosis and osteoporotic fractures are becoming a serious health care issue in the Western world. Although less frequent than in women, osteoporosis in men is a relatively common problem. Hip and vertebral fractures are particularly relevant, being associated with significant mortality and disability. Since bone loss and fragility fractures in men have been recognized as serious medical conditions, several randomized controlled trials (RCTs) have been undertaken in males with osteoporosis to investigate the anti-fracture efficacy of the pharmacological agents commonly used to treat postmenopausal osteoporosis. Overall, treatments for osteoporosis in men are less defined than in women, mainly due to the fact that there are fewer RCTs performed in male populations, to the relatively smaller sample sizes, and to the lack of long-term extension studies. However, the key question is whether men are expected to respond differently to osteoporosis therapies than women. The pharmacological properties of bisphosphonates, teriparatide, denosumab, and strontium ranelate make such differentiation unlikely, and available clinical data support their efficacy in men with primary osteoporosis as well as in women. In a series of well-designed RCTs, alendronate, risedronate, zoledronic acid, and teriparatide were demonstrated to reduce the risk of new vertebral fractures in men presenting with primary osteoporosis (including osteoporosis associated with low testosterone levels) and to improve the bone mineral density (BMD). In preliminary studies, ibandronate, denosumab, and strontium ranelate also showed their beneficial effects on surrogate outcomes (BMD and markers of bone turnover) in men with osteoporosis. Although direct evidence about their non-vertebral anti-fracture efficacy are lacking, the effects of bisphosphonates, denosumab, teriparatide, and strontium ranelate on surrogate outcomes (BMD and markers of bone turnover

Ottawa Panel Evidence-Based Clinical Practice Guidelines for Structured Physical Activity in the Management of Juvenile Idiopathic Arthritis.

PubMed

Cavallo, Sabrina; Brosseau, Lucie; Toupin-April, Karine; Wells, George A; Smith, Christine A; Pugh, Arlanna G; Stinson, Jennifer; Thomas, Roanne; Ahmed, Sara; Duffy, Ciarán M; Rahman, Prinon; Àlvarez-Gallardo, Inmaculada C; Loew, Laurianne; De Angelis, Gino; Feldman, Debbie Ehrmann; Majnemer, Annette; Gagnon, Isabelle J; Maltais, Désirée; Mathieu, Marie-Ève; Kenny, Glen P; Tupper, Susan; Whitney-Mahoney, Kristi; Bigford, Sarah

2017-05-01

To create guidelines focused on the use of structured physical activity (PA) in the management of juvenile idiopathic arthritis (JIA). A systematic literature search was conducted using the electronic databases Cochrane Central Register of Controlled Trials, MEDLINE (Ovid), EMBASE (Ovid), and Physiotherapy Evidence Database for all studies related to PA programs for JIA from January 1966 until December 2014, and was updated in May 2015. Study selection was completed independently by 2 reviewers. Studies were included if they involved individuals aged ≤21 years diagnosed with JIA who were taking part in therapeutic exercise or other PA interventions for which effects of various disease-related outcomes were compared with a control group (eg, no PA program or activity of lower intensity). Two reviewers independently extracted information on interventions, comparators, outcomes, time period, and study design. The statistical analysis was reported using the Cochrane Collaboration methods. The quality of the included studies was assessed according to the Physiotherapy Evidence Database Scale. Five randomized controlled trials (RCTs) fit the selection criteria; of these, 4 were high-quality RCTs. The following recommendations were developed: (1) Pilates for improving quality of life, pain, functional ability, and range of motion (ROM) (grade A); (2) home exercise program for improving quality of life and functional ability (grade A); (3) aquatic aerobic fitness for decreasing the number of active joints (grade A); and (4) and cardio-karate aerobic exercise for improving ROM and number of active joints (grade C+). The Ottawa Panel recommends the following structured exercises and physical activities for the management of JIA: Pilates, cardio-karate, home and aquatic exercises. Pilates showed improvement in a higher number of outcomes. Copyright © 2017. Published by Elsevier Inc.

Generation of novel pharmacogenomic candidates in the response to methotrexate in juvenile idiopathic arthritis: correlation between gene expression and genotype

PubMed Central

Moncrieffe, Halima; Hinks, Anne; Ursu, Simona; Kassoumeri, Laura; Etheridge, Angela; Hubank, Mike; Martin, Paul; Weiler, Tracey; Glass, David N; Thompson, Susan D.; Thomson, Wendy; Wedderburn, Lucy R

2010-01-01

Objectives Little is known about mechanisms of efficacy of methotrexate (MTX) in childhood arthritis, or genetic influences upon response to MTX. The aims of this study were to use gene expression profiling to identify novel pathways/genes altered by MTX and then investigate these genes for genotype associations with response to MTX treatment. Methods Gene expression profiling before and after MTX treatment was performed on 11 children with juvenile idiopathic arthritis (JIA) treated with MTX, in whom response at 6 months of treatment was defined. Genes showing the most differential gene expression after treatment were selected for SNP genotyping. Genotype frequencies were compared between non-responders and responders (ACR-Ped70). An independent cohort was available for validation. Results Gene expression profiling before and after MTX treatment revealed 1222 differentially expressed probes sets (fold change >1.7, p< 0.05) and 1065 when restricted to full responder cases only. Six highly differentially expressed genes were analysed for genetic association to response to MTX. Three SNPs in the SLC16A7 gene showed significant association with MTX response. One SNP showed validated association in an independent cohort. Conclusions This study is the first, to our knowledge, to evaluate gene expression profiles in children with JIA before and after MTX, and to analyse genetic variation in differentially expressed genes. We have identified a gene which may contribute to genetic variability in MTX response in JIA, and established as proof of principle that genes which are differentially expressed at mRNA level after drug administration may also be good candidates for genetic analysis. PMID:20827233

Association of tumour necrosis factor-alpha G/A -238 and G/A -308 single nucleotide polymorphisms with juvenile idiopathic arthritis.

PubMed

Maddah, M; Harsini, S; Ziaee, V; Moradinejad, M H; Rezaei, A; Zoghi, S; Sadr, M; Aghighi, Y; Rezaei, N

2016-12-01

Juvenile idiopathic arthritis (JIA) is a heterogeneous autoimmune disorder of unknown origin. As proinflammatory cytokines are known to contribute towards the pathogenesis of JIA, this case-control study was performed to examine the associations of certain single nucleotide polymorphisms (SNPs) of tumour necrosis factor-α (TNF-α) gene. Fifty-three patients with JIA participated in this study as patients group and compared with 137 healthy unrelated controls. Genotyping was performed for TNF-α gene at positions -308 and -238, using polymerase chain reaction with sequence-specific primers method. Results of the analysed data revealed a significant positive association for TNF-α gene at positions -308 and -238 for A allele in patients group compared with controls (P < 0.01). At the genotypic level, the frequency of TNF-α gene at positions -308 and -238 for GG genotype was discovered to be higher in the patients with JIA compared to the healthy controls (P < 0.01), while GA genotype at the same positions was observed to be less frequent in the case group than the controls (P < 0.01). At the haplotypic level, a significant positive association for TNF-α GG haplotype (positions -308, -238) together with a notable negative association for TNF-α AG and GA haplotypes at the same positions were detected in the patients group in comparison with the healthy individuals (P < 0.01). Cytokine gene polymorphisms might affect the development of JIA. Particular TNF-α gene variants could render individuals more susceptible to JIA.. © 2016 John Wiley & Sons Ltd.

Long-Term Health-Related Quality of Life in German Patients with Juvenile Idiopathic Arthritis in Comparison to German General Population.

PubMed

Barth, Swaantje; Haas, Johannes-Peter; Schlichtiger, Jenny; Molz, Johannes; Bisdorff, Betty; Michels, Hartmut; Hügle, Boris; Radon, Katja

2016-01-01

Aims of the study were to investigate health-related quality of life (HRQOL) in adult patients with former diagnosis of Juvenile Idiopathic Arthritis (JIA), to compare their HRQOL with the general population and to identify factors related to a poor outcome. In 2012, a cross-sectional survey was performed by mailing a questionnaire to a large cohort of former and current patients of the German Centre for Rheumatology in Children and Adolescents. Only adult patients (≥18 years) with a diagnosis compatible with JIA were included (n = 2592; response 66%). The questionnaire included information about HRQOL (EQ5D), disease-related questions and socio-demographics. Prevalence and 95% confidence intervals (CI) of problems with mobility, self-care, usual activities, pain and anxiety/depression were standardized to the German general population. Factors associated with low HRQOL in JIA patients were identified using logistic regression models. Sixty-two percent of the study population was female; age range was 18-73 years. In all dimensions, JIA patients reported statistically significantly more problems than the general population with largest differences in the pain dimension (JIA patients 56%; 95%CI 55-58%; general population 28%; 26-29%) and the anxiety/depression dimension (28%; 27-29% vs. 4%; 4-5%). Lower HRQOL in JIA patients was associated with female sex, older age, lower level of education, still being under rheumatic treatment and disability. HRQOL in adult JIA patients is considerably lower than in the general population. As this cohort includes historic patients the new therapeutic schemes available today are expected to improve HRQOL in future.

Internet Program for Physical Activity and Exercise Capacity in Children With Juvenile Idiopathic Arthritis: A Multicenter Randomized Controlled Trial.

PubMed

Armbrust, Wineke; Bos, G J F Joyce; Wulffraat, Nico M; van Brussel, Marco; Cappon, Jeannette; Dijkstra, Pieter U; Geertzen, Jan H B; Legger, G Elizabeth; van Rossum, Marion A J; Sauer, Pieter J J; Lelieveld, Otto T H M

2017-07-01

To determine the effects of Rheumates@Work, an internet-based program supplemented with 4 group sessions, aimed at improving physical activity, exercise capacity, health-related quality of life (HRQoL), and participation in children with juvenile idiopathic arthritis. Patients were recruited from 3 pediatric rheumatology centers in The Netherlands for an observer-blinded, randomized controlled multicenter trial. Physical activity level, time spent in rest, light, and moderate-to-vigorous physical activity (MVPA) were recorded in a diary and with an accelerometer, before intervention, after intervention, and at followup after 3 and 12 months (intervention group only). Exercise capacity was assessed using the Bruce treadmill protocol, HRQoL was assessed with the Pediatric Quality of Life Inventory generic core scale, and participation in school and in physical education classes were assessed by questionnaire. The intervention group consisted of 28 children, and there were 21 children in the control group. MVPA , exercise capacity, and participating in school and physical education classes improved significantly in the intervention group. HRQoL improved in the control group. No significant differences were found between groups. The effect of Rheumates@Work on physical activity and exercise capacity lasted during the 12 months of followup. Improvements in physical activity were significantly better for the cohort starting in winter compared to the summer cohort. Rheumates@Work had a positive, albeit small, effect on physical activity, exercise capacity, and participation in school and physical education class in the intervention group. Improvements lasted for 12 months. Participants who started in winter showed the most improvement. Rheumates@Work had no effect on HRQoL. © 2016, American College of Rheumatology.

Delineating the role of multiple intraarticular corticosteroid injections in the management of juvenile idiopathic arthritis in the biologic era.

PubMed

Papadopoulou, Charalampia; Kostik, Mikhail; Gonzalez-Fernandez, Maria Isabel; Bohm, Marek; Nieto-Gonzalez, Juan Carlos; Pistorio, Angela; Lanni, Stefano; Consolaro, Alessandro; Martini, Alberto; Ravelli, Angelo

2013-07-01

To investigate the outcome and predicting factors of multiple intraarticular corticosteroid (IAC) injections in children with juvenile idiopathic arthritis (JIA). The clinical charts of patients who received their first IAC injection in ≥3 joints between January 2002 and December 2011 were reviewed. The corticosteroid used was triamcinolone hexacetonide for large joints and methylprednisolone acetate for small or difficult to access joints. In each patient, the followup period after IAC injection was censored in case of synovitis flare or at the last visit with continued remission. Predictors included sex, age at disease onset, JIA category, antinuclear antibody (ANA) status, age and disease duration, disease course, general anesthesia, number and type of injected joints, acute-phase reactants, and concomitant systemic medications. A total of 220 patients who had 1,096 joints injected were included. Following IAC therapy, 66.4% of patients had synovitis flare after a median of 0.5 years, whereas 33.6% of patients had sustained remission after a median of 0.9 years. The cumulative probability of survival without synovitis flare was 50.0%, 31.5%, and 19.5% at 1, 2, and 3 years, respectively. On Cox regression analysis, positive C-reactive protein value, negative ANA, lack of concomitant methotrexate administration, and a polyarticular (versus an oligoarticular) disease course were the strongest predictors for synovitis flare. Multiple IAC injection therapy induced sustained remission of joint synovitis in a substantial proportion of patients. A controlled trial comparing multiple IAC injection therapy and methotrexate versus methotrexate and a tumor necrosis factor antagonist is worthy of consideration. Copyright © 2013 by the American College of Rheumatology.

A study of school adjustment, self-concept, self-esteem, general wellbeing and parent child relationship in Juvenile Idiopathic Arthritis.

PubMed

Yadav, Anita; Yadav, T P

2013-03-01

To assess school adjustment, self-concept, self-esteem, general wellbeing and parent-child relationship in children with Juvenile Idiopathic Arthritis (JIA)and to study the correlation of these parameters with chronicity of disease, number of active joints, laboratory parameters of disease activity and JIA subtypes. A total of 64 children (32 cases and 32 controls) were recruited for analysis. Self report questionnaires which included PGI General Wellbeing Measure, Adjustment Inventory for School Students, Parent Child Relationship Scale, Self Esteem Inventory and Self Concept Questionnaires were used to assess all the enrolled subjects. Cases had significantly lower general physical well being (p < 0.001), self- esteem (p = 0.039), social self-concept (p = 0.023) and poorer social (p = 0.002), educational (p = 0.002) and overall (p = 0.006) adjustment as compared to controls. Both parents of cases were significantly more demanding (p = 0.028, p = 0.004)and mothers were over protective (p = 0.009) and pampering with object rewards (p = 0.02). PGI wellbeing score (p = 0.042, p = 0.019) and self concept (p = 0.002, for social SCQ p = 0.030) correlated well with number of active joints and ESR. As the disease duration increased, fathers tended to neglect their children (p = 0.043) and with persistent disease activity (reflected by CRP positivity) even resorted to punishment (p = 0.022) or remained indifferent (p = 0.048). JIA significantly hampers the child's self-esteem, self-concept, adjustment in school, general wellbeing and evokes disturbed parent-child relationship.

Older Men's Explanatory Model for Osteoporosis

ERIC Educational Resources Information Center

Solimeo, Samantha L.; Weber, Thomas J.; Gold, Deborah T.

2011-01-01

Purpose: To explore the nature of men's experiences of osteoporosis by developing an understanding of men's explanatory models. Design and Methods: This descriptive study invited community-residing male osteoporosis patients aged 50+ to participate in interviews about osteoporosis. Participants were recruited from a hospital-affiliated bone…

Exercise, Eating, Estrogen, and Osteoporosis.

ERIC Educational Resources Information Center

Brown, Jim

1986-01-01

Osteoporosis affects millions of people, especially women. Three methods for preventing or managing osteoporosis are recommended: (1) exercise; (2) increased calcium intake; and (3) estrogen replacement therapy. (CB)

Clinical Practice. Postmenopausal Osteoporosis.

PubMed

Black, Dennis M; Rosen, Clifford J

2016-01-21

Key Clinical Points Postmenopausal Osteoporosis Fractures and osteoporosis are common, particularly among older women, and hip fractures can be devastating. Treatment is generally recommended in postmenopausal women who have a bone mineral density T score of -2.5 or less, a history of spine or hip fracture, or a Fracture Risk Assessment Tool (FRAX) score indicating increased fracture risk. Bisphosphonates (generic) and denosumab reduce the risk of hip, nonvertebral, and vertebral fractures; bisphosphonates are commonly used as first-line treatment in women who do not have contraindications. Teriparatide reduces the risk of nonvertebral and vertebral fractures. Osteonecrosis of the jaw and atypical femur fractures have been reported with treatment but are rare. The benefit-to-risk ratio for osteoporosis treatment is strongly positive for most women with osteoporosis. Because benefits are retained after discontinuation of alendronate or zoledronic acid, drug holidays after 5 years of alendronate therapy or 3 years of zoledronic acid therapy may be considered for patients at lower risk for fracture.

Osteoporosis Prevention and Management.

PubMed

Pai, Muralidhar V

2017-08-01

Osteoporosis, defined by BMD at the hip or lumbar spine that is less than or equal to 2.5 standard deviations below the mean BMD of a young-adult reference population, is the most common bone disease in humans affecting both sexes and all races. It's a silent killer affecting the quality of life due to fractures and postural changes. In osteoporosis there is an imbalance between bone formation and bone resorption in favor of latter. Preventive measures and treatments are available to combat this evil. Counseling is the integral part of prevention as well as treatment of osteoporosis. Preventive strategy includes life style changes, exercise, intake of calcium and vitamin D, avoiding alcohol, smoking and excessive intake of salt. Estrogen therapy/estrogen+progesterone therapy (ET/EPT) is no longer recommended as a first-line therapy for the prevention of osteoporosis. They may be used in the therapy for osteoporosis in women under 60. Diagnosis and classification are made by assessment of BMD using DEXA or ultrasound and laboratory investigations. Management includes estimation of 10-year fracture risk using FRAX, life style and diet modification and pharmacological therapy. The drugs used in osteoporosis may be those that inhibit bone resorption-bisphosphonates, denosumab, calcitonin, SERMs, estrogen and progesterone-or that stimulate bone formation-PTH, Teriparatide. Combination therapies are not recommended as they do not have proven additional BMD/fracture benefits. No therapy should be indefinite in duration. There are no uniform recommendations to all patients. Duration decisions need to be individualized. While on treatment monitoring should be done with BMD assessment by DEXA/ultrasound and bone turnover markers.

Building osteoporosis prevention into dental practice.

PubMed

Stewart, Stacey; Hanning, Rhona

2012-01-01

The National Report Card on Osteoporosis Care (2008) announced the need for comprehensive approaches to risk reduction and improvement in the early diagnosis of osteoporosis. Dental research has suggested that low systemic bone-mineral density also occurs in alveolar bone, and people with osteoporosis may have an increased risk of tooth loss. Whether or not a causal link exists, both conditions share similar modifiable risk factors, including a role for calcium and vitamin D. The purpose of this paper was to critically examine the role calcium and vitamin D play in the relationship between osteoporosis and the risk of tooth loss. Scientific articles were obtained through PubMed, MEDLINE, CINAHL, AgeLine and Web of Science. Publications were restricted to those involving human subjects, and English-language articles on calcium and vitamin D. The search yielded 8 articles relating to osteoporosis and tooth loss that included calcium and vitamin D intake. Despite methodological concerns, the evidence shows a relationship between osteoporosis and tooth loss for people who have an inadequate intake of calcium and vitamin D. Adequate calcium intake positively influences optimal peak bone mass and may also assist in tooth retention in later life. The dental sector can assist with national prevention strategies for osteoporosis care.

[Secondary osteoporosis induced by anticoagulants?].

PubMed

Riess, H; Loew, A; Himmelreich, G

2001-07-01

Generalized osteoporosis is a result of different causes and pathogenic mechanisms, which often combine forces to become clinically relevant. Among the different exogenic factors, drugs play an important role, frequently in connection with other factors such as immobilization or pregnancy. It has been suggested that anticoagulation therapy with heparins or coumarins may induce osteoporotic changes or enhance the development of osteoporosis for other reasons. According to in vitro experiments, preclinical trials, and clinical investigations, it seems reasonable to assume that heparins induce increased bone loss in a time- and dose-related manner. Low-molecular-weight heparins most likely have less effect on bone turnover when compared to unfractionated heparin. Oral anticoagulation therapy with vitamin K-antagonists is believed to have a weak effect on induction of osteoporosis, but clinical studies are contradictory. In spite of the fact that a relevant effect of these drugs on the induction of osteoporosis is questionable, it must be taken into consideration that anticoagulant drugs may enhance the negative effects on bone density of other risk factors capable of inducing osteoporosis such as immobilization, pregnancy, or endocrinological disorders.

The effect of genotype on methotrexate polyglutamate variability in juvenile idiopathic arthritis and association with drug response.

PubMed

Becker, Mara L; Gaedigk, Roger; van Haandel, Leon; Thomas, Bradley; Lasky, Andrew; Hoeltzel, Mark; Dai, Hongying; Stobaugh, John; Leeder, J Steven

2011-01-01

The response to and toxicity of methotrexate (MTX) are unpredictable in patients with juvenile idiopathic arthritis (JIA). Intracellular polyglutamation of MTX, assessed by measuring concentrations of MTX polyglutamates (MTXGlu), has been demonstrated to be a promising predictor of drug response. Therefore, this study was aimed at investigating the genetic predictors of MTXGlu variability and associations between MTXGlu and drug response in JIA. The study was designed as a single-center cross-sectional analysis of patients with JIA who were receiving stable doses of MTX at a tertiary care children's hospital. After informed consent was obtained from the 104 patients with JIA, blood was withdrawn during routine MTX-screening laboratory testing. Clinical data were collected by chart review. Genotyping for 34 single-nucleotide polymorphisms (SNPs) in 18 genes within the MTX metabolic pathway was performed. An ion-pair chromatographic procedure with mass spectrometric detection was used to measure MTXGlu1-7. Analysis and genotyping of MTXGlu was completed in the 104 patients. K-means clustering resulted in 3 distinct patterns of MTX polyglutamation. Cluster 1 had low red blood cell (RBC) MTXGlu concentrations, cluster 2 had moderately high RBC MTXGlu1+2 concentrations, and cluster 3 had high concentrations of MTXGlu, specifically MTXGlu3-5. SNPs in the purine and pyrimidine synthesis pathways, as well as the adenosine pathway, were significantly associated with cluster subtype. The cluster with high concentrations of MTXGlu3-5 was associated with elevated liver enzyme levels on liver function tests (LFTs), and there were higher concentrations of MTXGlu3-5 in children who reported gastrointestinal side effects and had abnormal findings on LFTs. No association was noted between MTXGlu and active arthritis. MTXGlu remains a potentially useful tool for determining outcomes in patients with JIA being treated with MTX. The genetic predictors of MTXGlu variability may also

Readout-segmented multi-shot diffusion-weighted MRI of the knee joint in patients with juvenile idiopathic arthritis.

PubMed

Sauer, Alexander; Li, Mengxia; Holl-Wieden, Annette; Pabst, Thomas; Neubauer, Henning

2017-10-12

Diffusion-weighted MRI has been proposed as a new technique for imaging synovitis without intravenous contrast application. We investigated diagnostic utility of multi-shot readout-segmented diffusion-weighted MRI (multi-shot DWI) for synovial imaging of the knee joint in patients with juvenile idiopathic arthritis (JIA). Thirty-two consecutive patients with confirmed or suspected JIA (21 girls, median age 13 years) underwent routine 1.5 T MRI with contrast-enhanced T1w imaging (contrast-enhanced MRI) and with multi-shot DWI (RESOLVE, b-values 0-50 and 800 s/mm 2 ). Contrast-enhanced MRI, representing the diagnostic standard, and diffusion-weighted images at b = 800 s/mm 2 were separately rated by three independent blinded readers at different levels of expertise for the presence and the degree of synovitis on a modified 5-item Likert scale along with the level of subjective diagnostic confidence. Fourteen (44%) patients had active synovitis and joint effusion, nine (28%) patients showed mild synovial enhancement not qualifying for arthritis and another nine (28%) patients had no synovial signal alterations on contrast-enhanced imaging. Ratings by the 1st reader on contrast-enhanced MRI and on DWI showed substantial agreement (κ = 0.74). Inter-observer-agreement was high for diagnosing, or ruling out, active arthritis of the knee joint on contrast-enhanced MRI and on DWI, showing full agreement between 1st and 2nd reader and disagreement in one case (3%) between 1st and 3rd reader. In contrast, ratings in cases of absent vs. little synovial inflammation were markedly inconsistent on DWI. Diagnostic confidence was lower on DWI, compared to contrast-enhanced imaging. Multi-shot DWI of the knee joint is feasible in routine imaging and reliably diagnoses, or rules out, active arthritis of the knee joint in paediatric patients without the need of gadolinium-based i.v. contrast injection. Possibly due to "T2w shine-through" artifacts, DWI does not reliably

Growth and weight gain in children with juvenile idiopathic arthritis: results from the ReACCh-Out cohort.

PubMed

Guzman, Jaime; Kerr, Tristan; Ward, Leanne M; Ma, Jinhui; Oen, Kiem; Rosenberg, Alan M; Feldman, Brian M; Boire, Gilles; Houghton, Kristin; Dancey, Paul; Scuccimarri, Rosie; Bruns, Alessandra; Huber, Adam M; Watanabe Duffy, Karen; Shiff, Natalie J; Berard, Roberta A; Levy, Deborah M; Stringer, Elizabeth; Morishita, Kimberly; Johnson, Nicole; Cabral, David A; Larché, Maggie; Petty, Ross E; Laxer, Ronald M; Silverman, Earl; Miettunen, Paivi; Chetaille, Anne-Laure; Haddad, Elie; Spiegel, Lynn; Turvey, Stuart E; Schmeling, Heinrike; Lang, Bianca; Ellsworth, Janet; Ramsey, Suzanne E; Roth, Johannes; Campillo, Sarah; Benseler, Susanne; Chédeville, Gaëlle; Schneider, Rayfel; Tse, Shirley M L; Bolaria, Roxana; Gross, Katherine; Feldman, Debbie; Cameron, Bonnie; Jurencak, Roman; Dorval, Jean; LeBlanc, Claire; St Cyr, Claire; Gibbon, Michele; Yeung, Rae S M; Duffy, Ciarán M; Tucker, Lori B

2017-08-22

With modern treatments, the effect of juvenile idiopathic arthritis (JIA) on growth may be less than previously reported. Our objective was to describe height, weight and body mass index (BMI) development in a contemporary JIA inception cohort. Canadian children newly-diagnosed with JIA 2005-2010 had weight and height measurements every 6 months for 2 years, then yearly up to 5 years. These measurements were used to calculate mean age- and sex-standardized Z-scores, and estimate prevalence and cumulative incidence of growth impairments, and the impact of disease activity and corticosteroids on growth. One thousand one hundred forty seven children were followed for median 35.5 months. Mean Z-scores, and the point prevalence of short stature (height < 2.5th percentile, 2.5% to 3.4%) and obesity (BMI > 95th percentile, 15.8% to 16.4%) remained unchanged in the whole cohort. Thirty-three children (2.9%) developed new-onset short stature, while 27 (2.4%) developed tall stature (>97.5th percentile). Children with systemic arthritis (n = 77) had an estimated 3-year cumulative incidence of 9.3% (95%CI: 4.3-19.7) for new-onset short stature and 34.4% (23-49.4) for obesity. Most children (81.7%) received no systemic corticosteroids, but 1 mg/Kg/day prednisone-equivalent maintained for 6 months corresponded to a drop of 0.64 height Z-scores (0.56-0.82) and an increase of 0.74 BMI Z-scores (0.56-0.92). An increase of 1 in the 10-cm physician global assessment of disease activity maintained for 6 months corresponded to a drop of 0.01 height Z-scores (0-0.02). Most children in this modern JIA cohort grew and gained weight as children in the general population. About 1 in 10 children who had systemic arthritis, uncontrolled disease and/or prolonged corticosteroid use, had increased risk of growth impairment.

[Lung is also involved in juvenile dermatomyositis].

PubMed

Pouessel, G; Thumerelle, C; Nève, V; Santangelo, T; Flammarion, S; Pruvot, I; Tillie-Leblond, I; Deschildre, A

2014-07-01

Juvenile dermatomyositis is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Lung involvement in inflammatory myopathies is well described in adults, involving mostly interstitial lung disease, aspiration pneumonia and alveolar hypoventilation. We propose to describe its specificities in children. Pulmonary involvement may be asymptomatic and therefore must be systematically screened for. In case of clinical or functional respiratory abnormality, a chest computed tomographic (CT) scan is necessary. In children, a decrease of respiratory muscle strength seems common and should be systematically and specifically searched for by non-invasive and reproducible tests (sniff test). Interstitial lung disease usually associates restrictive functional defect, impairment of carbon monoxide diffusion and interstitial lung disease on CT scan. As in adults, the first-line treatment of juvenile dermatomyositis is based on corticosteroids. Corticosteroid resistant forms require corticosteroid bolus or adjuvant immunosuppressive drugs (methotrexate or cyclosporine). There is no consensus in pediatrics for the treatment of diffuse interstitial lung disease. Complications of treatment, including prolonged steroid therapy, are frequent and therefore a careful assessment of the treatments risk-benefit ratio is necessary, especially in growing children. Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Osteoporosis education improves osteoporosis knowledge and dietary calcium: comparison of a 4 week and a one-session education course.

PubMed

Laslett, Laura L; Lynch, Joan; Sullivan, Thomas R; McNeil, Julian D

2011-08-01

Education is ideal for osteoporosis because many risk factors are modifiable. However, the efficacy of shortened education courses compared to a standard 4 week course for improving osteoporosis knowledge and healthy behaviours is not known. This study aimed to assess whether education changed knowledge and healthy behaviours over 3 months of follow-up; and whether changes in these outcomes were different between participants receiving the different education courses. Adults aged ≥ 50 years presenting to Emergency with mild to moderate trauma fractures received either the Osteoporosis Prevention and Self-Management Course (OPSMC) (4 × 2.5 h) (n = 75) or a one-session course (1 × 2.5 h) (n = 71) in a non-randomised prospective study with single-blinded allocation. Participants completed questionnaires measuring osteoporosis knowledge, dietary calcium, physical activity, calcium and exercise self-efficacy, and osteoporosis medications at baseline and 3 months. Data were analysed using mixed models and GEE regression models. Osteoporosis knowledge and calcium from food (% of RDI) increased from baseline to 3 months in both groups (P < 0.01). Use of osteoporosis medications increased between baseline and 3 months in the OPSMC group while decreasing in the one-session group (P = 0.039). There were no differences between the groups or over time in physical activity, calcium or exercise self-efficacy. Osteoporosis education (either the OPSMC or the one-session course) improved osteoporosis knowledge and calcium intake after 3 months. Participants attending the OPSMC maintained medication compliance. We were unable to determine other differences between the courses. This study confirms the value of osteoporosis education for improving osteoporosis knowledge. © 2011 The Authors. International Journal of Rheumatic Diseases © 2011 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd.

Improvement of reduced serum cartilage oligomeric matrix protein levels in systemic juvenile idiopathic arthritis patients treated with the anti-interleukin-6 receptor monoclonal antibody tocilizumab.

PubMed

Nakajima, Shoko; Naruto, Takuya; Miyamae, Takako; Imagawa, Tomoyuki; Mori, Masaaki; Nishimaki, Shigeru; Yokota, Shumpei

2009-01-01

In this study, we determined serum cartilage oligomeric matrix protein (COMP) levels in systemic juvenile idiopathic arthritis (sJIA) patients during both the active and the remission phases to investigate how the growth cartilage turnover changed under tocilizumab treatment. Specimens were collected from 201 healthy children under 16 years of age with no growth impairment, and paired sera were collected from 11 sJIA patients treated with tocilizumab. Disease activity was assessed from white blood cell count, erythrocyte sedimentation rate, C-reactive protein, and ferritin, and the COMP concentration was determined by sandwich enzyme-linked immunosorbent assay. Serum COMP concentrations were found independent of age, and the mean value in healthy children was 17.74+/-5.6 U/L. The mean serum COMP in sJIA patients during the active phase was 10.75+/-3.9 U/L, lower than that of healthy children. The mean serum COMP in the remission phase (14.89+/-3.9 U/L) was significantly higher than that in the active period (P<0.05). These results suggested that in sJIA patients, a reduced serum COMP concentration is a useful marker of active disease and growth impairment, and that the growth cartilage turnover suppressed during the active phase is improved in the remission phase under tocilizumab treatment.

A cohort study of patients with juvenile idiopathic arthritis and arthritis of the temporomandibular joint: outcome of arthrocentesis with and without the use of steroids.

PubMed

Olsen-Bergem, H; Bjørnland, T

2014-08-01

The purpose of this study was to evaluate the effects of intra-articular temporomandibular joint (TMJ) treatment in patients with juvenile idiopathic arthritis (JIA). The inclusion criteria were met by 21 patients (38 joints). Joints were randomly selected for either arthrocentesis alone (n=17) or arthrocentesis with the additional use of triamcinolone hexacetonide (n=21) using a closed single-needle system. Measurements of pain and function were performed at baseline and at follow-up after 3 and 8 months. Pain on opening and lateral excursion improved significantly after injections. Pain decreased significantly from baseline to first and second control on a visual analogue scale (VAS) for overall pain (49-18-8) and overall function (41-19-4). Significant improvement was recorded for pain on palpation of muscles and joints. There was no statistically significant difference between the treatment modalities, with or without glucocorticoid injection. Arthrocentesis in the TMJ treatment of patients with JIA may be beneficial and steroids had no additional effect. Further studies are needed to evaluate the long-term effects on the TMJ structures and on condylar growth from arthrocentesis and intra-articular steroid injections. Copyright © 2014 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

An overview and management of osteoporosis

PubMed Central

Sözen, Tümay; Özışık, Lale; Başaran, Nursel Çalık

2017-01-01

Osteoporosis -related to various factors including menopause and aging- is the most common chronic metabolic bone disease, which is characterized by increased bone fragility. Although it is seen in all age groups, gender, and races, it is more common in Caucasians (white race), older people, and women. With an aging population and longer life span, osteoporosis is increasingly becoming a global epidemic. Currently, it has been estimated that more than 200 million people are suffering from osteoporosis. According to recent statistics from the International Osteoporosis Foundation, worldwide, 1 in 3 women over the age of 50 years and 1 in 5 men will experience osteoporotic fractures in their lifetime. Every fracture is a sign of another impending one. Osteoporosis has no clinical manifestations until there is a fracture. Fractures cause important morbidity; in men, in particular, they can cause mortality. Moreover, osteoporosis results in a decreased quality of life, increased disability-adjusted life span, and big financial burden to health insurance systems of countries that are responsible for the care of such patients. With an early diagnosis of this disease before fractures occur and by assessing the bone mineral density and with early treatment, osteoporosis can be prevented. Therefore, increasing awareness among doctors, which, in turn, facilitates increase awareness of the normal populace, will be effective in preventing this epidemic. PMID:28293453

Causes, mechanisms and management of paediatric osteoporosis.

PubMed

Mäkitie, Outi

2013-08-01

Osteoporosis, a skeletal disorder characterized by compromised bone strength and an increased risk of fractures, is an important paediatric disorder that involves almost all paediatric subspecialties. Osteogenesis imperfecta is the most common form of childhood-onset primary osteoporosis, but several other forms are also known. Secondary osteoporosis is caused by an underlying chronic illness or its treatment. The most common causes of secondary osteoporosis include chronic systemic inflammation, glucocorticoid use and neuromuscular disabilities. The skeletal sequelae can present in childhood as low-energy peripheral and vertebral fractures, or become evident in adulthood as low bone mass and an increased propensity to develop osteoporosis. Management should aim at prevention, as interventions to treat symptomatic osteoporosis in the paediatric age group are scarce. Bisphosphonates are the principal pharmacological agents that can be used in this setting, but data on their efficacy and safety in paediatric populations remain inadequate, especially in patients with secondary osteoporosis. Consequently, it is important to understand the potential skeletal effects of paediatric illnesses and their therapies in order to institute effective and timely prevention of skeletal complications.

Influence of polymorphisms within the methotrexate pathway genes on the toxicity and efficacy of methotrexate in patients with juvenile idiopathic arthritis.

PubMed

Yanagimachi, Masakatsu; Naruto, Takuya; Hara, Takuma; Kikuchi, Masako; Hara, Ryoki; Miyamae, Takako; Imagawa, Tomoyuki; Mori, Masaaki; Kaneko, Tetsuji; Morita, Satoshi; Goto, Hiroaki; Yokota, Shumpei

2011-02-01

We investigated whether several polymorphisms within the methotrexate (MTX) pathway genes were related to the toxicity and efficacy of MTX in 92 Japanese patients with articular-type juvenile idiopathic arthritis (JIA). Eight gene polymorphisms within the MTX pathway genes, namely, RFC, BCRP, MTHFR (two), FPGS, γ-glutamyl hydrolase (GGH; two) and ATIC, were genotyped using TaqMan assays. Liver dysfunction was defined as an increase in alanine transaminase to five times the normal upper limit. Non-responders to MTX were defined as patients refractory to MTX and were therefore treated with biologics. The non-TT genotype at GGH T16C was associated with a high risk of liver dysfunction (P=0.028, odds ratio=6.90, 95% confidence interval 1.38-34.5), even after adjustment for the duration of MTX treatment. A longer interval from disease onset to treatment (8.5 and 21.3 months, P=0.029) and rheumatoid factor positivity (P=0.026, odds ratio=2.87, 95% confidence interval 1.11-7.39) were associated with lower efficacy of MTX. The non-TT genotype at GGH T16C was associated with a high risk of liver dysfunction, presumably because the C allele of GGH C16T may reduce the activity of GGH. The time interval before MTX treatment and rheumatoid factor positivity were associated with the efficacy of MTX treatment. The pharmacogenetics of the MTX pathway genes affects the toxicity and efficacy of MTX in Japanese JIA patients. © 2011 The Authors. British Journal of Clinical Pharmacology © 2011 The British Pharmacological Society.

Influence of polymorphisms within the methotrexate pathway genes on the toxicity and efficacy of methotrexate in patients with juvenile idiopathic arthritis

PubMed Central

Yanagimachi, Masakatsu; Naruto, Takuya; Hara, Takuma; Kikuchi, Masako; Hara, Ryoki; Miyamae, Takako; Imagawa, Tomoyuki; Mori, Masaaki; Kaneko, Tetsuji; Morita, Satoshi; Goto, Hiroaki; Yokota, Shumpei

2011-01-01

AIMS We investigated whether several polymorphisms within the methotrexate (MTX) pathway genes were related to the toxicity and efficacy of MTX in 92 Japanese patients with articular-type juvenile idiopathic arthritis (JIA). METHODS Eight gene polymorphisms within the MTX pathway genes, namely, RFC, BCRP, MTHFR (two), FPGS, γ-glutamyl hydrolase (GGH; two) and ATIC, were genotyped using TaqMan assays. Liver dysfunction was defined as an increase in alanine transaminase to five times the normal upper limit. Non-responders to MTX were defined as patients refractory to MTX and were therefore treated with biologics. RESULTS The non-TT genotype at GGH T16C was associated with a high risk of liver dysfunction (P = 0.028, odds ratio = 6.90, 95% confidence interval 1.38–34.5), even after adjustment for the duration of MTX treatment. A longer interval from disease onset to treatment (8.5 and 21.3 months, P = 0.029) and rheumatoid factor positivity (P = 0.026, odds ratio = 2.87, 95% confidence interval 1.11–7.39) were associated with lower efficacy of MTX. CONCLUSIONS The non-TT genotype at GGH T16C was associated with a high risk of liver dysfunction, presumably because the C allele of GGH C16T may reduce the activity of GGH. The time interval before MTX treatment and rheumatoid factor positivity were associated with the efficacy of MTX treatment. The pharmacogenetics of the MTX pathway genes affects the toxicity and efficacy of MTX in Japanese JIA patients. PMID:21219404

Promoting physical activity in children with juvenile idiopathic arthritis through an internet-based program: results of a pilot randomized controlled trial.

PubMed

Lelieveld, Otto T H M; Armbrust, Wineke; Geertzen, Jan H B; de Graaf, Inez; van Leeuwen, Miek A; Sauer, Pieter J J; van Weert, Ellen; Bouma, Jelte

2010-05-01

Patients with juvenile idiopathic arthritis (JIA) are less physically active than healthy peers. Therefore, we developed an Internet-based intervention to improve physical activity (PA). The aim of this study was to examine the effectiveness of the program in improving PA. PA was determined by activity-related energy expenditure, PA level, time spent on moderate to vigorous PA, and the number of days with > or =1 hour of moderate to vigorous activity, and was assessed with a 7-day activity diary. Aerobic exercise capacity was assessed by means of a Bruce treadmill test and was recorded as maximum endurance time. Disease activity was assessed by using the JIA core set. Adherence was electronically monitored. Of 59 patients, 33 eligible patients were included and randomized in an intervention (n = 17, mean +/- SD age 10.6 +/- 1.5 years) or control waiting-list group (n = 16, mean +/- SD age 10.8 +/- 1.4 years). All patients completed baseline and T1 testing. PA significantly improved in both groups. Maximum endurance time significantly improved in the intervention group but not in the control group. In a subgroup analysis for patients with low PA (intervention: n = 7, control: n = 5), PA improved in the intervention group but not in the control group. The intervention was safe, feasible, and showed a good adherence. An Internet-based program for children with JIA ages 8-12 years directed at promoting PA in daily life effectively improves PA in those patients with low PA levels. It is also able to improve endurance and it is safe, feasible, and has good adherence.

The impact of disease activity and tumour necrosis factor-α inhibitor therapy on cytokine levels in juvenile idiopathic arthritis.

PubMed

Walters, H M; Pan, N; Lehman, T J A; Adams, A; Kalliolias, G D; Zhu, Y S; Santiago, F; Nguyen, J; Sitaras, L; Cunningham-Rundles, S; Walsh, T J; Toussi, S S

2016-06-01

The aim of this study was to evaluate prospectively cytokine levels and disease activity in juvenile idiopathic arthritis (JIA) patients treated with and without tumour necrosis factor (TNF)-α inhibitors. TNF-α inhibitor-naive JIA subjects were followed prospectively for 6 months. Cytokine levels of TNF-α, interleukin (IL)-1β, IL-6, IL-8, IL-10 and IL-17 were measured at baseline for JIA subjects and healthy controls (HCs). Cytokine levels were then measured at four time-points after initiation of TNF-α inhibition for anti-TNF-α-treated (anti-TNF) JIA subjects, and at two subsequent time-points for other JIA (non-TNF) subjects. JIA disease activity by Childhood Health Assessment Questionnaire (CHAQ) disability index/pain score and physician joint count/global assessment was recorded. Sixteen anti-TNF, 31 non-TNF and 16 HCs were analysed. Among JIA subjects, those with higher baseline disease activity (subsequent anti-TNFs) had higher baseline TNF-α, IL-6 and IL-8 than those with lower disease activity (non-TNFs) (P < 0·05). TNF-α and IL-10 increased, and IL-6 and IL-8 no longer remained significantly higher after TNF-α inhibitor initiation in anti-TNF subjects. Subgroup analysis of etanercept versus adalimumab-treated subjects showed that TNF-α and IL-17 increased significantly in etanercept but not adalimumab-treated subjects, despite clinical improvement in both groups of subjects. JIA subjects with increased disease activity at baseline had higher serum proinflammatory cytokines. TNF-α inhibition resulted in suppression of IL-6 and IL-8 in parallel with clinical improvement in all anti-TNF-treated subjects, but was also associated with elevated TNF-α and IL-17 in etanercept-treated subjects. © 2016 British Society for Immunology.

Fine-mapping the MHC locus in juvenile idiopathic arthritis (JIA) reveals genetic heterogeneity corresponding to distinct adult inflammatory arthritic diseases

PubMed Central

Hinks, A; Cobb, J; Ainsworth, H C; Marion, M C; Comeau, M E; Sudman, M; Han, B; Becker, M L; Bohnsack, J F; de Bakker, P I W; Haas, J P; Hazen, M; Lovell, D J; Nigrovic, P A; Nordal, E; Punnaro, M; Rosenberg, A M; Rygg, M; Wise, C A; Videm, V; Wedderburn, L R; Yarwood, A; Yeung, R S M; Prahalad, S; Langefeld, C D; Raychaudhuri, S; Thompson, S D; Thomson, W

2017-01-01

Objectives Juvenile idiopathic arthritis (JIA) is a heterogeneous group of diseases, comprising seven categories. Genetic data could potentially be used to help redefine JIA categories and improve the current classification system. The human leucocyte antigen (HLA) region is strongly associated with JIA. Fine-mapping of the region was performed to look for similarities and differences in HLA associations between the JIA categories and define correspondences with adult inflammatory arthritides. Methods Dense genotype data from the HLA region, from the Immunochip array for 5043 JIA cases and 14 390 controls, were used to impute single-nucleotide polymorphisms, HLA classical alleles and amino acids. Bivariate analysis was performed to investigate genetic correlation between the JIA categories. Conditional analysis was used to identify additional effects within the region. Comparison of the findings with those in adult inflammatory arthritic diseases was performed. Results We identified category-specific associations and have demonstrated for the first time that rheumatoid factor (RF)-negative polyarticular JIA and oligoarticular JIA are genetically similar in their HLA associations. We also observe that each JIA category potentially has an adult counterpart. The RF-positive polyarthritis association at HLA-DRB1 amino acid at position 13 mirrors the association in adult seropositive rheumatoid arthritis (RA). Interestingly, the combined oligoarthritis and RF-negative polyarthritis dataset shares the same association with adult seronegative RA. Conclusions The findings suggest the value of using genetic data in helping to classify the categories of this heterogeneous disease. Mapping JIA categories to adult counterparts could enable shared knowledge of disease pathogenesis and aetiology and facilitate transition from paediatric to adult services. PMID:27998952

Long-Term Health-Related Quality of Life in German Patients with Juvenile Idiopathic Arthritis in Comparison to German General Population

PubMed Central

Barth, Swaantje; Haas, Johannes-Peter; Schlichtiger, Jenny; Molz, Johannes; Bisdorff, Betty; Michels, Hartmut; Hügle, Boris; Radon, Katja

2016-01-01

Objective Aims of the study were to investigate health-related quality of life (HRQOL) in adult patients with former diagnosis of Juvenile Idiopathic Arthritis (JIA), to compare their HRQOL with the general population and to identify factors related to a poor outcome. Methods In 2012, a cross-sectional survey was performed by mailing a questionnaire to a large cohort of former and current patients of the German Centre for Rheumatology in Children and Adolescents. Only adult patients (≥18 years) with a diagnosis compatible with JIA were included (n = 2592; response 66%). The questionnaire included information about HRQOL (EQ5D), disease-related questions and socio-demographics. Prevalence and 95% confidence intervals (CI) of problems with mobility, self-care, usual activities, pain and anxiety/depression were standardized to the German general population. Factors associated with low HRQOL in JIA patients were identified using logistic regression models. Results Sixty-two percent of the study population was female; age range was 18–73 years. In all dimensions, JIA patients reported statistically significantly more problems than the general population with largest differences in the pain dimension (JIA patients 56%; 95%CI 55–58%; general population 28%; 26–29%) and the anxiety/depression dimension (28%; 27–29% vs. 4%; 4–5%). Lower HRQOL in JIA patients was associated with female sex, older age, lower level of education, still being under rheumatic treatment and disability. Conclusions HRQOL in adult JIA patients is considerably lower than in the general population. As this cohort includes historic patients the new therapeutic schemes available today are expected to improve HRQOL in future. PMID:27115139

Osteoporosis-related life habits and knowledge about osteoporosis among women in El Salvador: A cross-sectional study

PubMed Central

Hernandez-Rauda, Roberto; Martinez-Garcia, Sandra

2004-01-01

Background Osteoporosis is a systemic skeletal disorder, characterized by reduced bone mass, deterioration of bone structure, increased bone fragility, and increased fracture risk. It is more frequent to find among women than men at a 4:1 ratio. Evidence suggests that to adopt changes on some life habits can prevent or delay development of osteoporosis. Several osteoporosis-risk factors have been confirmed in the US and western Europe, but in El Salvador there are neither reliable epidemiological statistics about this skeletal disorder nor studies addressing osteoporosis-risk factors in women. The aim of this study was to determinate the extent of osteoporosis knowledge, the levels of both daily calcium intake and weight-bearing physical activity, and the influence of several osteoporosis-risk factors on these variables in three age groups of Salvadorean women. Methods In this exploratory cross-sectional study, an osteoporosis knowledge assessment questionnaire incluiding a food frequency and a physical activity record section were used to collect data and it was delivered through a face-to-face interview. A convenience sample (n = 197) comprised of three groups of women aged 25–35 years, 36–49 years, and over 49 years was taken. Among-group comparisons of means were analyzed by two-way ANOVA. To determinate the overall influence of osteoporosis-risk factors, the multivariate analysis was used. Results Study results indicated that better educated women had more knowledge about osteoporosis than women with a low education level, regardless of age, even though this knowledge was rather fair. Older women got more weight-bearing physical activity at home and less at place of employment than reported by the younger women; however, neither group performed sufficient high-intensity WBPA to improve bone mass. Regardless of age, the most women consumed 60% or less than the Dietary Reference Intake of calcium and depend on household income, lactose intolerance and coffee

Osteoporosis in pregnancy: more than postural backache.

PubMed

Topping, J; Black, A J; Farquharson, R G; Fraser, W D

1998-01-01

Though uncommon, osteoporosis can occur in pregnancy or shortly after delivery. The most common feature is back pain, often severely disabling. Suspect osteoporosis if pain of sudden onset in the upper lumbar or thoracic spine is not relieved by simple analgesia, or if there is a noticeable loss of height. X-rays reveal low bone density and fractures of the vertebrae. 70% of cases occur in first pregnancies. Recurrence is unusual. Most cases resolve spontaneously; a minority cause disability lasting months or years. If osteoporosis is diagnosed, breast feeding should be discouraged because of its effect on bone mineral density. Anyone who has had osteoporosis of pregnancy is at risk of postmenopausal osteoporosis and should take medical advice.

Osteoporosis and Periodontitis.

PubMed

Wang, Chin-Wei Jeff; McCauley, Laurie K

2016-12-01

Osteoporosis and periodontitis are both diseases characterized by bone resorption. Osteoporosis features systemic degenerative bone loss that leads to loss of skeletal cancellous microstructure and subsequent fracture, whereas periodontitis involves local inflammatory bone loss, following an infectious breach of the alveolar cortical bone, and it may result in tooth loss. Most cross-sectional studies have confirmed the association of osteoporosis and periodontitis primarily on radiographic measurements and to a lesser degree on clinical parameters. Multiple shared risk factors include age, genetics, hormonal change, smoking, as well as calcium and vitamin D deficiency. Both diseases could also be risk factors for each other and have a mutual impact that requires concomitant management. Suggested mechanisms underlying the linkage are disruption of the homeostasis concerning bone remodeling, hormonal balance, and inflammation resolution. A mutual interventional approach is emerging with complex treatment interactions. Prevention and management of both diseases require interdisciplinary approaches and warrants future well-controlled longitudinal and interventional studies for evidence-based clinical guidelines.

Association between tea consumption and osteoporosis

PubMed Central

Sun, Kang; Wang, Le; Ma, Qingping; Cui, Qiaoyun; Lv, Qianru; Zhang, Wenzheng; Li, Xinghui

2017-01-01

Abstract Background: Previous reports have suggested a potential association of tea consumption with the risk of osteoporosis. As such association is controversial, we conducted a meta-analysis to assess the relationship between tea consumption and osteoporosis. Methods and Findings: We systematically searched PubMed, EMBASE and WanFang databases until March 30, 2016, using the keywords “tea and osteoporosis,” without limits of language. Odds ratios (ORs) with 95% confidence intervals (95% CIs) were derived by using random-effects models throughout the analyses. We conducted the analysis of the statistical heterogeneity using Cochrane I2. The funnel plot was used to speculate the publication bias, while the subgroup analysis and multiround elimination method were employed. Results: Our study was based on 17 journal articles, including 2 prospective cohort studies, 4 case–control studies, and 11 cross-sectional studies. In the present study, the total OR of osteoporosis for the highest versus the lowest categories of tea consumption was 0.62 (95% CI, 0.46–0.83), with significant heterogeneity among studies (I2 = 94%, P < .01). There was, however, no publication bias of the meta-analysis about tea consumption and osteoporosis. Subgroup analysis showed that tea consumption could reduce the risk of osteoporosis in all examined subgroups. Conclusion: In the present study, it can be concluded from the results that tea consumption can reduce the risk of osteoporosis. PMID:29245297

Unstimulated salivary flow, pH, proteins and oral health in patients with Juvenile Idiopathic Arthritis.

PubMed

Kobus, Agnieszka; Kierklo, Anna; Zalewska, Anna; Kuźmiuk, Anna; Szajda, Sławomir Dariusz; Ławicki, Sławomir; Bagińska, Joanna

2017-06-02

There have been inconsistent conclusions regarding salivary abnormalities and their effect on oral health of Juvenile Idiopathic Arthritis (JIA) patients. The purpose of the study was to evaluate the flow rate and selected biochemical parameters of unstimulated whole saliva in correlation to oral health in JIA children. Thirty-four JIA patients and 34 age- and sex-matched controls not affected by JIA (C) were divided into two groups: with mixed and permanent dentition. DMFT/dmft, gingival and simplified oral hygiene indices were evaluated. Salivary flow rate, pH, lysozyme, lactoferrin, salivary protein concentrations and peroxidase activity were assessed. The salivary flow rate was significantly lower in the total JIA group (0.41 ml/min) as compared with the C (0.51 ml/min) and in the permanent dentition of JIA children (0.43 ml/min) as compared with the C (0.61 ml/min). A significantly lower pH was observed in total (6.74), mixed (6.7) and permanent (6.76) dentition of JIA groups in comparison to the C (7.25, 7.21, 7.28 respectively). The specific activity of peroxidase was significantly higher in JIA patients (total 112.72 IU/l, mixed dentition 112.98 IU/l, permanent dentition 112.5 IU/l) than in the C group (total 70.03 IU/l, mixed dentition 71.83 IU/l, permanent dentition 68.61 IU/l). The lysozyme concentration in JIA patients (total and permanent dentition groups) was significantly higher than in the C group. There were no significant differences in lactoferrin and salivary protein concentrations. There were no statistically significant differences in oral status between JIA patients and C, respectively: DMFT = 5.71, dmft = 3.73, OHI-S = 0.95, GI = 0.25 and DMFT 5.71, dmft = 3.73, OHI-S = 0.85, GI = 0.24. The specific activity of peroxidase in the unstimulated whole saliva was inversely correlated with the GI index, whereas the salivary lysozyme concentration was inversely correlated with the dmft index in JIA patients. In

Spine mineral change during osteoporosis therapy

DOE Office of Scientific and Technical Information (OSTI.GOV)

Powell, M.R.; Kolb, F.O.; Meier, K.A.

1985-05-01

Osteoporosis therapy has been handicapped by lack of means to quantitate the process. Dual photon absorptiometry (DPA) offers accurate (4%) and precise (2%) estimation of lumbar spine mineral. The authors followed 42 osteoporotics to determine response to therapy. There were 17 patients with normal menopause (NM), 4 with surgical menopause (SM), 3 with premature menopause (PM), and 18 with idiopathic osteoporoses (10). Intervals between DPA spine mineral estimation were 16.5 +- 5.2 mo. for NM, 14.3 +- 8.4 mo. for SM, 14.0 +- 7.5 mo. for PM and 16.7 +- 5.8 mo. for 10. Observed average percent change of spinemore » mineral under therapy for those intervals was 5.2 +- 7.9% for NM, +7.3 +- 1.7% for SM, -2.4 +- 6.3% for PM and +1.8 +- 12.3% for 10. Therapy invariably was with Ca, low dose Premarin in NM and PM, often with phosphates in IO, sometimes with thiazides, often with Vitamin D and with occasional other modalities, including NaF. The authors find DPA is a cost-effective way to measure osteopenia in the osteoporeses, document response to therapy, identify need for therapy change when there is continued bone loss under therapy, and to encourage the patient's compliance with long-term, complex therapies.« less

Frequency of auditory involvement and of associated factors in patients with juvenile idiopathic arthritis.

PubMed

Céspedes Cruz, Adriana Ivonne; Méndez Núñez, Myriam; Solís Vallejo, Eunice; Zeferino Cruz, Maritza; Torres Jiménez, Alfonso Ragnar; Ocampo Sánchez, Verónica; Flores Meza, Beatriz; Quintana Ruiz, Norma

2017-09-08

Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disease characterized by the presence of arthritis in children under 16 years of age for more than 6 weeks in the absence of any other known cause. The extra-articular manifestations, especially in the audiovestibular system, are related to the involvement of the joints of the ossicular chain as a result of the inflammatory process in the synovium. Previous clinical studies in pediatric patients have shown conductive or sensorineural hearing loss. The aim of this study was to assess the frequency of hearing impairment and of associated factors in patients with JIA. A prospective, analytical study was conducted from January 2013 to August 2014 in 62 patients with JIA aged between 5 and 15 years. The study was approved by the local ethics committee and parents signed their informed consent. All subjects underwent audiological examination involving otomicroscopy, audiometry, tympanometry, stapedius reflex and test for transient otoacoustic emissions (TOAE); rheumatologic evaluation included joint examination and the application of a measure of functional ability (disability) using the Childhood Health Assessment Questionnaire (CHAQ). Measures of central tendency and of dispersion were used (chi-square for associations and P<.05 for statistical significance). Sixty-two patients were included: 56 girls and 6 boys, mean age 11.9 years and mean disease duration of 3.4 years; 46% had rheumatoid factor (RF)- positive polyarticular JIA, 40% had RF-negative polyarticular JIA, 15% had disease of systemic onset and 3% had oligoarthritis. Active disease was found in 29 patients and 33 were in remission with medication. Of the total of 124 ears evaluated according to the Jerger classification for tympanometry, abnormal findings were observed in 78 that were type As and in 1 that was type Ad, whereas there were 45 type A ears. Hearing loss was disclosed by speech audiometry, rather than by pure tone audiometry. The TOAE

[Severe osteoporosis in a patient with polycythaemia vera].

PubMed

Farmer, Sarah; Hermann, Anne Pernille; Vestergaard, Hanne

2014-07-14

This case report is about severe osteoporosis in a woman known with polycythaemia vera (PV). A 51-year-old woman with hereditary predisposition to osteoporosis had a dual X-ray absorptiometry scan without osteoporosis. Only one year later she was diagnosed with PV. Re-scan eight years later showed progression to severe osteoporosis. Whether this is secondary osteoporosis, initiated or accelerated by the presence of PV is not known. A recent Danish registry study has shown that PV patients have an increased incidence of fractures compared with the general population.

[Fetal programming and the etiology of osteoporosis].

PubMed

Pieńkowski, Wojciech; Wolski, Hubert; Drews, Krzysztof; Seremak-Mrozikiewicz, Agnieszka

2015-08-01

Osteoporosis is a multifactorial skeletal disorder characterized by low bone mass and microarchitectural deterioration of bone tissue, resulting in increased risk of fracture. Peak bone mass is an important predictor of later risk of osteoporosis. Epidemiological studies revealed that the risk of osteoporosis might be modified by exposure to environmental factors during intrauterine life and early postnatal period. This review summarizes the influence of fetal programming on the development of osteoporosis based on the epidemiological studies and potential mechanisms of epigenetic regulation of gene expression.

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

MedlinePlus

... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

What do men who are at risk of osteoporosis know about osteoporosis in developing countries? A pilot study in Isparta, Turkey.

PubMed

Cindaş, Abdullah; Savaş, Serpil

2004-06-01

The aim of this study was to investigate the knowledge, health beliefs and the knowledge sources of osteoporosis in Turkish males who are at risk of secondary causes of osteoporosis. One hundred and sixty men who had one of the systemic diseases or drugs which negatively affects bone metabolism were included in the study. Patients were asked to fill in a self-administered questionnaire which was modified from a validated questionnaire for Turkish female patients with osteoporosis. Twenty-two patients (15.7%) had never heard of a disease named osteoporosis. The mean score for general knowledge was 31.86 +/- 20.56 (over 100 points). Only 20 (16.9%) patients had received information from a doctor. Seventy-three (61.8%) of the patients stated that osteoporosis may be seen in men and only 42 (35.6%) patients were aware that they had a risk factor for osteoporosis. Most of the patients were unable to identify significant risk factors. Statistically significant positive correlation was found between KOS and patients' education levels (r = 0.453, p < 0.01). The findings in our study show that Turkish men who are at risk of osteoporosis do not have sufficient knowledge about osteoporosis and its consequences. We conclude that low education level of our patients accounts for poor osteoporosis knowledge.

[Pharmacologic treatment of osteoporosis--2011].

PubMed

Lakatos, Péter

2011-08-14

Osteoporosis affects approximately 9% of the population in Hungary resulting in about 100 000 osteoporotic fractures annually. Thirty-five percent of patients with hip fractures due to osteoporosis will die within 1 year. Direct costs of osteoporosis exceed 25 billion forints per year. Apparently, cost-effective reduction of bone loss and consequent fracture risk will add up to not only financial savings but improvement in quality of life, as well. A number of pharmacological modalities are available for this purpose. The mainstay of the treatment of osteoporosis is the bisphosphonate group that includes effective anti-resorptive compounds mitigating bone loss and fragility. The recently registered denosumab exhibits similar efficacy by neutralizing RANK ligand, however, marked differences can be observed between the two drug classes. Strontium has a unique mechanism of action by rebalancing bone turnover, and thus, providing an efficient treatment option for the not fast bone losers who are at high fracture risk. The purely anabolic teriparatide is available for the extremely severe osteoporotic patients and for those who do not respond to other types of therapy. Older treatment options such as hormone replacement therapy, raloxifene, tibolone or calcitonin may also have a restricted place in the management of osteoporosis.

Burden of osteoporosis and fractures.

PubMed

Keen, Richard W

2003-09-01

Osteoporosis currently affects up to one in three women and one in 12 men. In 1990, there were 1.6 million hip fractures per annum worldwide and this number is estimated to reach 6 million by 2050. This increase in the number of fractures is due to an increase in the number of elderly people in the population, improved survival, and an increase in the age-specific fracture rates of unknown etiology. The rising number of osteoporotic fractures and their associated morbidity will place a heavy burden on future health care resources. In the United States, the cost for the management of osteoporosis has been estimated at $17 billion. The majority of this cost is spent on the acute surgical and medical management following hip fracture, and the subsequent rehabilitation. Currently, only minimal costs are utilized for treatment and prevention of osteoporosis. Hopefully, however, an accurate assessment of the burden of osteoporosis on the individual and the health care system will enable the targeting of resources to tackle this growing problem. With an increasing number of effective pharmaceutical interventions, it is critical that these agents are targeted to those at greatest risk for future fracture. This will ultimately reduce the burden of osteoporosis in future years.

Wnt signaling and osteoporosis

PubMed Central

Manolagas, Stavros C.

2014-01-01

Major advances in understanding basic bone biology and the cellular and molecular mechanisms responsible for the development of osteoporosis, over the last 20 years, have dramatically altered the management of this disease. The purpose of this mini-review is to highlight the seminal role of Wnt signaling in bone homeostasis and disease and the emergence of novel osteoporosis therapies by targeting Wnt signaling with drugs. PMID:24815296

MRI of the wrist in juvenile idiopathic arthritis: proposal of a paediatric synovitis score by a consensus of an international working group. Results of a multicentre reliability study.

PubMed

Damasio, Maria Beatrice; Malattia, Clara; Tanturri de Horatio, Laura; Mattiuz, Chiara; Pistorio, Angela; Bracaglia, Claudia; Barbuti, Domenico; Boavida, Peter; Juhan, Karen Lambot; Ording, Lil Sophie Mueller; Rosendahl, Karen; Martini, Alberto; Magnano, GianMichele; Tomà, Paolo

2012-09-01

MRI is a sensitive tool for the evaluation of synovitis in juvenile idiopathic arthritis (JIA). The purpose of this study was to introduce a novel MRI-based score for synovitis in children and to examine its inter- and intraobserver variability in a multi-centre study. Wrist MRI was performed in 76 children with JIA. On postcontrast 3-D spoiled gradient-echo and fat-suppressed T2-weighted spin-echo images, joint recesses were scored for the degree of synovial enhancement, effusion and overall inflammation independently by two paediatric radiologists. Total-enhancement and inflammation-synovitis scores were calculated. Interobserver agreement was poor to moderate for enhancement and inflammation in all recesses, except in the radioulnar and radiocarpal joints. Intraobserver agreement was good to excellent. For enhancement and inflammation scores, mean differences (95 % CI) between observers were -1.18 (-4.79 to 2.42) and -2.11 (-6.06 to 1.83). Intraobserver variability (reader 1) was 0 (-1.65 to 1.65) and 0.02 (-1.39 to 1.44). Intraobserver agreement was good. Except for the radioulnar and radiocarpal joints, interobserver agreement was not acceptable. Therefore, the proposed scoring system requires further refinement.

COPD and osteoporosis: links, risks, and treatment challenges.

PubMed

Inoue, Daisuke; Watanabe, Reiko; Okazaki, Ryo

2016-01-01

Chronic obstructive pulmonary disease (COPD) is a chronic inflammatory airway disease associated with various systemic comorbidities including osteoporosis. Osteoporosis and its related fractures are common and have significant impacts on quality of life and even respiratory function in patients with COPD. COPD-associated osteoporosis is however extremely undertreated. Recent studies have suggested that both decreased bone mineral density (BMD) and impaired bone quality contribute to bone fragility, causing fractures in COPD patients. Various clinical risk factors of osteoporosis in COPD patients, including older age, emaciation, physical inactivity, and vitamin D deficiency, have also been described. It is critically important for pulmonologists to be aware of the high prevalence of osteoporosis in COPD patients and evaluate them for such fracture risks. Routine screening for osteoporosis will enable physicians to diagnose COPD patients with comorbid osteoporosis at an early stage and give them appropriate treatment to prevent fracture, which may lead to improved quality of life as well as better long-term prognosis.

The burden of illness of osteoporosis in Canada.

PubMed

Tarride, J-E; Hopkins, R B; Leslie, W D; Morin, S; Adachi, J D; Papaioannou, A; Bessette, L; Brown, J P; Goeree, R

2012-11-01

To update the 1993 burden of illness of osteoporosis in Canada, administrative and community data were used to calculate the 2010 costs of osteoporosis at $2.3 billion in Canada or 1.3% of Canada's healthcare expenditures. Prevention of fractures in high-risk individuals is key to decrease the financial burden of osteoporosis. Since the 1996 publication of the burden of osteoporosis in 1993 in Canada, the population has aged and the management of osteoporosis has changed. The study purpose was to estimate the current burden of illness due to osteoporosis in Canadians aged 50 and over. Analyses were conducted using five national administrative databases from the Canadian Institute for Health Information for the fiscal-year ending March 31 2008 (FY 2007/2008). Gaps in national data were supplemented by provincial and community data extrapolated to national levels. Osteoporosis-related fractures were identified using a combination of most responsible diagnosis at discharge and intervention codes. Fractures associated with severe trauma codes were excluded. Costs, expressed in 2010 dollars, were calculated for osteoporosis-related hospitalizations, emergency care, same day surgeries, rehabilitation, continuing care, homecare, long-term care, prescription drugs, physician visits, and productivity losses. Sensitivity analyses were conducted to measure the impact on the results of key assumptions. Osteoporosis-related fractures were responsible for 57,413 acute care admissions and 832,594 hospitalized days in FY 2007/2008. Acute care costs were estimated at $1.2 billion. When outpatient care, prescription drugs, and indirect costs were added, the overall yearly cost of osteoporosis was over $2.3 billion for the base case analysis and as much as $3.9 billion if a proportion of Canadians were assumed to be living in long-term care facilities due to osteoporosis. Osteoporosis is a chronic disease that affects a large segment of the adult population and results in a

SCOPE: a scorecard for osteoporosis in Europe.

PubMed

Kanis, J A; Borgström, F; Compston, J; Dreinhöfer, K; Nolte, E; Jonsson, L; Lems, W F; McCloskey, E V; Rizzoli, R; Stenmark, J

2013-01-01

The scorecard summarises key indicators of the burden of osteoporosis and its management in each of the member states of the European Union. The resulting scorecard elements were then assembled on a single sheet to provide a unique overview of osteoporosis in Europe. The scorecard for osteoporosis in Europe (SCOPE) is an independent project that seeks to raise awareness of osteoporosis care in Europe. The aim of this project was to develop a scorecard and background documents to draw attention to gaps and inequalities in the provision of primary and secondary prevention of fractures due to osteoporosis. The SCOPE panel reviewed the information available on osteoporosis and the resulting fractures for each of the 27 countries of the European Union (EU27). The information researched covered four domains: background information (e.g. the burden of osteoporosis and fractures), policy framework, service provision and service uptake e.g. the proportion of men and women at high risk that do not receive treatment (the treatment gap). There was a marked difference in fracture risk among the EU27. Of concern was the marked heterogeneity in the policy framework, service provision and service uptake for osteoporotic fracture that bore little relation to the fracture burden. For example, despite the wide availability of treatments to prevent fractures, in the majority of the EU27, only a minority of patients at high risk receive treatment for osteoporosis even after their first fracture. The elements of each domain in each country were scored and coded using a traffic light system (red, orange, green) and used to synthesise a scorecard. The resulting scorecard elements were then assembled on a single sheet to provide a unique overview of osteoporosis in Europe. The scorecard will enable healthcare professionals and policy makers to assess their country's general approach to the disease and provide indicators to inform future provision of healthcare.

Vocal cord paralysis: What matters between idiopathic and non-idiopathic cases?

PubMed

Özbal Koç, Ayça Eltaf; Türkoğlu, Seda Babakurban; Erol, Ozan; Erbek, Selim

2016-01-01

This study aims to evaluate the demographic and clinical characteristics of patients with idiopathic and non-idiopathic vocal cord paralysis (VCP). This retrospective cohort was performed on data extracted from medical files of 92 consecutive patients (43 males, 49 females; median age 52.1±23.1 years; min. 1 - max. 87) with VCP diagnosed in the otorhinolaryngology department between April 2012 and December 2015. Diagnoses associated with VCP, side of involvement (right, left or bilateral) and previous medical histories were noted and compared between patients with idiopathic and non-idiopathic VCP. Vocal cord paralysis occurred on the left side (n=56, 60.9%), right side (n=28, 30.4%) or bilaterally (n=8, 8.7%). A clinical entity related with VCP was identified in 63 patients (68.5%), while 29 (31.5%) patients had idiopathic VCP. Most common etiologies for VCP were thyroid surgery (n=32, 34.8%), cardiovascular surgery (n=9, 9.8%), lung cancer (n=6, 6.5%) and cardiac anomalies (n=4, 4.3%), respectively. Patients with idiopathic VCP were significantly older (p<0.001), while gender distribution (p=0.121) and side of involvement (p=0.340) did not differ between two groups. Vocal cord paralysis is a relatively common clinical entity with substantial rate of morbidity. Identification of the underlying etiology and awareness on the clinical characteristics are keystones for foreseeing complications and determining the appropriate therapeutic modality.

Total pubertal growth in patients with juvenile idiopathic arthritis treated with growth hormone: analysis of a single center.

PubMed

Bechtold, S; Beyerlein, A; Ripperger, P; Roeb, J; Dalla Pozza, R; Häfner, R; Haas, J P; Schmidt, H

2012-10-01

Growth failure is a permanent sequelae in juvenile idiopathic arthritis (JIA). The aim of the study was to compare pubertal growth in control and growth hormone (GH) treated JIA subjects. 64 children with JIA at a mean age of 10.38 ± 2.80 years were enrolled and followed until final height (measured in standard deviation (SD) scores). 39 children (20 m) received GH therapy and 24 (9 m) served as controls. GH dose was 0.33 mg/kg/week. Linear regression analysis was performed to identify factors influencing total pubertal growth. Mean total pubertal growth was 21.1 ± 1.3 cm (mean ± SD) in GH treated JIA patients and 13.8 ± 1.5 cm in controls. Final height was significantly higher with GH treatment (-1.67 ± 1.20 SD) compared to controls (-3.20 ± 1.84 SD). Linear regression model identified age at onset of puberty (ß=-4.2,CI: -5.9, -2.6 in controls and ß=-2.3,CI: -3.6, -1.1 in GH treated) as the main factor for total pubertal growth. Final height SDS was determined by the difference to target height at onset of puberty (ß=-0.59;CI: -0.80, -0.37 in controls and ß=-0.30,CI: -0.52, -0.08 in GH treated), age at onset of puberty (ß=0.47;CI:0.02,0.93 in controls and 0.23;CI: -0.00,0.46 in GH treated) and height gain during puberty (ß=0.13;CI:0.05,0.21 in controls and ß=0.11;CI:0.07,0.16 in GH treated). Total pubertal growth in JIA patients treated with GH was increased by a factor of 1.5 greater in comparison to controls leading to a significantly better final height. To maximize final height GH treatment should be initiated early to reduce the height deficit at onset of puberty. Copyright © 2012 Elsevier Ltd. All rights reserved.

Congruence between Polysomnography Obstructive Sleep Apnea and the Pediatric Sleep Questionnaire: Fatigue and Health-Related Quality of Life in Juvenile Idiopathic Arthritis

PubMed Central

Ward, Teresa M.; Chen, Maida Lynn; Landis, Carol A.; Ringold, Sarah; Beebe, Dean W.; Pike, Kenneth C.; Wallace, Carol A.

2016-01-01

Purpose To examine the congruence between polysomnography obstructive apnea hypopnea index (OAHI) and parent reported obstructive sleep apnea (OSA) symptoms in 6-to-11 year-old children with juvenile idiopathic arthritis (JIA) and controls; and to compare fatigue and quality of life in JIA and control children based on OAHI and OSA symptoms. Methods Sixty-eight children with JIA and 75 controls and a parent participated. Children underwent one night of polysomnography in a sleep laboratory. Parents completed the sleep-related breathing disorders scale - Pediatric Sleep Questionnaire (PSQ), and both children and parents completed the Pediatric Quality of Life Generic Core Scale and the Multidimensional Fatigue scale. Results In JIA, 86% who met the OAHI clinical criteria for OSA (≥ 1.5) were above the PSQ OSA symptom cut-off score with a sensitivity of 0.86 and a specificity of 0.28. In the control group, 63% who met the OAHI clinical criteria for OSA, were above the PSQ OSA symptom cut-off score, with a sensitivity of 0.63 and a specificity of 0.42. All children above both the clinical criteria for OAHI and OSA symptom cut-off score had the most impaired quality of life and greater fatigue compared to those below both the clinical criteria for OAHI and the OSA symptom cut-off score. Conclusion Children who meet clinical criteria for OSA and also scored high on a parent reported screening tool for OSA symptoms had the most impaired quality of life and more fatigue. The PSQ has potential to identify children at risk for OSA. PMID:27987106

Genome-scale case-control analysis of CD4+ T-cell DNA methylation in juvenile idiopathic arthritis reveals potential targets involved in disease.

PubMed

Ellis, Justine A; Munro, Jane E; Chavez, Raul A; Gordon, Lavinia; Joo, Jihoon E; Akikusa, Jonathan D; Allen, Roger C; Ponsonby, Anne-Louise; Craig, Jeffrey M; Saffery, Richard

2012-11-13

Juvenile Idiopathic Arthritis (JIA) is a complex autoimmune rheumatic disease of largely unknown cause. Evidence is growing that epigenetic variation, particularly DNA methylation, is associated with autoimmune disease. However, nothing is currently known about the potential role of aberrant DNA methylation in JIA. As a first step to addressing this knowledge gap, we have profiled DNA methylation in purified CD4+ T cells from JIA subjects and controls. Genomic DNA was isolated from peripheral blood CD4+ T cells from 14 oligoarticular and polyarticular JIA cases with active disease, and healthy age- and sex-matched controls. Genome-scale methylation analysis was carried out using the Illumina Infinium HumanMethylation27 BeadChip. Methylation data at >25,000 CpGs was compared in a case-control study design. Methylation levels were significantly different (FDR adjusted p<0.1) at 145 loci. Removal of four samples exposed to methotrexate had a striking impact on the outcome of the analysis, reducing the number of differentially methylated loci to 11. The methotrexate-naive analysis identified reduced methylation at the gene encoding the pro-inflammatory cytokine IL32, which was subsequently replicated using a second analysis platform and a second set of case-control pairs. Our data suggests that differential T cell DNA methylation may be a feature of JIA, and that reduced methylation at IL32 is associated with this disease. Further work in larger prospective and longitudinal sample collections is required to confirm these findings, assess whether the identified differences are causal or consequential of disease, and further investigate the epigenetic modifying properties of therapeutic regimens.

Osteoporosis: not only in women, but in men too.

PubMed

Gardiner, Annie; El Miedany, Yasser; Toth, Mathias

Osteoporosis is a chronic disease where the nurse specialist can make a significant impact. The osteoporosis nurse specialist has an important role, not only in the prevention and management of osteoporosis but also in the promotion of skeletal health across the public health and social care arenas and in the continuing development of services across primary and secondary care. The scale of the problem in women and the potential role of hormone replacement therapy in the prevention and treatment of osteoporosis has played down the problem of osteoporosis and osteoporotic fractures in men, thus facilitating a negligent attitude. This article summarizes the outcome of a dedicated male osteoporosis clinic, which was started by the lead clinicians in metabolic bone disease and falls and subsequently was run by the osteoporosis nurse specialist. A clinic proforma which can be used as a standard in clinical practice is presented. In addition, an algorithm for the management of men with osteoporosis is discussed.

Idiopathic hypersomnia.

PubMed

Billiard, Michel; Sonka, Karel

2016-10-01

Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double

Pharmacological management of severe postmenopausal osteoporosis.

PubMed

Gaudio, Agostino; Morabito, Nancy

2005-01-01

The most devastating consequence of osteoporosis is bone fracture, particularly at the vertebral or femoral level. As defined by the WHO, patients with osteoporosis who have had one or more fragility fractures have severe osteoporosis. Those who sustain a vertebral fracture represent a particularly vulnerable group whose risk of another vertebral fracture within the following year is increased by a factor of 3-5. In addition, the presence of a vertebral fracture is associated with an increased risk of hip fracture. In light of these data, treatment of established osteoporosis is extremely important to prevent other fragility fractures. This review examines the therapies approved by the US FDA for the treatment of osteoporosis that have been shown to reduce the incidence of new fractures in patients with established osteoporosis. We evaluated the mechanisms of action, available formulations, efficacy in preventing fractures and increasing bone mineral density (BMD), duration of treatment, adverse effects and contraindications to use of alendronic acid (alendronate), risedronic acid (risedronate), calcitonin, raloxifene and teriparatide. All these drugs are able to prevent new vertebral fractures in patients with established osteoporosis. Only alendronic acid and risedronic acid have also been shown to reduce the risk of fracture at the femoral level, but they are contraindicated in patients with upper gastrointestinal diseases. Calcitonin is a good option in subjects with back pain because of its analgesic effect. Raloxifene is useful when patients have high plasma lipid levels or a family history of breast cancer. Teriparatide is indicated in subjects with very low BMD and multiple vertebral fractures. Patient characteristics should determine selection of therapy but the decision is always difficult and fraught with uncertainty.

Osteoporosis in the Women's Health Initiative: Another Treatment Gap?

PubMed

Sattari, Maryam; Cauley, Jane A; Garvan, Cynthia; Johnson, Karen C; LaMonte, Michael J; Li, Wenjun; Limacher, Marian; Manini, Todd; Sarto, Gloria E; Sullivan, Shannon D; Wactawski-Wende, Jean; Beyth, Rebecca J

2017-08-01

Osteoporotic fractures are associated with high morbidity, mortality, and cost. We performed a post hoc analysis of the Women's Health Initiative (WHI) clinical trials data to assess osteoporosis treatment and identify participant characteristics associated with utilization of osteoporosis medication(s) after new diagnoses of osteoporosis or fracture. Information from visits prior to and immediately subsequent to the first fracture event or osteoporosis diagnosis were evaluated for medication use. A full logistic regression model was used to identify factors predictive of osteoporosis medication use after a fracture or a diagnosis of osteoporosis. The median length of follow-up from enrollment to the last WHI clinic visit for the study cohort was 13.9 years. Among the 13,990 women who reported new diagnoses of osteoporosis or fracture between enrollment and their final WHI visit, and also had medication data available, 21.6% reported taking an osteoporosis medication other than estrogen. Higher daily calcium intake, diagnosis of osteoporosis alone or both osteoporosis and fracture (compared with diagnosis of fracture alone), Asian or Pacific Islander race/ethnicity (compared with White/Caucasian), higher income, and hormone therapy use (past or present) were associated with significantly higher likelihood of osteoporosis pharmacotherapy. Women with Black/African American race/ethnicity (compared with White/Caucasian), body mass index ≥30 (compared with body mass index of 18.5-24.9), current tobacco use (compared with past use or lifetime nonusers), and history of arthritis were less likely to use osteoporosis treatment. Despite well-established treatment guidelines in postmenopausal women with osteoporosis or history of fractures, pharmacotherapy use was suboptimal in this study. Initiation of osteoporosis treatment after fragility fracture may represent an opportunity to improve later outcomes in these high-risk women. Specific attention needs to be paid to

Rural women and osteoporosis: awareness and educational needs.

PubMed

Matthews, Hollie L; Laya, Mary; DeWitt, Dawn E

2006-01-01

Little is known about rural women's knowledge about osteoporosis. To explore what women from high-prevalence rural communities know about osteoporosis and to assess their learning preferences. We surveyed 437 women in rural Washington and Oregon. The response rate was 93% (N = 406). The mean age of respondents was 63 years (range 16-95) and 74% (n = 301) of women were postmenopausal. While 27% over age 40 (n = 111) reported having a fracture as an adult, less than half of this group (42%, n = 47) considered themselves at risk for osteoporosis. Of the 42% (n = 171) who rated their knowledge of osteoporosis good or excellent, only 18% (n = 30) answered calcium and vitamin D questions correctly. About half (53%; n = 214) exercised 3 or more times per week. Reported sources of osteoporosis information included television, magazines, health care providers, and personal contacts. Over half of the women in this study wanted more information about osteoporosis, most wanted it before age 50, and health care providers were a preferred source. Less than half of participants reported having Internet access. While many participants underestimated their osteoporosis risk, most women wanted to learn more about osteoporosis and health care providers remain a preferred source of information.

Assessing osteoporosis risk factors in Spanish menopausal women.

PubMed

Martínez Pérez, José Antonio; Palacios, Santiago; García, Felipe Chavida; Pérez, Maite

2011-10-01

(1) To assess the prevalence of osteoporosis risk factors in Spanish menopausal women; (2) to detect medical and lifestyle risk factor differences between perimenopausal and postmenopausal women; (3) and to identify the main factors responsible for osteoporosis. Cross-sectional descriptive study encompassing women aged 45-65 across Spain. The study population sample was collected through random sampling and a total of 10,514 women were included. Socio-demographic, medical history, and lifestyle data were assessed. The prevalence of osteoporosis risk factors was 67.6%. The most common risk factors were physical inactivity (53.6%), use of medication related to osteoporosis risk (45.9%), and low calcium intake (30.1%). There were statistically significant differences between peri- and postmenopausal women in terms of smoking status, alcohol intake, personal history, poor dairy product intake, and medication use that could increase risk. Logistic regression analysis showed that osteoporosis was significantly associated with age, family history, age at onset of menopause, Kupperman Index, prolonged immobilization, weight loss, and other diseases that increase the probability of developing osteoporosis. A high prevalence of women taking osteoporosis risk-related medication was observed in our study. There was correlation between the menopausal symptoms' degree of severity and the risk of suffering from osteoporosis.

Predictors of Flare Following Etanercept Withdrawal in Patients with Rheumatoid Factor-negative Juvenile Idiopathic Arthritis Who Reached Remission while Taking Medication.

PubMed

Aquilani, Angela; Pires Marafon, Denise; Marasco, Emiliano; Nicolai, Rebecca; Messia, Virginia; Perfetti, Francesca; Magni-Manzoni, Silvia; De Benedetti, Fabrizio

2018-05-01

To evaluate the rate of flare after etanercept (ETN) withdrawal in patients with juvenile idiopathic arthritis (JIA) who attained clinical remission while taking medication, and to identify predictors of flare. Patients were included with oligo- (oJIA) and rheumatoid factor-negative polyarticular JIA (pJIA) who received a first course of ETN for at least 18 months, maintained clinically inactive disease (CID) for at least 6 months during treatment, and were followed for 12 months after ETN withdrawal. Demographic and clinical features were collected at onset, at baseline (initiation of ETN), and at time of disease flare. After ETN withdrawal, 66 of the 110 patients enrolled (60%) flared with arthritis (of whom 7 flared with concurrent anterior uveitis; none with uveitis alone). The median time to flare was 4.3 months (interquartile range 2.5-6.4) with no evident differences between oJIA and pJIA. The number and type of joints involved at baseline and characteristics of ETN treatment/discontinuation were not associated with flare. Patients who flared were more frequently males (p = 0.034), positive for antinuclear antibody (ANA; p = 0.047), and had higher values of C-reactive protein (CRP; p = 0.012) at baseline. These variables remained significantly associated with flare in a multivariate logistic analysis, a model accounting for only 14% of the variability of the occurrence of the flare. Our results show that a significant proportion of patients with JIA who maintain CID for at least 6 months experience a relapse after ETN withdrawal. Male sex, presence of ANA, and elevated CRP at baseline were associated with higher risk of flare.

Leflunomide is associated with a higher flare rate compared to methotrexate in the treatment of chronic uveitis in juvenile idiopathic arthritis.

PubMed

Bichler, J; Benseler, S M; Krumrey-Langkammerer, M; Haas, J-P; Hügle, B

2015-01-01

Chronic anterior uveitis is a serious complication of juvenile idiopathic arthritis (JIA); disease flares are highly associated with loss of vision. Leflunomide (LEF) is used successfully for JIA joint disease but its effectiveness in uveitis has not been determined. The aim of this study was to determine whether LEF improves flare rates of uveitis in JIA patients compared to preceding methotrexate (MTX) therapy. A single-centre retrospective study of consecutive children with JIA and chronic anterior uveitis was performed. All children initially received MTX and were then switched to LEF. Demographic, clinical, and laboratory data, dose and duration of MTX and LEF therapy, concomitant medications and rate of anterior uveitis flares, as determined by an expert ophthalmologist, were obtained. Flare rates were compared using a generalized linear mixed model with a negative binomial distribution. A total of 15 children were included (80% females, all antinuclear antibody positive). The median duration of MTX therapy was 51 (range 26-167) months; LEF was given for a median of 12 (range 4-47) months. Anti-tumour necrosis factor (anti-TNF-α) co-medication was given to four children while on MTX. By contrast, LEF was combined with anti-TNF-α treatment in six children. On MTX, JIA patients showed a uveitis flare rate of 0.0247 flares/month, while LEF treatment was associated with a significantly higher flare rate of 0.0607 flares/month (p = 0.008). Children with JIA had significantly more uveitis flares on LEF compared to MTX despite receiving anti-TNF-α co-medication more frequently. Therefore, LEF may need to be considered less effective in controlling chronic anterior uveitis.

Osteoporosis in men.

PubMed

Binkley, Neil

2006-08-01

Osteoporosis is defined as "a systemic skeletal disease characterized by low bone mass and microarchitectural deterioration of bone tissue with a consequent increase in bone fragility and susceptibility to fracture". Approximately 40-50% of women sustain osteoporotic fractures in their lifetime; as such, it is appropriate that studies initially focused upon females. Despite an increased recognition of osteoporotic fractures in men, there continues to be neglect of this disease in males. This ongoing neglect is inappropriate as 25-33% of men in some populations will sustain osteoporotic fractures in their lifetime. Testosterone plays an important role in male skeletal health. However, recent data suggest that estrogen may in fact be the dominant hormone regulating skeletal status in both men and women. BMD measurement may be utilized for osteoporosis diagnosis and to assist with fracture risk prediction in men prior to their sustaining a fracture. Recognizing this need, the International Society for Clinical Densitometry (ISCD) recommended and recently reaffirmed use of a BMD T-score of -2.5 or below be utilized to diagnose osteoporosis in men. Androgen therapy of hypogonadal men may be considered with the caveat that data do not exist to document that this treatment reduces fracture risk. At this time, the data is inadequate to support use of androgen treatment in eugonadal men with osteoporosis. Parathyroid hormone treatment does increase BMD; existing studies have not been of adequate size or duration to document fracture reduction efficacy. Bisphosphonate therapy increases BMD, reduces vertebral fracture risk and is considered the standard of care for osteoporotic men at this point in time.

Osteoporosis: Peak Bone Mass in Women

MedlinePlus

... Osteoporosis: Peak Bone Mass in Women Osteoporosis: Peak Bone Mass in Women Bones are the framework for ... that affect peak bone mass. Factors Affecting Peak Bone Mass A variety of genetic and environmental factors ...

An exploration of parents' preferences for foot care in juvenile idiopathic arthritis: a possible role for the discrete choice experiment.

PubMed

Hendry, Gordon J; Turner, Debbie E; Gardner-Medwin, Janet; Lorgelly, Paula K; Woodburn, James

2014-02-06

An increased awareness of patients' and parents' care preferences regarding foot care is desirable from a clinical perspective as such information may be utilised to optimise care delivery. The aim of this study was to examine parents' preferences for, and valuations of foot care and foot-related outcomes in juvenile idiopathic arthritis (JIA). A discrete choice experiment (DCE) incorporating willingness-to-pay (WTP) questions was conducted by surveying 42 parents of children with JIA who were enrolled in a randomised-controlled trial of multidisciplinary foot care at a single UK paediatric rheumatology outpatients department. Attributes explored were: levels of pain; mobility; ability to perform activities of daily living (ADL); waiting time; referral route; and footwear. The DCE was administered at trial baseline. DCE data were analysed using a multinomial-logit-regression model to estimate preferences and relative importance of attributes of foot care. A stated-preference WTP question was presented to estimate parents' monetary valuation of health and service improvements. Every attribute in the DCE was statistically significant (p < 0.01) except that of cost (p = 0.118), suggesting that all attributes, except cost, have an impact on parents' preferences for foot care for their child. The magnitudes of the coefficients indicate that the strength of preference for each attribute was (in descending order): improved ability to perform ADL, reductions in foot pain, improved mobility, improved ability to wear desired footwear, multidisciplinary foot care route, and reduced waiting time. Parents' estimated mean annual WTP for a multidisciplinary foot care service was £1,119.05. In terms of foot care service provision for children with JIA, parents appear to prefer improvements in health outcomes over non-health outcomes and service process attributes. Cost was relatively less important than other attributes suggesting that it does not appear to impact on

Comparable Efficacy of Abatacept Used as First-line or Second-line Biological Agent for Severe Juvenile Idiopathic Arthritis-related Uveitis.

PubMed

Birolo, Carolina; Zannin, Maria Elisabetta; Arsenyeva, Svetlana; Cimaz, Rolando; Miserocchi, Elisabetta; Dubko, Margarita; Deslandre, Chantal Job; Falcini, Fernanda; Alessio, Maria; La Torre, Francesco; Denisova, Ekaterina; Martini, Giorgia; Nikishina, Irina; Zulian, Francesco

2016-11-01

Abatacept (ABA) has recently been proposed as second-line treatment in patients with juvenile idiopathic arthritis (JIA)-associated uveitis refractory to anti-tumor necrosis factor-α (anti-TNF) agents, but little is known about its efficacy as a first-line approach. The aim of the present study was to compare the safety and efficacy of ABA as a first-line biological agent (ABA-1) with that of ABA as a second-line treatment after 1 or more anti-TNF agents (ABA-2), in patients with severe JIA-related uveitis. In this multicenter study, we collected data on patients with severe JIA-related uveitis treated with ABA as a first-line or second-line biological agent. Changes in frequency of uveitis flares/year and ocular complications before and after ABA treatment, clinical remission, and side effects were recorded. Thirty-five patients with a mean age of 10.8 years were treated with ABA for a mean period of 19.6 months. In 4 patients, ABA administration was discontinued, owing to inefficacy on arthritis in 3 cases and allergic reaction in 1. Thirty-one patients, 14 in the ABA-1 group and 17 in the ABA-2 group, completed the 12-month followup period; of these, 17 (54.8%) had clinical remission. The mean frequency of uveitis flares decreased from 4.1 to 1.2 in the ABA-1 group (p = 0.002) and from 3.7 to 1.2 in the ABA-2 group (p = 0.004). Preexisting ocular complications improved or remained stable in all but 5 patients, all in the ABA-2 group. No significant difference was found between the efficacy of the 2 treatment modalities. ABA confirmed its good safety profile. ABA, used as first-line biological treatment or after 1 or more anti-TNF agents, induces a comparable improvement in severe refractory JIA-related uveitis.

An exploration of parents’ preferences for foot care in juvenile idiopathic arthritis: a possible role for the discrete choice experiment

PubMed Central

2014-01-01

Background An increased awareness of patients’ and parents’ care preferences regarding foot care is desirable from a clinical perspective as such information may be utilised to optimise care delivery. The aim of this study was to examine parents’ preferences for, and valuations of foot care and foot-related outcomes in juvenile idiopathic arthritis (JIA). Methods A discrete choice experiment (DCE) incorporating willingness-to-pay (WTP) questions was conducted by surveying 42 parents of children with JIA who were enrolled in a randomised-controlled trial of multidisciplinary foot care at a single UK paediatric rheumatology outpatients department. Attributes explored were: levels of pain; mobility; ability to perform activities of daily living (ADL); waiting time; referral route; and footwear. The DCE was administered at trial baseline. DCE data were analysed using a multinomial-logit-regression model to estimate preferences and relative importance of attributes of foot care. A stated-preference WTP question was presented to estimate parents’ monetary valuation of health and service improvements. Results Every attribute in the DCE was statistically significant (p < 0.01) except that of cost (p = 0.118), suggesting that all attributes, except cost, have an impact on parents’ preferences for foot care for their child. The magnitudes of the coefficients indicate that the strength of preference for each attribute was (in descending order): improved ability to perform ADL, reductions in foot pain, improved mobility, improved ability to wear desired footwear, multidisciplinary foot care route, and reduced waiting time. Parents’ estimated mean annual WTP for a multidisciplinary foot care service was £1,119.05. Conclusions In terms of foot care service provision for children with JIA, parents appear to prefer improvements in health outcomes over non-health outcomes and service process attributes. Cost was relatively less important than other attributes

Fine-mapping the MHC locus in juvenile idiopathic arthritis (JIA) reveals genetic heterogeneity corresponding to distinct adult inflammatory arthritic diseases.

PubMed

Hinks, A; Bowes, J; Cobb, J; Ainsworth, H C; Marion, M C; Comeau, M E; Sudman, M; Han, B; Becker, M L; Bohnsack, J F; de Bakker, P I W; Haas, J P; Hazen, M; Lovell, D J; Nigrovic, P A; Nordal, E; Punnaro, M; Rosenberg, A M; Rygg, M; Smith, S L; Wise, C A; Videm, V; Wedderburn, L R; Yarwood, A; Yeung, R S M; Prahalad, S; Langefeld, C D; Raychaudhuri, S; Thompson, S D; Thomson, W

2017-04-01

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of diseases, comprising seven categories. Genetic data could potentially be used to help redefine JIA categories and improve the current classification system. The human leucocyte antigen (HLA) region is strongly associated with JIA. Fine-mapping of the region was performed to look for similarities and differences in HLA associations between the JIA categories and define correspondences with adult inflammatory arthritides. Dense genotype data from the HLA region, from the Immunochip array for 5043 JIA cases and 14 390 controls, were used to impute single-nucleotide polymorphisms, HLA classical alleles and amino acids. Bivariate analysis was performed to investigate genetic correlation between the JIA categories. Conditional analysis was used to identify additional effects within the region. Comparison of the findings with those in adult inflammatory arthritic diseases was performed. We identified category-specific associations and have demonstrated for the first time that rheumatoid factor (RF)-negative polyarticular JIA and oligoarticular JIA are genetically similar in their HLA associations. We also observe that each JIA category potentially has an adult counterpart. The RF-positive polyarthritis association at HLA-DRB1 amino acid at position 13 mirrors the association in adult seropositive rheumatoid arthritis (RA). Interestingly, the combined oligoarthritis and RF-negative polyarthritis dataset shares the same association with adult seronegative RA. The findings suggest the value of using genetic data in helping to classify the categories of this heterogeneous disease. Mapping JIA categories to adult counterparts could enable shared knowledge of disease pathogenesis and aetiology and facilitate transition from paediatric to adult services. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

IL-6 blockade in systemic juvenile idiopathic arthritis - achievement of inactive disease and remission (data from the German AID-registry).

PubMed

Bielak, M; Husmann, E; Weyandt, N; Haas, J-P; Hügle, B; Horneff, G; Neudorf, U; Lutz, T; Lilienthal, E; Kallinich, T; Tenbrock, K; Berendes, R; Niehues, T; Wittkowski, H; Weißbarth-Riedel, E; Heubner, G; Oommen, P; Klotsche, J; Foell, Dirk; Lainka, E

2018-04-05

Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in the pathogenesis has been ascribed to proinflammatory cytokines like interleukin-6 (IL-6), and effective drugs inhibiting their signaling are being developed. This study evaluates sJIA patients treated with the IL-6 inhibitor tocilizumab (TCZ) concerning clinical response rate, disease course and adverse effects in a real-life clinical setting. In 2009 a clinical and research consortium was established, including an online registry for autoinflammatory diseases (AID) ( https://aid-register.de ). Data for this retrospective TCZ study were documented by 13 centers. From 7/2009 to 4/2014, 200 patients with sJIA were recorded in the AID-registry. Out of these, 46 (19 m, 27 f, age 1-18 years) received therapy with TCZ. Long term treatment (median 23 months) has been documented in 24/46 patients who were evaluated according to Wallace criteria (active disease 6/24, inactive disease 5/24, remission 13/24 cases). Under observation co-medication were used in 40/46 cases. Adverse events were reported in 11/46 patients. The clinical response rate (no clinical manifestation, no increased inflammation parameters) within the first 12 weeks of treatment was calculated to be 35%. Out of 200 sJIA children reported in the German AID-registry, 46 were treated with TCZ, showing a clinical response rate of 35% during the first 12 weeks, and inactive disease and/or remission under medication in 75% after one year. Adverse events were seen in 24% and severe adverse events in 4%. The AID-Registry is funded by the BMBF (01GM08104, 01GM1112D, 01GM1512D).

Agreement between calcaneal quantitative ultrasound and osteoporosis self-assessment tool for Asians in identifying individuals at risk of osteoporosis

PubMed Central

Chin, Kok-Yong; Low, Nie Yen; Kamaruddin, Alia Annessa Ain; Dewiputri, Wan Ilma; Soelaiman, Ima-Nirwana

2017-01-01

Background Calcaneal quantitative ultrasound (QUS) is a useful tool in osteoporosis screening. However, QUS device may not be available at all primary health care settings. Osteoporosis self-assessment tool for Asians (OSTA) is a simple algorithm for osteoporosis screening that does not require any sophisticated instruments. This study explored the possibility of replacing QUS with OSTA by determining their agreement in identifying individuals at risk of osteoporosis. Methods A cross-sectional study was conducted to recruit Malaysian men and women aged ≥50 years. Their bone health status was measured using a calcaneal QUS device and OSTA. The association between OSTA and QUS was determined using Spearman’s correlation and their agreement was assessed using Cohen Kappa and receiver-operating curve. Results All QUS indices correlated significantly with OSTA (p<0.05). The agreement between QUS and OSTA was minimal but statistically significant (p<0.05). The performance of OSTA in identifying subjects at risk of osteoporosis according to QUS was poor-to-fair in women (p<0.05), but not statistically significant for men (p>0.05). Changing the cut-off values improved the performance of OSTA in women but not in men. Conclusion The agreement between QUS and OSTA is minimal in categorizing individuals at risk of osteoporosis. Therefore, they cannot be used interchangeably in osteoporosis screening. PMID:29070951

Anti-type II collagen antibodies, anti-CCP, IgA RF and IgM RF are associated with joint damage, assessed eight years after onset of juvenile idiopathic arthritis (JIA)

PubMed Central

2014-01-01

Background Early appearance of antibodies specific for native human type II collagen (anti-CII) characterizes an early inflammatory and destructive phenotype in adults with rheumatoid arthritis (RA). The objective of this study was to investigate the occurrence of anti-CII, IgM RF, IgA RF and anti-CCP in serum samples obtained early after diagnosis, and to relate the occurrence of autoantibodies to outcome after eight years of disease in children with juvenile idiopathic arthritis (JIA). Methods The Nordic JIA database prospectively included JIA patients followed for eight years with data on remission and joint damage. From this database, serum samples collected from 192 patients, at a median of four months after disease onset, were analysed for IgG anti-CII, IgM RF, IgA RF and IgG anti-CCP. Joint damage was assessed based on Juvenile Arthritis Damage Index for Articular damage (JADI-A), a validated clinical instrument for joint damage. Results Elevated serum levels of anti-CII occurred in 3.1%, IgM RF in 3.6%, IgA RF in 3.1% and anti-CCP in 2.6% of the patients. Occurrence of RF and anti-CCP did to some extent overlap, but rarely with anti-CII. The polyarticular and oligoarticular extended categories were overrepresented in patients with two or more autoantibodies. Anti-CII occurred in younger children, usually without overlap with the other autoantibodies and was associated with high levels of C-reactive protein (CRP) early in the disease course. All four autoantibodies were significantly associated with joint damage, but not with active disease at the eight-year follow up. Conclusions Anti-CII, anti-CCP, IgA RF and IgM RF detected early in the disease course predicted joint damage when assessed after eight years of disease. The role of anti-CII in JIA should be further studied. PMID:24944545

Pharmacological treatment of osteoporosis in the oldest old

PubMed Central

Vandenbroucke, A; Luyten, FP; Flamaing, J; Gielen, E

2017-01-01

The incidence of osteoporotic fractures increases with age. Consequently, the global prevalence of osteoporotic fractures will increase with the aging of the population. In old age, osteoporosis is associated with a substantial burden in terms of morbidity and mortality. Nevertheless, osteoporosis in old age continues to be underdiagnosed and undertreated. This may, at least partly, be explained by the fact that evidence of the antifracture efficacy of osteoporosis treatments comes mainly from randomized controlled trials in postmenopausal women with a mean age of 70–75 years. However, in the last years, subgroup analyses of these landmark trials have been published investigating the efficacy and safety of osteoporosis treatment in the very elderly. Based on this evidence, this narrative review discusses the pharmacological management of osteoporosis in the oldest old (≥80 years). Because of the high prevalence of calcium and/or vitamin D deficiency in old age, these supplements are essential in the management of osteoporosis in the elderly people. Adding antiresorptive or anabolic treatments or combinations, thereof, reduces the risk of vertebral fractures even more, at least in the elderly with documented osteoporosis. The reduction of hip fracture risk by antiresorptive treatments is less convincing, which may be explained by insufficient statistical power in some subanalyses and/or a higher impact of nonskeletal risk factors in the occurrence of hip fractures. Compared with younger individuals, a larger absolute risk reduction is observed in the elderly because of the higher baseline fracture risk. Therefore, the elderly will benefit more of treatment. In addition, current osteoporosis therapies also appear to be safe in the elderly. Although more research is required to further clarify the effect of osteoporosis drugs in the elderly, especially with respect to hip fractures, there is currently sufficient evidence to initiate appropriate treatment in the

Learning-based landmarks detection for osteoporosis analysis

NASA Astrophysics Data System (ADS)

Cheng, Erkang; Zhu, Ling; Yang, Jie; Azhari, Azhari; Sitam, Suhardjo; Liang, Xin; Megalooikonomou, Vasileios; Ling, Haibin

2016-03-01

Osteoporosis is the common cause for a broken bone among senior citizens. Early diagnosis of osteoporosis requires routine examination which may be costly for patients. A potential low cost diagnosis is to identify a senior citizen at high risk of osteoporosis by pre-screening during routine dental examination. Therefore, osteoporosis analysis using dental radiographs severs as a key step in routine dental examination. The aim of this study is to localize landmarks in dental radiographs which are helpful to assess the evidence of osteoporosis. We collect eight landmarks which are critical in osteoporosis analysis. Our goal is to localize these landmarks automatically for a given dental radiographic image. To address the challenges such as large variations of appearances in subjects, in this paper, we formulate the task into a multi-class classification problem. A hybrid feature pool is used to represent these landmarks. For the discriminative classification problem, we use a random forest to fuse the hybrid feature representation. In the experiments, we also evaluate the performances of individual feature component and the hybrid fused feature. Our proposed method achieves average detection error of 2:9mm.

Higher prevalence of osteoporosis among female Holocaust survivors.

PubMed

Marcus, E-L; Menczel, J

2007-11-01

The prevalence of osteoporosis was statistically significantly higher among female Holocaust survivors than among those who were not exposed to the Holocaust. These findings support the importance of nutrition and environmental conditions during childhood and adolescence on BMD in older adults. Holocaust survivors during childhood and adolescence experienced undernutrition and lack of exercise and sunlight. The study aimed to establish if Holocaust survivors have higher prevalence of osteoporosis than subjects who were not Holocaust survivors. Seventy-three female Jewish Holocaust survivors > or = 60 years old and 60 female European-born Jews > or =60 years old who were not in the Holocaust were examined. BMD was measured using DXA of the lumbar spine and hips. The Cochran-Armitage trend test was used to test for an increasing trend in decreased BMD in the Holocaust survivors versus controls. Among Holocaust survivors 54.8% had osteoporosis, 39.7% osteopenia, and 5.5% normal BMD, whereas among controls 25.0% had osteoporosis, 55.0% osteopenia, and 20.0% normal BMD (p = 0.0001). In those who were <17 years old in 1945, among Holocaust survivors 58.0% had osteoporosis, 34.0% osteopenia, and 8.0% normal BMD, whereas among controls 20.0% had osteoporosis, 57.8% osteopenia, and 22.2% normal BMD (p = 0.0003). In those > or =17 years old in 1945, among Holocaust survivors 47.8% had osteoporosis, 52.2% osteopenia and none had normal BMD, whereas among controls 40.0% had osteoporosis, 46.7% osteopenia, and 13.3% normal BMD (p = 0.28). The prevalence of osteoporosis was significantly higher among Holocaust survivors.

Education and employment in patients with juvenile idiopathic arthritis - a standardized comparison to the German general population.

PubMed

Schlichtiger, Jenny; Haas, Johannes-Peter; Barth, Swaantje; Bisdorff, Betty; Hager, Lisa; Michels, Hartmut; Hügle, Boris; Radon, Katja

2017-05-22

Although several studies show that JIA-patients have significantly lower employment rates than the general population, the research on educational and occupational attainments in patients with juvenile idiopathic arthritis (JIA) remain conflicting most likely due to small sample sizes. Therefore, aim of this study is to compare the educational achievements and employment status of 3698 JIA-patients with the German general population (GGP). "SEPIA" was a large cross-sectional study on the current status of a historic cohort of JIA-patients treated in a single center between 1952 and 2010. For the analyses of education and employment a sub-cohort was extracted, including only adult cases with a confirmed diagnosis of JIA (N = 2696). Participants were asked to fill out a standardized written questionnaire on education and employment. Outcome measures (education/unemployment) were directly standardized to the GGP using data obtained from the National Educational Panel Study 2013 (N = 11,728) and the German Unemployment Statistics 2012 of the Federal Statistical Office (N = 42,791,000). After age- and sex-standardization, 3% (95% Confidence Interval 1.9 to 4.1%) more of the JIA-patients (26%) than of the GGP (23%) had only reached primary education. In contrast, parents of JIA-patients had similar levels of education as parents in the GGP. With a standardized difference of 0.2% (95% CI: 0.16 to 0.19%), the unemployment rate in JIA-patients was slightly, but not significantly higher than in the GGP. Stratifying for disease duration and the current treatment status, differences were confirmed for persons diagnosed before 2001, whilst for patients diagnosed after 2000, differences were found only in JIA-patients with ongoing disease. Medium and high educational achievements did not differ statistically significant between JIA patients and the GPP. Educational achievements in German JIA-patients are significantly lower than in the GGP. Furthermore we were able to

Quantifying the Effect of Temporomandibular Joint Intra-Articular Steroid Injection on Synovial Enhancement in Juvenile Idiopathic Arthritis.

PubMed

Resnick, Cory M; Vakilian, Pouya M; Kaban, Leonard B; Peacock, Zachary S

2016-12-01

To quantify the effect of intra-articular steroid injections (IASIs) on temporomandibular joint (TMJ) synovitis in children with juvenile idiopathic arthritis (JIA) using gadolinium-enhanced magnetic resonance imaging (MRI). The present study was a retrospective study of children with JIA who had undergone TMJ IASIs at Boston Children's Hospital. The patients were included if they had undergone contrast-enhanced MRI both before and after IASI and if the pre-IASI MRI had demonstrated synovitis (enhancement ratio [ER] >1.55). Patients with TMJ pathology or pain unrelated to JIA or a history of facial trauma were excluded. The predictor variables were age, gender, JIA subtype, exposure to medications for arthritis, and a family history of autoimmune disease. The primary outcome variable was the ER. Additional outcome variables included patient-reported pain and the maximal incisal opening (MIO). Twenty-nine subjects (83% female) with a total of 50 injected TMJs were included. The average age at JIA diagnosis and at IASI was 6.8 ± 1.7 years and 12.1 ± 1.9 years, respectively. The mean follow-up period was 22.9 ± 4.3 months (range 5 to 48). The ER decreased in all injected joints, with a mean reduction of 1.05 ± 1.01 (P < .001). The post-IASI ER was less than the normal threshold (1.55) in 18% of the injected TMJs. IASI was associated with an elimination of pain in 89% of the subjects (P < .001) and in augmentation of the MIO by 5.8 ± 2.6 mm (P < .001). In children with JIA and TMJ synovitis, TMJ IASI was associated with a reduction in synovial enhancement, decreased pain, and an increased MIO. Only 18% of injected joints, however, experienced complete resolution of synovitis. These results support the use of IASI in the management of the pain and dysfunction associated with TMJ synovitis. Further study is required to determine the efficacy of IASI in limiting inflammation and future joint destruction. Copyright © 2016 American Association of Oral and

Osteoporosis in Children with Chronic Disease.

PubMed

Högler, Wolfgang; Ward, Leanne

2015-01-01

Serious illness in children and its therapy can cause osteoporosis, manifesting as vertebral and nonvertebral fractures, pain, skeletal deformity and temporary or even permanent loss of ambulation. In contrast to adults, skeletal growth in children offers tremendous potential to recover bone mineral density and to reshape fractured vertebral bodies, even without bone-targeted therapy, provided that bone health threats are transient and residual growth is sufficient. Here, we outline the principles of bone strength development and the risk factors for osteoporosis due to various paediatric systemic illnesses. We also explain why the approach to the diagnosis and monitoring of childhood osteoporosis has moved away from a bone density-centric focus to a more functional assessment. Finally, we discuss the best candidates for and current approaches to the treatment of osteoporosis in children. © 2015 S. Karger AG, Basel.

Pathophysiology of immobilization osteoporosis

NASA Technical Reports Server (NTRS)

Doty, S. B.; DiCarlo, E. F.

1995-01-01

The reduction of gravity-related forces on the skeleton creates a type of osteoporosis that is unique because its severity is dependent on the mechanical stress bearing function of the skeleton as well as the length of time that the forces are absent or reduced. Bones that bear weight under normal conditions are more affected than bones that normally do not bear weight. The cytokine environment and the cells in the affected bones are altered in time so that stem cells produce fewer new cells and the differentiated cells tend to be less active. These alterations in the local environment of the affected parts appear to resemble those of age- and disease-associated systemic forms of osteoporosis. The osteoporosis produced as a result of the loss of normal activity however, appears to be at least partially reversible through remobilization, strenuous exercise, and--possibly in the future--cytokine therapy.

Awareness of osteoporosis in postmenopausal Indian women: An evaluation of Osteoporosis Health Belief Scale.

PubMed

Gopinathan, Nirmal Raj; Sen, Ramesh Kumar; Behera, Prateek; Aggarwal, Sameer; Khandelwal, Niranjan; Sen, Mitali

2016-01-01

The level of awareness about osteoporosis in postmenopausal women who are the common sufferers. This study aims to evaluate the level of awareness in postmenopausal women using the Osteoporosis Health Belief Scale (OHBS). Osteoporosis has emerged as a common health problem in geriatric population. A proactive role needs to be played for preventing its consequences. Before initiating any preventive measures, an evaluation of awareness level of the target population is necessary. The questionnaire-based study design was used for this study. A questionnaire (OHBS)-based study in 100 postmenopausal women in Chandigarh was conducted. The bone mineral density (BMD) was measured in each case by dual energy X-ray absorptiometry. Height, weight, and body mass index (BMI) of the participants were noted. Statistical analysis was conducted to evaluate any correlation between the various components of the OHBS and the BMD. No statistically significant difference was noted in the seven component parameters of OHBS among the normal, osteopenic, and osteoporotic women suggesting that the health belief regarding susceptibility is not much different between the three groups of the study population. A statistically significant difference between the mean BMI of normal and osteoporotic population was noted. The results show that there is a great deficit in the awareness level of postmenopausal Indian women regarding osteoporosis. Most of the women were unaware of the condition and the means to prevent it. The study emphasizes that health care professionals have lot of ground to cover to decrease the incidence of osteoporosis and its associated health problem.

Focal seizure symptoms in idiopathic generalized epilepsies.

PubMed

Seneviratne, Udaya; Woo, Jia J; Boston, Ray C; Cook, Mark; D'Souza, Wendyl

2015-08-18

We sought to study the frequency and prognostic value of focal seizure symptoms (FSS) in idiopathic generalized epilepsies (IGE) using a validated tool: Epilepsy Diagnostic Interview Questionnaire and Partial Seizure Symptom Definitions. Participants with IGE were recruited from epilepsy clinics at 2 tertiary hospitals. The diagnosis was validated and classified into syndromes according to the International League Against Epilepsy criteria by 2 epileptologists independently with discordance resolved by consensus. The Epilepsy Diagnostic Interview Questionnaire utilizes both open- and closed-ended questions to elicit FSS in association with generalized tonic-clonic seizures, myoclonus, and absences. The elicited FSS were classified according to the Partial Seizure Symptom Definitions. Regression analysis was conducted to examine the relationship between the duration of seizure freedom and FSS. A total of 135 patients were studied, of whom 70 (51.9%) reported FSS. Those symptoms occurred in association with generalized tonic-clonic seizures (53.1%) as well as myoclonus and absences (58%). FSS were reported with similar frequency in juvenile absence epilepsy (62.5%) and juvenile myoclonic epilepsy (60%), and with a lesser frequency in generalized epilepsy with tonic-clonic seizures only (39.5%) and childhood absence epilepsy (33.3%). A strong relationship between FSS and duration of seizure freedom was found (regression coefficient -0.665, p = 0.037). FSS are frequently reported by patients with IGE. A shorter duration of seizure freedom is associated with FSS. Recognition of the presence of FSS in IGE is important to avoid misdiagnosis and delayed diagnosis as well as to choose appropriate antiepileptic drug therapy. © 2015 American Academy of Neurology.

Understanding Preferences for Osteoporosis Information to Develop an Osteoporosis Patient Education Brochure.

PubMed

Edmonds, Stephanie W; Solimeo, Samantha L; Nguyen, Vu-Thuy; Wright, Nicole C; Roblin, Douglas W; Saag, Kenneth G; Cram, Peter

2017-01-01

Patient education materials can provide important information related to osteoporosis prevention and treatment. However, available osteoporosis education materials fail to follow best-practice guidelines for patient education. To develop an educational brochure on bone health for adults aged 50 years and older using mixed-method, semistructured interviews. This project consisted of 3 phases. In Phase 1, we developed written content that included information about osteoporosis. Additionally, we designed 2 graphic-rich brochures, Brochure A (photographs) and Brochure B (illustrations). In Phase 2, interviewers presented the text-only document and both brochure designs to 53 participants from an academic Medical Center in the Midwest and an outpatient clinic in the Southeastern region of the US. Interviewers used open- and closed-ended questions to elicit opinions regarding the brochures. In Phase 3, using feedback from Phase 2, we revised the brochure and presented it to 11 participants at a third site in the Southeastern US. Participants' comprehension of brochure text and acceptability of brochure design. We enrolled 64 participants. Most were women, white, and college-educated, with an average age of 66.1 years. Participants were able to restate the basic content of the brochure and preferred Brochure A's use of photographs. Using feedback from older adults, we developed and refined a brochure for communicating bone health information to older adults at risk of osteoporosis and fragility fractures. The methods outlined in this article may serve to guide others in developing health educational brochures for chronic medical conditions.

Motor co-activation in siblings of patients with juvenile myoclonic epilepsy: an imaging endophenotype?

PubMed Central

Wandschneider, Britta; Centeno, Maria; Vollmar, Christian; Symms, Mark; Thompson, Pamela J.; Duncan, John S.

2014-01-01

Juvenile myoclonic epilepsy is a heritable idiopathic generalized epilepsy syndrome, characterized by myoclonic jerks and frequently triggered by cognitive effort. Impairment of frontal lobe cognitive functions has been reported in patients with juvenile myoclonic epilepsy and their unaffected siblings. In a recent functional magnetic resonance imaging study we reported abnormal co-activation of the motor cortex and increased functional connectivity between the motor system and prefrontal cognitive networks during a working memory paradigm, providing an underlying mechanism for cognitively triggered jerks. In this study, we used the same task in 15 unaffected siblings (10 female; age range 18–65 years, median 40) of 11 of those patients with juvenile myoclonic epilepsy (six female; age range 22–54 years, median 35) and compared functional magnetic resonance imaging activations with 20 age- and gender-matched healthy control subjects (12 female; age range 23–46 years, median 30.5). Unaffected siblings showed abnormal primary motor cortex and supplementary motor area co-activation with increasing cognitive load, as well as increased task-related functional connectivity between motor and prefrontal cognitive networks, with a similar pattern to patients (P < 0.001 uncorrected; 20-voxel threshold extent). This finding in unaffected siblings suggests that altered motor system activation and functional connectivity is not medication- or seizure-related, but represents a potential underlying mechanism for impairment of frontal lobe functions in both patients and siblings, and so constitutes an endophenotype of juvenile myoclonic epilepsy. PMID:25001494

Genetic background of osteoporosis.

PubMed

Obermayer-Pietsch, B; Chararas, C; Kotschan, S; Walter, D; Leb, G

2000-01-01

Osteoporosis is a systemic disorder of decreased skeletal mass as measured by bone mineral density (BMD), and disturbed skeletal architecture and function which results in an increased risk for bone fractures with consecutively increased morbidity and mortality. Twin and family studies have shown an important genetic component of BMD of about 40-60%. This exceeds other well known factors influencing BMD such as environmental factors like dietary calcium, physical activity or several drugs and diseases. Therefore, interest increased in the genetic background of bone mineral density. Polymorphisms of the Vitamin D receptor gene were the first to be published in this area. Studies on other loci or candidate genes such as the estrogen receptor gene or the collagen type I alpha1 gene also showed associations with bone mineral density that could explain at least a part of the genetic background of osteoporosis. Recently published data suggest that these genetic markers of bone metabolism are important in interaction with each other or in certain bone-affecting diseases. In the future, genetic studies on osteoporosis will have to screen further relevant genes and markers for bone metabolism as well as to evaluate the complex interactions of genetic influences, so that it would be possible to calculate a patient's individual risk for osteoporosis in the context of environmental influences.

Juvenile idiopathic arthritis patients and their skeletal status: possible role of vitamin D receptor gene polymorphism.

PubMed

Kostik, M M; Smirnov, A M; Demin, G S; Scheplyagina, L A; Larionova, V I

2014-01-01

We evaluated bone mineralization and metabolism changes related to vitamin D receptor (VDR) polymorphic genotypes in children with juvenile idiopathic arthritis. One hundred and ninety eight children (82 boys and 116 girls) were included in our study. Bone mineral density (BMD) was measured by lumbar spine DXA. Osteocalcin, CTX, parathyroid hormone, total and ionized calcium, inorganic phosphate, total alkaline phosphatase activity was utilized for assessment of bone metabolism. Molecular testing: TaqI (rs731236) and Cdx2 (rs11568820) polymorphisms of VDR were detected by RFLP. No differences in TaqI and Cdx2 haplotypes, genotypes and alleles distribution related with normal and low BMD (Zscore <-2SD) were found. Children with low linear growth (<10th percentile) had more allele T-contained genotypes of TagI VDR (p = 0.037), compare with medium or high linear growth children. Children with high linear growth (>90th percentile) had the highest frequency of allele A-contained genotypes (GA+AA) of Cdx2 VDR (p = 0.009). Girls with TT TaqI VDR, who never been treated by glucocorticoides had lower BMD-Zscore than C allele carriers (TT = -0.94SD [IQR: -2.1;-0.5], TC+CC = -0.62SD [IQR: -1.26;0.39], p = 0.03). Girls with Tanner I with TT had higher total and ionized Ca level than carriers of C allele (Ca: TT = 2.43 ± 0.15 mmol/l, TC+CC = 2.28 ± 0.2 mmol/l, p = 0.024; Ca(2+): TT = 1.15 ± 0.08 mmol/l, TC+CC = 1.06 ± 0.13 mmol/l, p = 0.026). Presence of TT genotype negatively correlated with BMD-Zscore (r = -0.28, p = 0.04), and positively with frequency of LBMD (r = 0.3, p = 0.037). Boy with GG Cdx2 genotype had lower total Ca (GG = 2.3 ± 0.17 mmol/l, GA+AA = 2.43 ± 0.17 mmol/l, p = 0.004) compare with carriers of A allele. Pubertal boys (Tanner IV-V) with GG had higher CTX (GG = 1.75 ± 0.11 ng/ml, GA+AA = 1.06 ± 0.07 ng/ml, p = 0.04. TT genotype of TaqI and GG genotype of Cdx2 VDR is a negative factor impact bone mineralization metabolism and linear growth.

Elevated S100A8/A9 and S100A12 Serum Levels Reflect Intraocular Inflammation in Juvenile Idiopathic Arthritis-Associated Uveitis: Results From a Pilot Study.

PubMed

Walscheid, Karoline; Heiligenhaus, Arnd; Holzinger, Dirk; Roth, Johannes; Heinz, Carsten; Tappeiner, Christoph; Kasper, Maren; Foell, Dirk

2015-12-01

Juvenile idiopathic arthritis-associated uveitis (JIAU) is the most common uveitis entity in childhood. As S100A8/A9 and S100A12 proteins are valuable biomarkers in childhood arthritis, we investigated the occurrence of these proteins in childhood uveitis. Serum samples from patients with JIAU (n = 79) or idiopathic anterior uveitis (IAU, n = 24), as well as from nonuveitic controls (n = 24), were collected. Furthermore, aqueous humor samples (JIAU n = 17, nonuveitic controls n = 16, IAU n = 12) were obtained. Samples were analyzed for S100A8/A9 and S100A12 protein levels by ELISA. Intergroup comparisons were performed, involving patient data, clinical data, and S100 levels. S100A8/A9 and S100A12 serum levels were elevated in IAU and JIAU patients as compared to nonuveitic controls (all P < 0.05). S100 serum levels in JIAU patients were higher in active arthritis (not significant; P = 0.289 for S100A8/A9 and P = 0.196 for S100A12) and active uveitis (P = 0.010 for S100A8/A9 and P = 0.026 for S100A12) than in controls. No significant differences in S100 levels were found in a subgroup analysis for sex, antinuclear antibody (ANA) status, disease duration, or presence of uveitis complications. In JIAU patients, S100 serum levels correlated with age and age at onset of uveitis. A longitudinal analysis in JIAU patients showed a correlation of serum S100A8/A9 and S100A12 levels with uveitis activity (both P = 0.03). S100A8/A9 levels in aqueous humor of patients with JIAU (P = 0.001) and IAU (P = 0.0002) were increased as compared to nonuveitic controls. Increased S100A8/A9 and S100A12 levels are found in the serum and aqueous humor of patients with autoimmune uveitis. Serum levels reflect activity of joint and eye disease.

Glucocorticoid-Induced Osteoporosis and Osteonecrosis

PubMed Central

Weinstein, Robert S.

2012-01-01

SYNOPSIS Glucocorticoid administration is the most common cause of secondary osteoporosis and the leading cause of nontraumatic osteonecrosis. In patients receiving long-term therapy, glucocorticoids induce fractures in 30 to 50% and osteonecrosis in 9 to 40%. This article reviews glucocorticoid-induced osteoporosis and osteonecrosis addressing the risk factors, pathogenesis, evaluation, treatment, and uncertainties in the clinical management of these disorders. PMID:22877431

[High prevalence of osteoporosis in asymptomatic postmenopausal Mapuche women].

PubMed

Ponce, Lucía; Larenas, Gladys; Riedemann, Pablo

2002-12-01

Genetic and environmental factors are responsible for variations in the frequency of osteoporosis. Prevalence of osteoporosis in Mapuche women (native Chileans) is unknown. To assess the prevalence and risk factors for osteoporosis in Mapuche women. A random sample of 95 asymptomatic postmenopausal Mapuche females, stratified by age, was studied. Women with diseases or medications that could interfere with calcium metabolism were excluded. Spine and femoral neck bone mass density was determined using a Lunar DPX Alpha densitometer. Seventeen percent of women had normal bone mineral density in both spine and femoral neck. In the spine, 25.3% had a normal bone mineral density, 17.9% had osteopenia and 56.8% had osteoporosis. In the femoral neck, 34.7% had a normal bone mineral density, 57.9% had osteopenia, and 7.4% had osteoporosis. There was a positive correlation between bone mineral density and body mass index. Women with more than one hour per day of physical activity, had a significantly lower proportion of osteopenia or osteoporosis. No association between bone mineral density and parity or calcium intake, was observed. There is a high prevalence of osteopenia and osteoporosis among Mapuche women. Osteoporosis was associated with low body mass index.

New Horizons in Osteoporosis

PubMed Central

Rachner, Tilman D.; Khosla, Sundeep; Hofbauer, Lorenz C.

2013-01-01

Summary Osteoporosis is a common disease characterised by a systemic impairment of bone mass and microarchitecture that results in fragility fractures. With an ageing population, the medical and socioeconomic impact of osteoporosis in general and postmenopausal osteoporosis in particular, will increase further. A detailed knowledge of bone biology with molecular insights into the communication between bone-forming osteoblasts and bone-resorbing osteoclasts and the orchestrating signalling network has led to the identification of novel therapeutic targets. Based on this, therapeutic strategies have been developed aimed at (I) inhibiting excessive bone resorption and by (II) increasing bone formation. The most promising novel treatments include denosumab, a monoclonal antibody against receptor activator of NF-κB ligand, a key osteoclast cytokine, odanacatib, a specific inhibitor of the osteoclast protease cathepsin K, and antibodies against the proteins sclerostin and dickkopf-1, two endogenous inhibitors of bone formation. This review provides an overview on these novel therapies and explains their underlying physiology. PMID:21450337

Race, ethnicity, and disease outcomes in juvenile idiopathic arthritis: a cross-sectional analysis of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.

PubMed

Ringold, Sarah; Beukelman, Timothy; Nigrovic, Peter A; Kimura, Yukiko

2013-06-01

To measure the associations between self-reported race and ethnicity and disease outcomes, including joint damage, pain, and functional ability, in children with juvenile idiopathic arthritis (JIA). A cross-sectional analysis of children with JIA enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry between May 2010 and March 2012. Mann-Whitney U test and chi-square testing were used to compare patient characteristics between race (white, African American, or Asian) and ethnicity (Non-Hispanic and Non-Latino; Hispanic or Latino) categories. Logistic regression was used to measure the associations between each race or ethnicity category and the outcome of interest. Race category was available for 4292 of 4682 children (93% white, 5% African American, Asian 3%). Ethnicity data were available for 4644 (11% Hispanic or Latino). African American children with polyarticular-course JIA had an elevated OR for joint damage on radiographic imaging compared to white children (OR 1.9, 95% CI 1.0-3.1; p = 0.04). Hispanic/Latino children had increased odds of having disability scores > 75th percentile (OR 1.5, 95% CI 1.1-2.1; p < 0.01) compared to non-Hispanic/Latino children; however, these odds were no longer significant when the cohort was limited to children with polyarticular-course JIA. Asian children had decreased odds of higher pain and functional disability compared to white children (p < 0.05). Race and ethnicity were variably associated with joint damage, pain, and functional ability. Understanding outcome variation between different race and ethnicity groups may help to optimize care for children with JIA.

Repeated exposure to antibiotics in infancy: a predisposing factor for juvenile idiopathic arthritis or a sign of this group's greater susceptibility to infections?

PubMed

Arvonen, Miika; Virta, Lauri J; Pokka, Tytti; Kröger, Liisa; Vähäsalo, Paula

2015-03-01

Previous exposure to antibiotics has been associated with the pathogenesis of several autoimmune diseases. Our objective was to explore whether childhood exposure to antibiotics would be associated with the risk of developing juvenile idiopathic arthritis (JIA). The material was collected from national registers containing all children born in 2000-2010 in Finland and diagnosed with JIA by the end of December 2012 (n = 1298) and appropriate controls (n = 5179) matched for age, sex, and place of birth. All purchases of antibiotics were collected from birth until the index date (i.e., the date of special reimbursement for JIA medications). A conditional logistic regression was performed to evaluate the association between the exposure to antibiotics and the risk of JIA. The risk of JIA increased with the number of antibiotic purchases from birth to the index date: for ≥ 1 purchases versus none, OR 1.6, 95% CI 1.3-1.9 with an upward trend in OR (p < 0.001). Antibiotic groups lincosamides and cephalosporins showed the strongest association with JIA (OR 6.6, 95% CI 3.7-11.7, and OR 1.6, 95% CI 1.4-1.8, respectively). Overall exposure to antibiotics before 2 years of age was associated with an increased risk of JIA (OR 1.4, 95% CI 1.2-1.6), with the trend test of OR (p < 0.001). Previous early and repeated exposure to antibiotics may predispose individuals to develop JIA. Alternatively, the apparent association may reflect shared susceptibility to infections and JIA.

Evidence for Updating the Core Domain Set of Outcome Measures for Juvenile Idiopathic Arthritis: Report from a Special Interest Group at OMERACT 2016.

PubMed

Morgan, Esi M; Riebschleger, Meredith P; Horonjeff, Jennifer; Consolaro, Alessandro; Munro, Jane E; Thornhill, Susan; Beukelman, Timothy; Brunner, Hermine I; Creek, Emily L; Harris, Julia G; Horton, Daniel B; Lovell, Daniel J; Mannion, Melissa L; Olson, Judyann C; Rahimi, Homaira; Gallo, Maria Chiara; Calandra, Serena; Ravelli, Angelo; Ringold, Sarah; Shenoi, Susan; Stinson, Jennifer; Toupin-April, Karine; Strand, Vibeke; Bingham, Clifton O

2017-12-01

The current Juvenile Idiopathic Arthritis (JIA) Core Set was developed in 1997 to identify the outcome measures to be used in JIA clinical trials using statistical and consensus-based techniques, but without patient involvement. The importance of patient/parent input into the research process has increasingly been recognized over the years. An Outcome Measures in Rheumatology (OMERACT) JIA Core Set Working Group was formed to determine whether the outcome domains of the current core set are relevant to those involved or whether the core set domains should be revised. Twenty-four people from the United States, Canada, Australia, and Europe, including patient partners, formed the working group. Guided by the OMERACT Filter 2.0 process, we performed (1) a systematic literature review of outcome domains, (2) a Web-based survey (142 patients, 343 parents), (3) an idea-generation study (120 parents), (4) 4 online discussion boards (24 patients, 20 parents), and (5) a Special Interest Group (SIG) activity at the OMERACT 13 (2016) meeting. A MEDLINE search of outcome domains used in studies of JIA yielded 5956 citations, of which 729 citations underwent full-text review, and identified additional domains to those included in the current JIA Core Set. Qualitative studies on the effect of JIA identified multiple additional domains, including pain and participation. Twenty-one participants in the SIG achieved consensus on the need to revise the entire JIA Core Set. The results of qualitative studies and literature review support the need to expand the JIA Core Set, considering, among other things, additional patient/parent-centered outcomes, clinical data, and imaging data.

Association between sarcopenia and osteoporosis in chronic liver disease.

PubMed

Hayashi, Manabu; Abe, Kazumichi; Fujita, Masashi; Okai, Ken; Takahashi, Atsushi; Ohira, Hiromasa

2018-05-07

Sarcopenia and osteoporosis are important complications in chronic liver disease (CLD). The aim of this study was to investigate the relationship between sarcopenia and osteoporosis in patients with CLD. We retrospectively investigated the relationship between sarcopenia and osteoporosis in 112 CLD patients (57 males and 55 females), including 40 cirrhotic patients (36%), by measuring the appendicular skeletal muscle mass index (ASMI) using bio-impedance analysis. Bone mineral density (BMD) was measured by dual-energy X-ray absorptiometry. The sarcopenia rate was 13% (14/112), and the osteoporosis and osteopenia rates were 17% (19/112) and 65% (73/112), respectively. The rate of osteoporosis was significant and high in patients with sarcopenia or cirrhosis. In linear regression analysis, sarcopenia was significantly associated with the BMD of the lumbar spine (Coefficient = -0.149, P = 0.014) and the femur neck (Coefficient = -0.110, P = 0.003). Cirrhosis was also significantly associated with low BMD of the lumbar spine (Coefficient = -0.160, P < 0.001) and the femur neck (Coefficient = -0.066, P = 0.015). In the logistic analysis, sarcopenia (odds ratio = 6.16, P = 0.039) and cirrhosis (odds ratio = 15.8, P = 0.002) were independent risk factors for osteoporosis. The ASMI cut-off values for osteoporosis were 7.33 kg/m 2 in males and 5.71 kg/m 2 in females. Sarcopenia was closely associated with osteoporosis, and a low ASMI was a potential predictor of osteoporosis in CLD patients. Screening for BMD may be required to detect osteoporosis in cirrhotic patients. This article is protected by copyright. All rights reserved.

A model of health education and management for osteoporosis prevention.

PubMed

Wang, Liang; Xu, Xiaowen; Zhang, Yan; Hao, Hongxia; Chen, Liying; Su, Tianjiao; Zhang, Yan; Ma, Weifeng; Xie, Yuanyuan; Wang, Tiantian; Yang, Fan; He, Li; Wang, Wenjiao; Fu, Xuemei; Ma, Yuanzheng

2016-12-01

Osteoporosis, a chronic disease with no therapeutic cure, affects a growing number of people as the aging population in China rapidly increases. Therefore, developing an evidence-based model of health education and management for osteoporosis prevention is required. In the present study, an osteoporosis club was established, which is a novel model of health education and management for osteoporosis prevention. A unified management of membership was used based on a digitized database. A total of 436 patients with osteoporosis were randomly assigned to the osteoporosis club group or the self-management control group. For the osteoporosis club group, multiple activities of health education were performed, including monthly systematic health education lectures, exercise programs and communication parties once a year. For the control group, the participants took charge of their own musculoskeletal health. All data of the participants were collected and evaluated prior to and following intervention. In the pre-intervention assessment, no significant difference was identified in the health education between the two groups. Through the four-year intervention, the osteoporosis knowledge, health beliefs, living behavior, medication compliance, quality of life and bone mineral density of the osteoporosis club group were improved significantly compared with the control group (P<0.001), while the pain degree of the osteoporosis club group was relieved significantly more compared with the control group (P<0.001). The results in the present study suggest that setting up an osteoporosis club is an evidence-based model of health education and management for osteoporosis prevention in China.

Awareness and knowledge of osteoporosis in Vietnamese women.

PubMed

Nguyen, Nguyen V; Dinh, Tri A; Ngo, Quang V; Tran, Vinh D; Breitkopf, Carmen Radecki

2015-03-01

Vietnamese women are at particular risk of osteoporosis and its complications. This study examined osteoporosis knowledge and awareness among Vietnamese women who have accessed health care. A sample of 217 women, 13 to 76 years of age, who were attending 1 of 2 health care facilities in Da Nang, Vietnam, between November and December 2009 completed a questionnaire assessing their awareness of osteoporosis and measuring their knowledge using a 30-item instrument reflecting 9 knowledge domains (eg, risk factors, diagnosis, prognosis). A majority (81.6%) of the women had heard of osteoporosis. Awareness was associated with education, working in health care, and having a family member with osteoporosis. On average, Vietnamese women answered 49% of the knowledge questions correctly; scores ranged from 0 to 26 questions correct out of 30 (mean = 14.71 ± 5.2, median = 15). Mean knowledge scores were higher among those reporting a family member with osteoporosis, nurses (vs other vocations), and women with a high school education or greater (relative to those who had not completed high school). More than 90% of the women expressed interest in a prevention and treatment program. Vietnamese women may have heard of osteoporosis, yet they would benefit from education targeting prevention and treatment of the disease. © 2011 APJPH.

What People with Diabetes Need to Know about Osteoporosis

MedlinePlus

... What People With Diabetes Need to Know About Osteoporosis What Is Diabetes? Diabetes is a disorder of ... who are older, overweight, and inactive. What Is Osteoporosis? Osteoporosis is a condition in which the bones ...

What Prostate Cancer Survivors Need to Know about Osteoporosis

MedlinePlus

... What Prostate Cancer Survivors Need to Know About Osteoporosis The Impact of Prostate Cancer The National Cancer ... Management Strategies Resources For Your Information Facts About Osteoporosis Osteoporosis is a condition in which bones become ...

What People with Asthma Need to Know about Osteoporosis

MedlinePlus

... What People With Asthma Need to Know About Osteoporosis What Is Asthma? According to the National Heart, ... Management Strategies Resources For Your Information What Is Osteoporosis? Osteoporosis is a condition in which the bones ...

What Breast Cancer Survivors Need to Know about Osteoporosis

MedlinePlus

... What Breast Cancer Survivors Need to Know About Osteoporosis The Impact of Breast Cancer Other than skin ... Management Strategies Resources For Your Information Facts About Osteoporosis Osteoporosis is a condition in which the bones ...

Estimating prevalence of osteoporosis: examples from industrialized countries.

PubMed

Wade, S W; Strader, C; Fitzpatrick, L A; Anthony, M S; O'Malley, C D

2014-01-01

In nine industrialized countries in North America, Europe, Japan, and Australia, country-specific osteoporosis prevalence (estimated from published data) at the total hip or hip/spine ranged from 9 to 38 % for women and 1 to 8 % for men. In these countries, osteoporosis affects up to 49 million individuals. Standardized country-specific prevalence estimates are scarce, limiting our ability to anticipate the potential global impact of osteoporosis. This study estimated the prevalence of osteoporosis in several industrialized countries (USA, Canada, five European countries, Australia, and Japan) using the World Health Organization (WHO) bone mineral density (BMD)-based definition of osteoporosis: BMD T-score assessed by dual-energy x-ray absorptiometry ≤-2.5. Osteoporosis prevalence was estimated for males and females aged 50 years and above using total hip BMD and then either total hip or spine BMD. We compiled published location-specific data, using the National Health and Nutrition Examination Survey (NHANES) III age and BMD reference groups, and adjusted for differences in disease definitions across sources. Relevant NHANES III ratios (e.g., male to female osteoporosis at the total hip) were applied where data were missing for countries outside the USA. Data were extrapolated from geographically similar countries as needed. Population counts for 2010 were used to estimate the number of individuals with osteoporosis in each country. For females, osteoporosis prevalence ranged from 9 % (UK) to 15 % (France and Germany) based on total hip BMD and from 16 % (USA) to 38 % (Japan) when spine BMD data were included. For males, prevalence ranged from 1 % (UK) to 4 % (Japan) based on total hip BMD and from 3 % (Canada) to 8 % (France, Germany, Italy, and Spain) when spine BMD data were included. Up to 49 million individuals met the WHO osteoporosis criteria in a number of industrialized countries in North America, Europe, Japan, and Australia.

Idiopathic Inflammatory Myopathies

PubMed Central

Dimachkie, Mazen M.; Barohn, Richard J.

2012-01-01

The idiopathic inflammatory myopathies are a group of rare disorders including polymyositis (PM), dermatomyositis (DM), and autoimmune necrotizing myopathies (NMs). The idiopathic inflammatory myopathies share many similarities. They present acutely, subacutely, or chronically with marked proximal and symmetric muscle weakness, except for associated distal and asymmetric weakness in inclusion body myositis. The idiopathic inflammatory myopathies also share a variable degree of creatine kinase (CK) elevation and a nonspecifically abnormal electromyogram demonstrating an irritative myopathy. The muscle pathology demonstrates inflammatory exudates of variable distribution within the muscle fascicle. Despite these similarities, the idiopathic inflammatory myopathies are a heterogeneous group. The overlap syndrome (OS) refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus. In addition to elevated antinuclear antibodies (ANA), patients with OS may be weaker in the proximal arms than the legs mimicking the pattern seen in some muscular dystrophies. In this review, we focus on DM, PM, and NM and examine current and promising therapies. PMID:23117947

Quantitative imaging methods in osteoporosis.

PubMed

Oei, Ling; Koromani, Fjorda; Rivadeneira, Fernando; Zillikens, M Carola; Oei, Edwin H G

2016-12-01

Osteoporosis is characterized by a decreased bone mass and quality resulting in an increased fracture risk. Quantitative imaging methods are critical in the diagnosis and follow-up of treatment effects in osteoporosis. Prior radiographic vertebral fractures and bone mineral density (BMD) as a quantitative parameter derived from dual-energy X-ray absorptiometry (DXA) are among the strongest known predictors of future osteoporotic fractures. Therefore, current clinical decision making relies heavily on accurate assessment of these imaging features. Further, novel quantitative techniques are being developed to appraise additional characteristics of osteoporosis including three-dimensional bone architecture with quantitative computed tomography (QCT). Dedicated high-resolution (HR) CT equipment is available to enhance image quality. At the other end of the spectrum, by utilizing post-processing techniques such as the trabecular bone score (TBS) information on three-dimensional architecture can be derived from DXA images. Further developments in magnetic resonance imaging (MRI) seem promising to not only capture bone micro-architecture but also characterize processes at the molecular level. This review provides an overview of various quantitative imaging techniques based on different radiological modalities utilized in clinical osteoporosis care and research.

HLA-B27 subtypes in enthesitis-related arthritis category of juvenile idiopathic arthritis and ankylosing spondylitis in northern India.

PubMed

Srivastava, R; Agnihotry, S; Aggarwal, R; Bajpai, P; Aggarwal, A

2015-01-01

Enthesitis-related arthritis (ERA) is the most common form of juvenile idiopathic arthritis (JIA) in the Asian and Indian populations. The presence of HLA-B27 has a strong association with JIA-ERA similar to that with adult ankylosing spondylitis (AS). The HLA-B27gene is highly polymorphic. Susceptibility to AS varies between different HLA-B27 subtypes; data on the relationship of susceptibility to JIA-ERA with HLA-B27 types are scant. In this study, we determined HLA-B27 subtypes in patients with JIA-ERA and AS to find out whether there is any difference in the HLA-B27 subtypes prevalent in these two diseases. Genomic DNA from 135 patients with JIA-ERA and 121 with AS was tested for the presence of HLA-B27. In patients testing positive, HLA-B27subtyping was done by sequencing a genomic region that contained second and third exons and the intervening intron of this gene; this method permitted identification of common HLA-B27 subtypes (HLA-B*27:01 to HLA-B*27:09). One hundred and seven (79%) patients with JIA-ERA and 102 (84%) patients with AS tested positive for HLA-B27. In both groups, HLA-B*27:05 and HLA-B*27:04 were the common subtypes; some patients had HLA-B*27:07(7.4%) and HLA-B*27:18. Patients with JIA-ERA had a higher frequency of HLA-B*27:05 than those with AS (70% vs. 57%, p=0.047), and a lower frequency of HLA-B*27:04 (21% vs. 36%, p=0.018). HLA-B*27:05 and HLA-B*27:04 were the most common HLA-27 subtypes in both JIA-ERA and AS. However, HLA-B*27:05 was more frequent and HLA-B*27:04 was less frequent in JIA-ERA. It is possible that HLA-B*27:05 being the ancestral HLA-27 subtype leads to expression of disease early in life.

Determining the cut-off point of osteoporosis based on the osteoporosis self-assessment tool, body mass index and weight in Taiwanese young adult women.

PubMed

Chang, Shu Fang; Yang, Rong Sen

2014-09-01

To examine the cut-off point of the osteoporosis self-assessment tool, age, weight and body mass index for osteoporosis among young adult Taiwanese women, using a large-scale health examination database containing bone mineral density tests. The cut-off points of osteoporosis risk factors identified earlier focus on menopausal or senior Caucasian and Asian women. However, young adult Asian women have seldom been identified. A retrospective historical cohort study. Using the 2009-2011 health examination database of a large-scale medical centre in northern Taiwan, this study investigated young adult Asian women (i.e. range in age from 30-49 years) in Taiwan who had received dual-energy X-ray absorptiometry test. This study also explored the cut-off point, sensitivity, specificity and diagnostic accuracy of receiver operating characteristics of osteoporosis among young adult females in Taiwan. This study collected 2454 young adult Asian women in Taiwan. Cochran-Armitage analysis results indicated that the prevalence of osteoporosis increased with decreasing weight, body mass index and osteoporosis self-assessment method quartiles. According to the results of receiver operating characteristics, weight, body mass index and osteoporosis self-assessment tool approaches can generally be used as indicators to predict osteoporosis among young adult Asian women. Results of this study demonstrate that Taiwanese women contracting osteoporosis tend to be young and underweight, as well as having a low body mass index and osteoporosis self-assessment scores. Those results further suggest that the assessment indicators for cut-off points are appropriately suitable for young adult women in Taiwan. Early detection is the only available means of preventing osteoporosis. Professional nurses should apply convenient and accurate assessment procedures to help young adult women to adopt preventive strategies against osteoporosis early, thus eliminating the probability of osteoporotic

Evaluation of the association between Hispanic ethnicity and disease activity and severity in a large cohort of patients with juvenile idiopathic arthritis.

PubMed

Pelajo, Christina F; Angeles-Han, Sheila T; Prahalad, Sampath; Sgarlat, Caitlin M; Davis, Trevor E; Miller, Laurie C; Lopez-Benitez, Jorge M

2013-10-01

To examine the association between ethnicity and disease activity in patients with juvenile idiopathic arthritis (JIA), and to determine the association of ethnicity with disease severity and disability in this population. CARRAnet, a US database containing information (collected between May 2010 and June 2011) on almost 3,000 subjects with JIA, was used. Demographic variables were compared between Hispanic patients and non-Hispanic patients. Mann-Whitney and chi-square tests were used to compare indicators of disease activity, as well as imaging evidence of joint damage, and Childhood Health Assessment Questionnaire (CHAQ) scores between ethnicities. Two linear regression models were used to determine the association of ethnicity with number of active joints in JIA, and the association between ethnicity and disability (CHAQ scores). A total of 2,704 patients with JIA (277 Hispanic; 2,427 non-Hispanic) were included. Income and health insurance coverage were higher in non-Hispanics. RF-positive polyarticular JIA, positive RF and anti-CCP, as well as use of systemic steroids were more frequent in Hispanics. Imaging evidence of joint damage was present in 32 % of the Hispanic patients compared to 24 % of the non-Hispanic patients (p = 0.008). In multivariate linear regression analyses, the number of active joints was significantly higher in Hispanics than in non-Hispanics (p = 0.03), as well as CHAQ scores (p = 0.003), after adjusting for confounders. Hispanic patients with JIA had higher disease activity than non-Hispanic patients, as well as higher disease severity and disability. Since ethnicity influences disease activity, severity, and disability, different management and treatment plans should be planned accordingly.

Understanding Preferences for Osteoporosis Information to Develop an Osteoporosis Patient Education Brochure

PubMed Central

Edmonds, Stephanie W; Solimeo, Samantha L; Nguyen, Vu-Thuy; Wright, Nicole C; Roblin, Douglas W; Saag, Kenneth G; Cram, Peter

2017-01-01

Context Patient education materials can provide important information related to osteoporosis prevention and treatment. However, available osteoporosis education materials fail to follow best-practice guidelines for patient education. Objective To develop an educational brochure on bone health for adults aged 50 years and older using mixed-method, semistructured interviews. Design This project consisted of 3 phases. In Phase 1, we developed written content that included information about osteoporosis. Additionally, we designed 2 graphic-rich brochures, Brochure A (photographs) and Brochure B (illustrations). In Phase 2, interviewers presented the text-only document and both brochure designs to 53 participants from an academic Medical Center in the Midwest and an outpatient clinic in the Southeastern region of the US. Interviewers used open- and closed-ended questions to elicit opinions regarding the brochures. In Phase 3, using feedback from Phase 2, we revised the brochure and presented it to 11 participants at a third site in the Southeastern US. Main Outcome Measures Participants’ comprehension of brochure text and acceptability of brochure design. Results We enrolled 64 participants. Most were women, white, and college-educated, with an average age of 66.1 years. Participants were able to restate the basic content of the brochure and preferred Brochure A’s use of photographs. Conclusions Using feedback from older adults, we developed and refined a brochure for communicating bone health information to older adults at risk of osteoporosis and fragility fractures. The methods outlined in this article may serve to guide others in developing health educational brochures for chronic medical conditions. PMID:28080957

Short term effectiveness and experiences of a peer guided web-based self-management intervention for young adults with juvenile idiopathic arthritis.

PubMed

Ammerlaan, Judy; van Os-Medendorp, Harmieke; de Boer-Nijhof, Nienke; Scholtus, Lieske; Kruize, Aike A; van Pelt, Philomine; Prakken, Berent; Bijlsma, Hans

2017-10-13

A web-based self-management intervention guided by peer-trainers was developed to support young adults' self-management in coping with Juvenile Idiopathic Arthritis (JIA). To investigate its effectiveness, a randomized controlled trial (RCT) was conducted. In addition, the content of the chat and participants' goals were studied to identify underlying processes. An RCT with a six-month follow up period was conducted among 72 young adults with JIA, aged between 16 and 25 years old, randomly assigned to the intervention or to the usual care control group. After 24 weeks, in both groups 24 participants completed all measurements. Intentions to treat analyses were carried out by means of linear mixed models for longitudinal measurements. With self-efficacy as primary outcome, self-management, disease activity, quality of life, absenteeism of school/work, health care medication use and adherence to the intervention were studied. The participants' goals, personal achievements, interactions on the chat, and their appreciation of the intervention were analyzed using thematic analyses. No significant differences were found on self-efficacy, quality of life, and self-management between the participants of the control group and the intervention group. In the intervention group, modeling and sharing experiences were the most recognized themes. Fifty-five goals were formulated and divided into the following categories: improvement and maintaining balance, setting and recognizing boundaries, communicating and coping with incomprehension. Adherence, appreciation of the own learning experience, and personal achievements were rated positively. The web-based intervention did not lead to an improvement of self-efficacy. However, additional qualitative analyses showed that the intervention was appreciated and valuable for the participants. More research is needed on how to measure the added value of this intervention compared to the usual care. Trial registration number NTR4679 .

HLA-B27 predicts a more chronic disease course in an 8-year followup cohort of patients with juvenile idiopathic arthritis.

PubMed

Berntson, Lillemor; Nordal, Ellen; Aalto, Kristiina; Peltoniemi, Suvi; Herlin, Troels; Zak, Marek; Nielsen, Susan; Rygg, Marite

2013-05-01

We investigated associations of HLA-B27 with clinical manifestations and longterm outcome in a near population-based setting among patients with juvenile idiopathic arthritis (JIA). We studied clinical and serological data from 410 patients with HLA-B27 results among 440 prospectively collected patients with JIA with 8-year followup data in a Nordic database. The study was structured to be as close to a population-based study as possible. HLA-B27 was analyzed in 93% of patients, and was positive in 21% of the cohort, in 18.4% of the girls and in 25.9% of the boys. Boys who were HLA-B27-positive had significantly higher age at onset compared to HLA-B27-negative boys and compared to both HLA-B27-negative and positive girls. This difference in onset age in relation to HLA-B27 was not found in girls. HLA-B27 was associated with clinical signs of sacroiliitis, enthesitis, and tenosynovitis in boys, but not in girls. After 8 years of disease, 46 children (11.2%) were classified as having enthesitis-related arthritis (ERA). Boys with ERA had clinical signs of sacroiliitis more often than girls with ERA. HLA-B27-positive children, as well as children with clinical signs of sacroiliitis, enthesitis, and hip arthritis, had higher odds of not being in remission off medication after 8 years of disease. In this near population-based Nordic JIA cohort we found significant differences between HLA-B27-positive boys and girls in age at disease onset, clinical signs of sacroiliitis, and ERA classification. HLA-B27 was negatively associated with longterm remission status, possibly because of its association with clinical disease characteristics, such as sacroiliitis, rather than being a general marker of persistent disease.

HLA-DRB1*11 and variants of the MHC class II locus are strong risk factors for systemic juvenile idiopathic arthritis.

PubMed

Ombrello, Michael J; Remmers, Elaine F; Tachmazidou, Ioanna; Grom, Alexei; Foell, Dirk; Haas, Johannes-Peter; Martini, Alberto; Gattorno, Marco; Özen, Seza; Prahalad, Sampath; Zeft, Andrew S; Bohnsack, John F; Mellins, Elizabeth D; Ilowite, Norman T; Russo, Ricardo; Len, Claudio; Hilario, Maria Odete E; Oliveira, Sheila; Yeung, Rae S M; Rosenberg, Alan; Wedderburn, Lucy R; Anton, Jordi; Schwarz, Tobias; Hinks, Anne; Bilginer, Yelda; Park, Jane; Cobb, Joanna; Satorius, Colleen L; Han, Buhm; Baskin, Elizabeth; Signa, Sara; Duerr, Richard H; Achkar, J P; Kamboh, M Ilyas; Kaufman, Kenneth M; Kottyan, Leah C; Pinto, Dalila; Scherer, Stephen W; Alarcón-Riquelme, Marta E; Docampo, Elisa; Estivill, Xavier; Gül, Ahmet; de Bakker, Paul I W; Raychaudhuri, Soumya; Langefeld, Carl D; Thompson, Susan; Zeggini, Eleftheria; Thomson, Wendy; Kastner, Daniel L; Woo, Patricia

2015-12-29

Systemic juvenile idiopathic arthritis (sJIA) is an often severe, potentially life-threatening childhood inflammatory disease, the pathophysiology of which is poorly understood. To determine whether genetic variation within the MHC locus on chromosome 6 influences sJIA susceptibility, we performed an association study of 982 children with sJIA and 8,010 healthy control subjects from nine countries. Using meta-analysis of directly observed and imputed SNP genotypes and imputed classic HLA types, we identified the MHC locus as a bona fide susceptibility locus with effects on sJIA risk that transcended geographically defined strata. The strongest sJIA-associated SNP, rs151043342 [P = 2.8 × 10(-17), odds ratio (OR) 2.6 (2.1, 3.3)], was part of a cluster of 482 sJIA-associated SNPs that spanned a 400-kb region and included the class II HLA region. Conditional analysis controlling for the effect of rs151043342 found that rs12722051 independently influenced sJIA risk [P = 1.0 × 10(-5), OR 0.7 (0.6, 0.8)]. Meta-analysis of imputed classic HLA-type associations in six study populations of Western European ancestry revealed that HLA-DRB1*11 and its defining amino acid residue, glutamate 58, were strongly associated with sJIA [P = 2.7 × 10(-16), OR 2.3 (1.9, 2.8)], as was the HLA-DRB1*11-HLA-DQA1*05-HLA-DQB1*03 haplotype [6.4 × 10(-17), OR 2.3 (1.9, 2.9)]. By examining the MHC locus in the largest collection of sJIA patients assembled to date, this study solidifies the relationship between the class II HLA region and sJIA, implicating adaptive immune molecules in the pathogenesis of sJIA.

Pathogenesis and management of primary osteoporosis.

PubMed

Bauwens, S F; Drinka, P J; Boh, L E

1986-08-01

The pathophysiology of primary osteoporosis and the various therapeutic regimens that have been used are reviewed. Osteoporosis is a major public health problem because the incidence of hip, wrist, and vertebral fractures associated with bone loss is high. Postmenopausal women are at increased risk for developing osteoporosis because bone mineral content is lower in women than in men, dietary calcium intake is frequently insufficient, intestinal absorption of calcium decreases with age, and the rate of bone loss accelerates at menopause. The efficacy of many single and combination therapies in preventing or treating osteoporosis has been studied. Differences in study design and diagnostic techniques and the heterogeneous nature of osteoporosis make evaluation of clinical trials difficult. Exercise helps to maintain skeletal mass, but amenorrhea caused by vigorous activity may be harmful. The efficacy of estrogen replacement therapy is documented best; many studies have shown that estrogens slow the rate of bone loss and reduce the incidence of fractures, but the association of estrogen use with endometrial cancer and breast cancer is of concern. Progesterones may protect against endometrial cancer, but undesirable effects of oral contraceptives have resulted in a hesitancy to use combination hormonal therapy. All adults should meet daily nutritional requirements for calcium, but this intake may be insufficient for elderly persons and is below recommended doses for treating osteoporosis. A daily intake of at least 1000-1500 mg of elemental calcium has been shown to slow the rate of bone loss. Nutritional requirements for vitamin D should be met, but benefits from pharmacologic doses have not been demonstrated. The role of fluoride, calcitonin, anabolic steroids, and vitamin D metabolites is unclear. Fluoride has the potential to increase bone mass, but effects on bone histology and fracture rates require further study. The major goals for the management of

Evaluating The Risk Of Osteoporosis Through Bone Mass Density.

PubMed

Sayed, Sayeeda Amber; Khaliq, Asif; Mahmood, Ashar

2016-01-01

Osteoporosis is a bone disorder, characterized by loss of bone mass density. Osteoporosis affects more than 30% of post-menopausal women. Osteoporosis is often associated with restricted body movement, pain and joint deformities. Early identification and early intervention can help in reducing these complications. The primary objective of this study was to estimate the burden of Osteoporosis in Urban setting of Sindh among women of different age groups and to access the effect of different protective measures that can reduce the risk of Osteoporosis. In this study, 500 women's of 3 major cities of Sindh were approached by non-probability convenience sampling technique. Women bearing age 20 years or more were included. Women who fall under inclusion criteria were screened for BMD (Bone mineral density) test and were classified as Healthy, Osteopenic and Osteoporotic based on their T-score. The association of different protective measures and risk of osteoporosis was assessed by prevalence relative risk (PRR). The result of this study indicate that the burden of Osteoporosis is very high among the women of Sindh, only 17.4% (84) women were found to have normal BMD score. The life style of majority of women was sedentary. The PRR calculated for Exposure to sunlight, regular exercise, and use of nutritional supplement was 12.5, 5.19 and 2.72 folds respectively. The results of study reveal that exposure to sunlight, regular physical exercise and use of nutritional supplements found to be effective in reducing the risk of osteoporosis among women of all age group. Health education and promotion toward osteoporosis prevention can significantly contribute in reducing the morbidity of osteoporosis.

Osteoporosis and body composition.

PubMed

Crepaldi, G; Romanato, G; Tonin, P; Maggi, S

2007-01-01

The Epidemiologic Study on the Prevalence of Osteoporosis in Italy showed that the prevalence of osteoporosis among women and men aged 60 yr and over is 22.8% and 14.5%, respectively, giving rise to about 80,000 new fractures a yr. Sarcopenia is considered to be one of the main features of the aging process. It is characterized by a reduction in muscle mass and muscle strength, and affects women more than men. It is associated with a increased risk of fractures consequent upon a greater predisposition to falls, but also to the lack of bone remodeling due to reduced muscle mechanical strength. Muscle strength determines quality bone modifications such as density, strength, and microarchitecture. Variations in the ratios of cortical and muscle areas give rise to various types of osteoporosis, with different risks of fracture. Bone mineral density increases with body fat mass, and obesity has a protective effect against osteoporosis. This protective effect is explained by a combination of hormonal (peripheral aromatization of androgens to estrogens in adipose tissue) and mechanical factors (on weight-bearing bone sites), but the hormone leptin also probably mediates fat and bone mass. Serum leptin levels are closely related to body fat mass, and some findings suggest the peripheral effect of leptin, which exerts estrogenic effects, enhancing osteoblastic differentiation and inhibiting late adipocytic differentiation. The overall effect of leptin on bone results from a balance between negative central effects and positive direct peripheral effects, according to serum leptin levels.

Animal models for osteoporosis

NASA Technical Reports Server (NTRS)

Turner, R. T.; Maran, A.; Lotinun, S.; Hefferan, T.; Evans, G. L.; Zhang, M.; Sibonga, J. D.

2001-01-01

Animal models will continue to be important tools in the quest to understand the contribution of specific genes to establishment of peak bone mass and optimal bone architecture, as well as the genetic basis for a predisposition toward accelerated bone loss in the presence of co-morbidity factors such as estrogen deficiency. Existing animal models will continue to be useful for modeling changes in bone metabolism and architecture induced by well-defined local and systemic factors. However, there is a critical unfulfilled need to develop and validate better animal models to allow fruitful investigation of the interaction of the multitude of factors which precipitate senile osteoporosis. Well characterized and validated animal models that can be recommended for investigation of the etiology, prevention and treatment of several forms of osteoporosis have been listed in Table 1. Also listed are models which are provisionally recommended. These latter models have potential but are inadequately characterized, deviate significantly from the human response, require careful choice of strain or age, or are not practical for most investigators to adopt. It cannot be stressed strongly enough that the enormous potential of laboratory animals as models for osteoporosis can only be realized if great care is taken in the choice of an appropriate species, age, experimental design, and measurements. Poor choices will results in misinterpretation of results which ultimately can bring harm to patients who suffer from osteoporosis by delaying advancement of knowledge.

[Glucocorticoid induced osteoporosis].

PubMed

Anić, Branimir; Mayer, Miroslav

2014-01-01

Secondary osteoporosis most often develops due to glucocorticoid therapy. Glucocorticoids affect all stages of the bone remodeling cycle, its formation and resorption. Osteoblasts are primarily affected, decreasing their activity and enhancing apoptosis. Patients treated with glucocorticoids have lower bone mineral density and increased fracture risk. Glucocorticoid-induced osteoporosis can be prevented by administering the minimal effective dose of glucocorticoids, calcium and vitamin D supplementation or, if possible, by hormone replace- ment therapy. Moreover, appropriate physical activity should be encouraged. Patients who are at higher risk for low-energy fractures (for example post-menopausal women) have to be actively treated, usually with antiresorptive drugs among which bisphosphonates are currently the first line therapy.

Osteomalacia and osteoporosis associated with primary intestinal lymphangiectasis.

PubMed

Li, Xin-Ping; Shen, Wen-Bin; Long, Ming-Qing; Meng, Xun-Wu; Lian, Xiao-Lan; Yu, Miao

2012-05-01

Primary Intestinal lymphangiectasia (PIL) is a common cause of protein losing enteropathy (PLE). It will affect enter-hepatic circulation of lipid-soluble vitamin, and absorption of electrolytes, cause malnutrition related osteomalacia or osteoporosis. While seldom health care workers noted to assess and treat osteomalacia or osteoporosis in PIL. Here we report a related case. We found increased parathyroid hormone, decreased 25(OH)D3, low bone mineral density, which indicated that the PIL patient had osteomalacia and/or osteoporosis. Adequate calcium and vitamin D supply can relieve the condition efficaciously. We should pay attention to osteomalacia and osteoporosis in PIL patients.

Validation of an osteoporosis self-assessment tool to identify primary osteoporosis and new osteoporotic vertebral fractures in postmenopausal Chinese women in Beijing

PubMed Central

2013-01-01

Background This study aimed to validate the effectiveness of the Osteoporosis Self-assessment Tool for Asians (OSTA) in identifying postmenopausal women at increased risk of primary osteoporosis and painful new osteoporotic vertebral fractures in a large selected Han Chinese population in Beijing. Methods We assessed the performance of the OSTA in 1201 women. Subjects with an OSTA index > -1 were classified as the low risk group, and those with an index ≤ -1 were classified as the increased risk group. Osteoporosis is defined by a T-score ≤ 2.5 standard deviations according to the WHO criteria. All painful, new vertebral fractures were identified by X-ray and MRI scans with correlating clinical signs and symptoms. We determined the sensitivity, specificity, and area under the receiver operating characteristic (ROC) curve for correctly selecting women with osteoporosis and painful new vertebral fractures. Results Of the study subjects, 29.3% had osteoporosis, and the prevalence of osteoporosis increased progressively with age. The areas under the ROC curves of the OSTA index (cutoff = -1) to identify osteoporosis in the femoral neck, total hip, and lumbar spine were 0.824, 0.824, and 0.776, respectively. The sensitivity and specificity of the OSTA index (cutoff = -1) to identify osteoporosis in healthy women were 66% and 76%, respectively. With regard to painful new vertebral fractures, the area under the ROC curve relating the OSTA index (cutoff = -1) to new vertebral fractures was 0.812. Conclusions The OSTA may be a simple and effective tool for identifying the risk of osteoporosis and new painful osteoporotic vertebral fractures in Han Chinese women. PMID:24053509

What People with Lupus Need to Know about Osteoporosis

MedlinePlus

... Need to Know About Osteoporosis What People With Lupus Need to Know About Osteoporosis What Is Lupus? Lupus is an autoimmune disease, a disorder in ... new habits for healthy bones. The Link Between Lupus and Osteoporosis Studies have found an increase in ...

Use of Osteoporosis Medications in Older Nursing Facility Residents

PubMed Central

Wright, Rollin M.

2007-01-01

Introduction Epidemiologic studies demonstrated that 70 – 85% of nursing home residents have osteoporosis. Few studies report comprehensive information about treatment of osteoporosis in nursing facilities. Objective To determine the prevalence osteoporosis treatment and identify resident characteristics associated with the use of anti-resorptive medications or supplements indicated to treat osteoporosis in nursing homes. Methods The study design was cross-sectional. The Systematic Assessment of Geriatric Drug Use via Epidemiology database provided the data. From this database, 186,221 residents were identified as newly admitted to nursing facilities in Kansas, Maine, Missouri, Ohio, and South Dakota between 1998 and 2000. The outcome measure was the use of anti-resorptive medications (alendronate, risedronate, calcitonin, estrogen, raloxifene) or supplements (calcium with vitamin D) indicated for treatment of osteoporosis. The independent variables included demographic, health status and fracture risk factors. Results Of the overall sample, 9.1% received anti-resorptive medications and/or supplements indicated for osteoporosis treatment. The most commonly used treatment was the combination of calcium and vitamin D (5.0%). Calcitonin (2.5%) use exceeded that of any other anti-resorptive. Multivariable logistic regression analyses revealed that a diagnosis of osteoporosis and female gender were strongly associated with being more likely to receive an osteoporosis treatment (OR 6.34 with 95% CI 6.11–6.64 and OR 2.67 with 95% CI 2.53–2.83 respectively). The number of medications residents received was also strongly associated with receiving osteoporosis treatment. Being black and having four or more active diagnoses were strongly associated with lower odds of receiving treatment (OR 0.63 with 95% CI 0.57–0.68 and OR 0.71 with 95% CI 0.68–0.74 for 4 to 6 diagnoses). Discussion Newly admitted nursing facility residents infrequently received an indicated

District nurses' perceptions of osteoporosis management: a qualitative study.

PubMed

Claesson, A; Toth-Pal, E; Piispanen, P; Salminen, H

2015-07-01

Underdiagnosis of osteoporosis is common. This study investigated Swedish district nurses' perceptions of osteoporosis management. They perceived the condition as having low priority, and the consequences of this perception were insufficient awareness of the condition and perceptions of bone-specific medication as unsafe. They perceived, though, competency when working with fall prevention. Undertreatment of patients with osteoporosis is common. Sweden's medical care strategy dictates prioritisation of various conditions; while guidelines exist, osteoporosis is not prioritised. The aim of this study was to investigate district nurses' perceptions of osteoporosis management within Sweden's primary health care system. Four semi-structured focus group interviews were conducted with 13 female district nurses. The interviews were analysed using thematic analysis. The overall theme was perceiving osteoporosis management as ambiguous. The themes were perceiving barriers and perceiving opportunities. These subthemes were linked to perceiving barriers: (i) insufficient procedures, lack of time and not aware of the condition; (ii) insufficient knowledge about diagnosis and about fracture risk assessment tools; (iii) low priority condition and unclear responsibility for osteoporosis management; and (iv) bone-specific medication was sometimes perceived to be unsafe. These subthemes were linked to perceiving opportunities: (i) professional competency when discussing fall prevention in home visit programs, (ii) willingness to learn more about osteoporosis management, (iii) collaboration with other professionals and (iv) willingness to identify individuals at high risk of fracture. Osteoporosis was reported, by the district nurses, to be a low-priority condition with consequences being unawareness of the condition, insufficient knowledge about bone-specific medications, fracture risk assessment tools and procedures. These may be some of the explanations for the undertreatment of

Osteoporosis: strategies for prevention and management.

PubMed

Keen, Richard

2007-02-01

Osteoporosis is a serious public health issue, affecting up to 1 in 2 women and 1 in 5 men over the age of 50 years. The common osteoporotic fractures occur at the spine, wrist and hip. For the patient affected by osteoporosis, these fractures are associated with significant morbidity and, in the case of hip and spine fractures, an excess mortality. The treatment of osteoporotic fractures is also associated with a significant healthcare cost for society. Currently, measurement of bone mineral density using dual energy X-ray absorptiometry is the gold standard for the diagnosis of osteoporosis. In the future, however, assessment of fracture risk will be based on algorithms incorporating clinical risk factors and bone density measurements, where appropriate. The goal of treatment is to reduce the risk of future fracture. Patients at high risk for fracture should be assessed and screened to exclude secondary causes for osteoporosis. Bisphosphonates (alendronate, etidronate, ibandronate, risedronate) are the first-line therapy for the majority of patients and these treatments can be given either orally or intravenously. Alternative treatment options include strontium ranelate and raloxifene. Anabolic therapy with parathyroid hormone can be considered for patients with severe disease. These patients will often require referral for specialist assessment and monitoring. All patients at risk of developing osteoporosis should be given lifestyle advice regarding dietary intake of calcium and vitamin D and regular weight-bearing exercise.

Osteoporosis and vertebral fractures in men aged 60-74 years.

PubMed

Frost, Morten; Wraae, Kristian; Abrahamsen, Bo; Høiberg, Mikkel; Hagen, Claus; Andersen, Marianne; Brixen, Kim

2012-03-01

limited information on the prevalence of osteoporosis and VFxs in men in high-risk populations is available. The choice of reference values for dual X-ray absorptiometry (DXA) is debated. We evaluated the prevalence of osteoporosis and vertebral deformities in a population-based sample of men. bone mineral density (BMD) was measured and vertebral deformities assessed using DXA and VFx assessment (VFA), respectively, in a random sample of 600 Danish men aged 60-74 years. Osteoporosis was defined as a T-score of -2.5 or less. the study population was comparable with the background population with regard to age, body mass index and co-morbidity. Osteoporosis was diagnosed in less than 1% of the participants at inclusion. Using Danish and NHANES III reference data, 10.2 and 11.5% of the study population had osteoporosis, respectively. In all, 6.3% participants had at least one VFx. BMD was significantly lower in participants with vertebral deformities, but only 24% of these cases had osteoporosis. osteoporosis and VFxs are prevalent in men aged 60-74 years. Although the majority of deformities were present in individuals without osteoporosis, BMD was lower in patients with VFxs at all sites investigated. Male osteoporosis was markedly underdiagnosed.

An Increased Risk of Osteoporosis during Acquired Immunodeficiency Syndrome

PubMed Central

2004-01-01

Osteoporosis is characterized by decreased bone mineral density and mechanistic imbalances of bone tissue that may result in reduced skeletal strength and an enhanced susceptibility to fractures. Osteoporosis in its most common form affects the elderly (both sexes) and all racial groups of human beings. Multiple environmental risk factors like acquired immune deficiency syndrome (AIDS) are believed to be one of the causes of osteoporosis. Recently a high incidence of osteoporosis has been observed in human immunodeficiency virus (HIV) infected individuals. The etiology of this occurrence in HIV infections is controversial. This problem seems to be more frequent in patients receiving potent antiretroviral therapy. In AIDS, the main suggested risk factors for the development of osteoporosis are use of protease inhibitors, longer duration of HIV infection, lower body weight before antiretroviral therapy, high viral load. Variations in serum parameters like osteocalcin, c-telopeptide, levels of elements like Calcium, Magnesium, Phosphorus, concentration of vitamin-D metabolites, lactate levels, bicarbonate concentrations, amount of alkaline phosphatase are demonstrated in the course of development of osteoporosis. OPG/RANKL/RANK system is final mediator of bone remodeling. Bone mineral density (BMD) test is of added value to assess the risk of osteoporosis in patients infected with AIDS. The biochemical markers also aid in this assessment. Clinical management mostly follows the lines of treatment of osteoporosis and osteopenia. PMID:15912194

An Increased Risk of Osteoporosis during Acquired Immunodeficiency Syndrome.

PubMed

Annapoorna, N; Rao, G Venkateswara; Reddy, N S; Rambabu, P; Rao, K R S Samabasiva

2004-01-01

Osteoporosis is characterized by decreased bone mineral density and mechanistic imbalances of bone tissue that may result in reduced skeletal strength and an enhanced susceptibility to fractures. Osteoporosis in its most common form affects the elderly (both sexes) and all racial groups of human beings. Multiple environmental risk factors like acquired immune deficiency syndrome (AIDS) are believed to be one of the causes of osteoporosis. Recently a high incidence of osteoporosis has been observed in human immunodeficiency virus (HIV) infected individuals. The etiology of this occurrence in HIV infections is controversial. This problem seems to be more frequent in patients receiving potent antiretroviral therapy. In AIDS, the main suggested risk factors for the development of osteoporosis are use of protease inhibitors, longer duration of HIV infection, lower body weight before antiretroviral therapy, high viral load. Variations in serum parameters like osteocalcin, c-telopeptide, levels of elements like Calcium, Magnesium, Phosphorus, concentration of vitamin-D metabolites, lactate levels, bicarbonate concentrations, amount of alkaline phosphatase are demonstrated in the course of development of osteoporosis. OPG/RANKL/RANK system is final mediator of bone remodeling. Bone mineral density (BMD) test is of added value to assess the risk of osteoporosis in patients infected with AIDS. The biochemical markers also aid in this assessment. Clinical management mostly follows the lines of treatment of osteoporosis and osteopenia.

Osteoporosis in men: a review.

PubMed

Adler, Robert A

2014-01-01

Osteoporosis and consequent fracture are not limited to postmenopausal women. There is increasing attention being paid to osteoporosis in older men. Men suffer osteoporotic fractures about 10 years later in life than women, but life expectancy is increasing faster in men than women. Thus, men are living long enough to fracture, and when they do the consequences are greater than in women, with men having about twice the 1-year fatality rate after hip fracture, compared to women. Men at high risk for fracture include those men who have already had a fragility fracture, men on oral glucocorticoids or those men being treated for prostate cancer with androgen deprivation therapy. Beyond these high risk men, there are many other risk factors and secondary causes of osteoporosis in men. Evaluation includes careful history and physical examination to reveal potential secondary causes, including many medications, a short list of laboratory tests, and bone mineral density testing by dual energy X-ray absorptiometry (DXA) of spine and hip. Recently, international organizations have advocated a single normative database for interpreting DXA testing in men and women. The consequences of this change need to be determined. There are several choices of therapy for osteoporosis in men, with most fracture reduction estimation based on studies in women.

Osteoporosis in men: a review

PubMed Central

Adler, Robert A

2014-01-01

Osteoporosis and consequent fracture are not limited to postmenopausal women. There is increasing attention being paid to osteoporosis in older men. Men suffer osteoporotic fractures about 10 years later in life than women, but life expectancy is increasing faster in men than women. Thus, men are living long enough to fracture, and when they do the consequences are greater than in women, with men having about twice the 1-year fatality rate after hip fracture, compared to women. Men at high risk for fracture include those men who have already had a fragility fracture, men on oral glucocorticoids or those men being treated for prostate cancer with androgen deprivation therapy. Beyond these high risk men, there are many other risk factors and secondary causes of osteoporosis in men. Evaluation includes careful history and physical examination to reveal potential secondary causes, including many medications, a short list of laboratory tests, and bone mineral density testing by dual energy X-ray absorptiometry (DXA) of spine and hip. Recently, international organizations have advocated a single normative database for interpreting DXA testing in men and women. The consequences of this change need to be determined. There are several choices of therapy for osteoporosis in men, with most fracture reduction estimation based on studies in women. PMID:26273515

Juvenile angiofibroma

MedlinePlus

Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor; Juvenile nasal angiofibroma; JNA ... Juvenile angiofibroma is not very common. It is most often found in adolescent boys. The tumor contains many blood ...

Comparison of monocyte gene expression among patients with neurocysticercosis-associated epilepsy, Idiopathic Epilepsy and idiopathic headaches in India.

PubMed

Prabhakaran, Vasudevan; Drevets, Douglas A; Ramajayam, Govindan; Manoj, Josephine J; Anderson, Michael P; Hanas, Jay S; Rajshekhar, Vedantam; Oommen, Anna; Carabin, Hélène

2017-06-01

Neurocysticercosis (NCC), a neglected tropical disease, inflicts substantial health and economic costs on people living in endemic areas such as India. Nevertheless, accurate diagnosis using brain imaging remains poorly accessible and too costly in endemic countries. The goal of this study was to test if blood monocyte gene expression could distinguish patients with NCC-associated epilepsy, from NCC-negative imaging lesion-free patients presenting with idiopathic epilepsy or idiopathic headaches. Patients aged 18 to 51 were recruited from the Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, India, between January 2013 and October 2014. mRNA from CD14+ blood monocytes was isolated from 76 patients with NCC, 10 Recovered NCC (RNCC), 29 idiopathic epilepsy and 17 idiopathic headaches patients. A preliminary microarray analysis was performed on six NCC, six idiopathic epilepsy and four idiopathic headaches patients to identify genes differentially expressed in NCC-associated epilepsy compared with other groups. This analysis identified 1411 upregulated and 733 downregulated genes in patients with NCC compared to Idiopathic Epilepsy. Fifteen genes up-regulated in NCC patients compared with other groups were selected based on possible relevance to NCC, and analyzed by qPCR in all patients' samples. Differential gene expression among patients was assessed using linear regression models. qPCR analysis of 15 selected genes showed generally higher gene expression among NCC patients, followed by RNCC, idiopathic headaches and Idiopathic Epilepsy. Gene expression was also generally higher among NCC patients with single cyst granulomas, followed by mixed lesions and single calcifications. Expression of certain genes in blood monocytes can distinguish patients with NCC-related epilepsy from patients with active Idiopathic Epilepsy and idiopathic headaches. These findings are significant because they may lead to the development of new tools to

Osteoporosis in survivors of early life starvation.

PubMed

Weisz, George M; Albury, William R

2013-01-01

The objective of this study was to provide evidence for the association of early life nutritional deprivation and adult osteoporosis, in order to suggest that a history of such deprivation may be an indicator of increased risk of osteoporosis in later life. The 'fetal programming' of a range of metabolic and cardiovascular disorders in adults was first proposed in the 1990s and more recently extended to disorders of bone metabolism. Localised famines during World War II left populations in whom the long-term effects of maternal, fetal and infantile nutritional deprivation were studied. These studies supported the original concept of 'fetal programming' but did not consider bone metabolism. The present paper offers clinical data from another cohort of World War II famine survivors - those from the Holocaust. The data presented here, specifically addressing the issue of osteoporosis, report on 11 Holocaust survivors in Australia (five females, six males) who were exposed to starvation in early life. The cases show, in addition to other metabolic disorders associated with early life starvation, various levels of osteoporosis, often with premature onset. The cohort studied is too small to support firm conclusions, but the evidence suggests that the risk of adult osteoporosis in both males and females is increased by severe starvation early in life - not just in the period from gestation to infancy but also in childhood and young adulthood. It is recommended that epidemiological research on this issue be undertaken, to assist planning for the future health needs of immigrants to Australia coming from famine affected backgrounds. Pending such research, it would be prudent for primary care health workers to be alert to the prima facie association between early life starvation and adult osteoporosis, and to take this factor into account along with other indicators when assessing a patient's risk of osteoporosis in later life.

Juveniles in court.

PubMed

Soulier, Matthew F; Scott, Charles L

2010-01-01

Nineteenth-century American reformers were concerned about the influence of immaturity and development in juvenile offenses. They responded to their delinquent youths through the creation of juvenile courts. This early American juvenile justice system sought to treat children as different from adults and to rehabilitate wayward youths through the state's assumption of a parental role. Although these rehabilitative goals were never fully realized, the field of American child psychiatry was spawned from these efforts on behalf of delinquent youths. Early child psychiatrists began by caring for juvenile offenders. The function of a child psychiatrist with juvenile delinquents expanded beyond strictly rehabilitation, however, as juvenile courts evolved to resemble criminal adult courts-due to landmark Supreme Court decisions and also juvenile legislation between 1966 and 1975. In response to dramatically increased juvenile violence and delinquency rates in the 1980s, juvenile justice became more retributional, and society was forced to confront issues such as capital punishment for juveniles, their transfer to adult courts, and their competency to stand trial. In the modern juvenile court, child psychiatrists are often asked to participate in the consideration of such issues because of their expertise in development. In that context we review the role of psychiatrists in assisting juvenile courts.

How should clinicians manage osteoporosis in ankylosing spondylitis?

PubMed

Bessant, Rupa; Keat, Andrew

2002-07-01

Osteoporosis is a common complication of AS, with an incidence between 18.7% and 62%. The prevalence of osteoporosis is greater in males, and increases with increasing patient age and disease duration. Osteoporosis is also more common in patients with syndesmophytes, cervical fusion, and peripheral joint involvement. These variables are not all independent, as they may be indicators of disease duration. Osteoporosis in patients with AS is largely confined to the axial skeleton, in contrast to the pattern of osteoporosis seen in rheumatoid arthritis. BMD at the lumbar spine and femoral neck may be severely reduced, while most studies indicate that carpal and radial BMD remain within normal limits. The development of syndesmophytes in late AS can lead to difficulties in the use of DEXA scanning to determine lumbar BMD, as the extraspinal bone may obscure osteoporotic vertebrae. Under these circumstances more accurate assessment of lumbar BMD, and one that correlates better with femoral neck BMD, may be obtained by quantitative CT scanning or DEXA scanning of the lateral aspect of the L3 vertebra. Osteoporosis in AS significantly increases the risk of vertebral compression fractures within 5 years of the diagnosis of AS. The risk of a vertebral compression fracture occurring over a 30 year period following the diagnosis of AS is 14%, compared to 3.4% for population controls. In patients with vertebral osteoporosis relatively minor trauma, such as slipping, can lead to spinal fracture and dislocatior with subsequent damage to the spinal cord. There is a higher incidence of spinal cord injury following spinal fracture dislocations in patients with AS, and the resulting neurological deficit can range from mild sensory loss to complete paraplegia. Cytokines such as TNF-alpha and IL-6 may play an important part in the pathogenesis of osteoporosis in early AS, and IL-6 levels have been correlated with markers of disease activity and severity. In late AS, mechanical factors

Clinical improvement of renal amyloidosis in a patient with systemic-onset juvenile idiopathic arthritis who received tocilizumab treatment: a case report and literature review.

PubMed

Chantarogh, Songkiat; Vilaiyuk, Soamarat; Tim-Aroon, Thipwimol; Worawichawong, Suchin

2017-05-12

Juvenile idiopathic arthritis (JIA) is a common rheumatic disease in children and adolescents. Although JIA may cause secondary amyloidosis, this is a rare complication in patients with JIA and other rheumatic diseases. Many previous studies have revealed that common heterozygous or homozygous mutations in the MEFV gene are associated with systemic-onset JIA (SJIA). We herein report a case involving a 19-year-old female patient with difficult-to-control SJIA. She developed progressive proteinuria without clinical signs or symptoms of edema. Renal amyloidosis was diagnosed by renal pathologic examination, which demonstrated deposition of eosinophilic amorphous material in the interlobular arteries, arterioles, and interstitium. Electron microscopy showed fibrillary material deposits with a diameter of 8 to 10 nm. A heterozygous E148Q mutation in the MEFV gene was identified. Conventional disease-modifying anti-rheumatic drugs and etanercept had been used to treat the SJIA, but the disease could not be controlled. Therefore, we decided to start tocilizumab to control the disease activity. However, the patient was unable to receive a standard dose of tocilizumab in the early period of treatment because of socioeconomic limitations. Her disease course was still active, and proteinuria was found. Therefore, tocilizumab was increased to a dose of 8 mg/kg every 2 weeks (standard dose of SJIA), and the patient exhibited a clinical response within 3 months. Refractory SJIA associated with renal amyloidosis is an uncommon cause of proteinuria in adolescents. Tocilizumab may be a beneficial treatment for renal amyloidosis in patients with SJIA.

Concern and risk perception of osteoporosis and fracture among post-menopausal Australian women: results from the Global Longitudinal Study of Osteoporosis in Women (GLOW) cohort.

PubMed

Barcenilla-Wong, A L; Chen, J S; March, L M

2013-01-01

The purpose of this study is to identify factors associated with concern and perception of risks of osteoporosis and osteoporotic fractures and determine whether bone mineral density (BMD) testing influenced concern and risk perception. Study subjects (n = 1,082, age 55-94 years) were female Australian participants of the Global Longitudinal Study of Osteoporosis in Women (GLOW). Self-administered questionnaires were sent annually from 2007 to 2010. Study outcomes included 'concern about osteoporosis', 'perception of getting osteoporosis' and 'perception of fracture risk' compared to similar aged women. The closest post-BMD testing or baseline questionnaires were used for women with and without BMD testing, respectively. Multinomial logistic regression was used for the analysis. BMD testing, prior fracture after age 45, younger age and lower self-reported general health were significantly associated with being 'very' or 'somewhat concerned' about osteoporosis and having a 'much higher' or 'little higher' risk perception of osteoporosis and fractures. A poorer BMD result was associated with higher concern and higher risk perceptions. The presence of comorbidities, having ≥2 falls in the preceding year and maternal osteoporosis were associated with higher concern. Maternal osteoporosis, presence of comorbidities, weight loss of ≥5 kg in the preceding year and low body mass index were associated with higher perceptions of osteoporosis risk. Women's concern and risk perception of osteoporosis and osteoporotic fractures were reasonably well founded. However, increasing age, height loss, smoking and drinking were not associated with concern and perception despite being known osteoporosis risk factors. These factors should be considered in planning for education and awareness raising programmes.

New Antiresorptive Therapies for Postmenopausal Osteoporosis

PubMed Central

2015-01-01

Osteoporosis is a systemic skeletal disease whose risk increases with age and it is common among postmenopausal women. Currently, almost all pharmacological agents for osteoporosis target the bone resorption component of bone remodeling activity. Current antiresorptive agents are effective, but the effectiveness of some agents is limited by real or perceived intolerance, longterm adverse events (AEs), coexisting comorbidities, and inadequate long-term adherence. New antiresorptive therapies that may expand options for the prevention and treatment of osteoporosis include denosumab, combination of conjugated estrogen/bazedoxifene and cathepsin K inhibitors. However, the long-term efficacy and AEs of these antiresorptive therapies need to be confirmed in studies with a longer follow-up period. PMID:26046031

Estrogen and Osteoporosis.

ERIC Educational Resources Information Center

Lindsay, Robert

1987-01-01

This article reviews the use of estrogen in the prevention and treatment of osteoporosis. Dosage levels, interactions with other factors, side effects, and the mechanism of estrogen action are discussed. (Author/MT)

Postmenopausal Osteoporosis: The Role of Immune System Cells

PubMed Central

Faienza, Maria Felicia; Ventura, Annamaria; Marzano, Flaviana; Cavallo, Luciano

2013-01-01

In the last years, new evidences of the relationship between immune system and bone have been accumulated both in animal models and in humans affected by bone disease, such as rheumatoid arthritis, bone metastasis, periodontitis, and osteoporosis. Osteoporosis is characterized by low bone mass and microarchitectural deterioration of bone tissue with a subsequent increase in bone fragility and susceptibility to fractures. The combined effects of estrogen deprivation and raising of FSH production occurring in menopause cause a marked stimulation of bone resorption and a rapid bone loss which is central for the onset of postmenopausal osteoporosis. This review focuses on the role of immune system in postmenopausal osteoporosis and on therapeutic strategies targeting osteoimmunology pathways. PMID:23762093

[Daily practice using the guidelines for prevention and treatment of osteoporosis. Effectiveness of exercise for preventing and treating osteoporosis].

PubMed

Miyakoshi, Naohisa

2008-08-01

There is increasing evidence that exercise is an effective strategy for the prevention and treatment of osteoporosis. The randomized controlled trials and their meta-analyses to date, evaluating the effects of exercise on osteoporosis reveal that the exercise is effective in preserving bone mass, preventing fractures and falls, and improving quality of life in patients with osteoporosis. Emphasis is also given to the importance of the specific protocols of exercises needed to achieve positive effects safely, keeping in view the age and general physical condition of the person.

What People with Rheumatoid Arthritis Need to Know about Osteoporosis

MedlinePlus

... People With Rheumatoid Arthritis Need to Know About Osteoporosis What Is Rheumatoid Arthritis? Rheumatoid arthritis is an ... Management Strategies Resources For Your Information What Is Osteoporosis? Osteoporosis is a condition in which the bones ...

What Are Osteoporosis and Arthritis and How Are They Different?

MedlinePlus

... Series of Publications for the Public What Are Osteoporosis and Arthritis and How Are They Different? Fast ... and Arthritis Cope? What About Pain? What Is Osteoporosis? Osteoporosis is a disease that makes bones weak ...

What People Recovering from Alcoholism Need to Know about Osteoporosis

MedlinePlus

... People Recovering from Alcoholism Need to Know About Osteoporosis Alcoholism and Recovery According to the National Institute ... Management Strategies Resources For Your Information What Is Osteoporosis? Osteoporosis is a condition in which bones become ...

What People with Celiac Disease Need to Know about Osteoporosis

MedlinePlus

... People With Celiac Disease Need to Know About Osteoporosis What Is Celiac Disease? Celiac disease, sometimes called ... Management Strategies Resources For Your Information What Is Osteoporosis? Osteoporosis is a condition in which the bones ...

EULAR-PReS points to consider for the use of imaging in the diagnosis and management of juvenile idiopathic arthritis in clinical practice.

PubMed

Colebatch-Bourn, A N; Edwards, C J; Collado, P; D'Agostino, M-A; Hemke, R; Jousse-Joulin, S; Maas, M; Martini, A; Naredo, E; Østergaard, M; Rooney, M; Tzaribachev, N; van Rossum, M A; Vojinovic, J; Conaghan, P G; Malattia, C

2015-11-01

To develop evidence based points to consider the use of imaging in the diagnosis and management of juvenile idiopathic arthritis (JIA) in clinical practice. The task force comprised a group of paediatric rheumatologists, rheumatologists experienced in imaging, radiologists, methodologists and patients from nine countries. Eleven questions on imaging in JIA were generated using a process of discussion and consensus. Research evidence was searched systematically for each question using MEDLINE, EMBASE and Cochrane CENTRAL. Imaging modalities included were conventional radiography, ultrasound, MRI, CT, scintigraphy and positron emission tomography. The experts used the evidence obtained from the relevant studies to develop a set of points to consider. The level of agreement with each point to consider was assessed using a numerical rating scale. A total of 13 277 references were identified from the search process, from which 204 studies were included in the systematic review. Nine points to consider were produced, taking into account the heterogeneity of JIA, the lack of normative data and consequent difficulty identifying pathology. These encompassed the role of imaging in making a diagnosis of JIA, detecting and monitoring inflammation and damage, predicting outcome and response to treatment, use of guided therapies, progression and remission. Level of agreement for each proposition varied according to the research evidence and expert opinion. Nine points to consider and a related research agenda for the role of imaging in the management of JIA were developed using published evidence and expert opinion. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Methotrexate treatment may prevent uveitis onset in patients with juvenile idiopathic arthritis: experiences and subgroup analysis in a cohort with frequent methotrexate use.

PubMed

Kostik, Mikhail M; Gaidar, Ekaterina V; Hynnes, Alla Y; Dubko, Margarita F; Masalova, Vera V; Snegireva, Ludmil S; Chikova, Irina A; Isupova, Eugenia A; Nikitina, Tatiana N; Serogodskaya, Elena D; Kalashnikova, Olga V; Ravelli, Angelo; Chasnyk, Vyacheslav G

2016-01-01

To re-evaluate the ability of methotrexate (MTX) to prevent the onset of uveitis in Russian children with juvenile idiopathic arthritis (JIA). The clinical charts for all consecutive patients who received a stable management for at least 2 years with or without MTX were reviewed. Patients who were given systemic medications other than MTX (except NSAID) and patients with systemic arthritis, rheumatoid factor-positive arthritis, or enthesitis-related arthritis were excluded. Each patient was examined after at least a 2-year follow-up period after the first visit to establish whether uveitis had occurred. A total of 281 patients with a median disease duration of 3.8 years were included. 191 patients (68%) were treated with MTX. During the observation period, 64 patients (22.8%) developed uveitis, a median of 1.6 year after disease onset. The frequency of uveitis was lower in MTX-treated than in MTX-untreated patients (11.5% vs. 46.7%, respectively, OR=6.7 (95%CI:3.7-12.3), p=0.0000001). Survival analysis confirmed that patients treated with MTX had a lower probability of developing uveitis (HR=4.35, p=0.000001). In subgroup analysis it was shown that MTX was more preventive in boys than in girls, and in patients with JIA onset age of over 5 years compared to those with disease onset less than 5 years. The data of survival analysis of MTX prevention has shown that benefits do not depend on the number of active joints and ANA status. MTX therapy may prevent the onset of uveitis in children with JIA. Further randomised controlled trials are required to confirm our results.

Medicines for osteoporosis

MedlinePlus

... Evista); Teriparatide (Forteo); Denosumab (Prolia); Low bone density - medicines; Osteoporosis - medicines ... Your doctor may prescribe certain medicines to help lower your ... make the bones in your hips, spine, and other areas less likely ...

Increased risk of osteoporosis in patients with erectile dysfunction

PubMed Central

Wu, Chieh-Hsin; Lu, Ying-Yi; Chai, Chee-Yin; Su, Yu-Feng; Tsai, Tai-Hsin; Tsai, Feng-Ji; Lin, Chih-Lung

2016-01-01

Abstract In this study, we aimed to investigate the risk of osteoporosis in patients with erectile dysfunction (ED) by analyzing data from the Taiwan National Health Insurance Research Database (NHIRD). From the Taiwan NHIRD, we analyzed data on 4460 patients aged ≥40 years diagnosed with ED between 1996 and 2010. In total, 17,480 age-matched patients without ED in a 1:4 ratio were randomly selected as the non-ED group. The relationship between ED and the risk of osteoporosis was estimated using Cox proportional hazard regression models. During the follow-up period, 264 patients with ED (5.92%) and 651 patients without ED (3.65%) developed osteoporosis. The overall incidence of osteoporosis was 3.04-fold higher in the ED group than in the non-ED group (9.74 vs 2.47 per 1000 person-years) after controlling for covariates. Compared with patients without ED, patients with psychogenic and organic ED were 3.19- and 3.03-fold more likely to develop osteoporosis. Our results indicate that patients with a history of ED, particularly younger men, had a high risk of osteoporosis. Patients with ED should be examined for bone mineral density, and men with osteoporosis should be evaluated for ED. PMID:27368024

The family journey-to-diagnosis with systemic juvenile idiopathic arthritis: a cross-sectional study of the changing social media presence.

PubMed

Modica, Renee F; Lomax, Kathleen Graham; Batzel, Pamela; Shapardanis, Leah; Katzer, Kimberly Compton; Elder, Melissa E

2016-01-01

Children with systemic juvenile idiopathic arthritis (SJIA) often encounter a delay between symptom onset and disease diagnosis, partly due to the broad differential of fever and lack of symptom recognition by providers. Families often seek multiple medical opinions and post on social media about their frustrations. This linguistic analysis observed the changing language patterns and social media posting behaviors of parents in the time leading to, during, and after SJIA diagnosis. Public social media sites were manually reviewed by a linguistic team to evaluate posts about SJIA from US-based parents. A total of 3,979 posts between July 2001 and January 2015 were reviewed from 108 sites. Pre-SJIA diagnosis parents sought answers and shared status updates on social media, focusing primarily on the following three site types: alternative/natural lifestyle forums (39%), Facebook (27%), and disease-specific forums (17%). Posts during early prediagnosis phases were characterized by expressive language showing confidence in health care providers and trust in parental instincts. At later prediagnosis stages, parents continued to use social media, but the posts demonstrated increased frustration with delays in diagnosis and gaps in communication with providers. More objective symptom descriptions and a greatly reduced child-centered emotional focus were observed as parents shifted into caregiving roles. Once the diagnosis of SJIA was confirmed, parents used straightforward, less expressive language, and Facebook (47%) to make "announcement" posts and increased their use of SJIA websites (30%). With treatment initiation, the posts demonstrated a slow return of expressive language and an increased parental understanding of the "new normal". Parents use different language styles, frames of reference, and websites before and after SJIA diagnosis. Gaps in parent-provider communication, especially before diagnosis, and their new roles as caregivers lead to parental use of social

IL1RN Variation Influences both Disease Susceptibility and Response to Human Recombinant IL-1RA Therapy in Systemic Juvenile Idiopathic Arthritis.

PubMed

Arthur, Victoria L; Shuldiner, Emily; Remmers, Elaine F; Hinks, Anne; Grom, Alexei A; Foell, Dirk; Martini, Alberto; Gattorno, Marco; Özen, Seza; Prahalad, Sampath; Zeft, Andrew S; Bohnsack, John F; Ilowite, Norman T; Mellins, Elizabeth D; Russo, Ricardo; Len, Claudio; Oliveira, Sheila; Yeung, Rae S M; Rosenberg, Alan M; Wedderburn, Lucy R; Anton, Jordi; Haas, Johannes-Peter; Rösen-Wolff, Angela; Minden, Kirsten; Szymanski, Ann Marie; Thomson, Wendy; Kastner, Daniel L; Woo, Patricia; Ombrello, Michael J

2018-04-02

To determine whether systemic juvenile idiopathic arthritis (sJIA) susceptibility loci identified by candidate gene studies demonstrated association with sJIA in the largest study population assembled to date. Single nucleotide polymorphisms (SNPs) from 11 previously reported sJIA risk loci were examined for association in 9 populations, including 770 sJIA cases and 6947 control subjects. The effect of sJIA-associated SNPs on gene expression was evaluated in silico in paired whole genome and RNA sequencing data from lymphoblastoid cell lines (LCL) of 373 European 1000 Genomes Project subjects. The relationship between sJIA-associated SNPs and response to anakinra treatment was evaluated in 38 US patients for whom treatment response data were available. We found no association of the 26 SNPs previously reported as sJIA-associated. Expanded analysis of the regions containing the 26 SNPs revealed only one significant association, the promoter region of IL1RN (p<1E-4). sJIA-associated SNPs correlated with IL1RN expression in LCLs, with an inverse correlation between sJIA risk and IL1RN expression. The presence of homozygous IL1RN high expression alleles correlated strongly with non-response to anakinra therapy (OR 28.7 [3.2, 255.8]). IL1RN was the only candidate locus associated with sJIA in our study. The implicated SNPs are among the strongest known determinants of IL1RN and IL1RA levels, linking low expression with increased sJIA risk. Homozygous high expression alleles predicted non-response to anakinra therapy, nominating them as candidate biomarkers to guide sJIA treatment. This is an important first step towards the personalized treatment of sJIA. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Is Intra-Articular Steroid Injection to the Temporomandibular Joint for Juvenile Idiopathic Arthritis More Effective and Efficient When Performed With Image Guidance?

PubMed

Resnick, Cory M; Vakilian, Pouya M; Kaban, Leonard B; Peacock, Zachary S

2017-04-01

To compare short-term outcomes and procedure times for intra-articular steroid injection (IASI) to the temporomandibular joint (TMJ) with and without the use of intraoperative image guidance for patients with juvenile idiopathic arthritis (JIA). This is a retrospective study of children with JIA who underwent TMJ IASI at Boston Children's Hospital (Boston, MA). Patients were divided into groups according to IASI technique: 1) "landmark" group if performed by an oral and maxillofacial surgeon using an anatomic landmark technique with no intraoperative image guidance or 2) "image-guided" group if performed by an interventional radiologist using intraoperative ultrasound and computed tomography. Predictor variables included IASI technique (landmark vs image guided), age, gender, JIA subtype, category of medications for arthritis, and presence of family history of autoimmune disease. Outcome variables were changes in patient-reported pain, maximal incisal opening (MIO), synovial enhancement ratio (ER), and total procedure time. Forty-five patients with 71 injected TMJs were included. Twenty-two patients with 36 injected TMJs were in the landmark group and 23 patients with 35 injected joints were in the image-guided group. There were no relevant differences in age, gender, family history of rheumatologic disease, or disease subtype between groups. There were no differences in resolution of pain (P = 1.00), increase in MIO (P = .975), or decrease in ER (P = .492) between groups, but procedure times averaged 49 minutes longer for the image-guided group (P < .008). There were no statistical differences in short-term outcomes, but procedure times were longer for the image-guided group. Although specific indications for the use of image guidance might exist, routine use of this procedure cannot be justified. Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Benefits, Harms, and Costs of Osteoporosis Screening in Male Veterans

DTIC Science & Technology

2014-10-01

over age 50 years have osteopenia or osteoporosis, and nearly 12% of those over age 75 years have osteoporosis, a rate nearly double the non...give reasons) (n= ) Lost to follow-up (give reasons) (n= ) Received DXA screening (n= ) Osteoporosis (n= )  Osteopenia (n= ) Neither (n...Lost to follow-up (give reasons) (n= ) No DXA screening (n= ) Osteoporosis (n= )  Osteopenia (n= ) Neither (n= ) Analysed (n

Evaluation of compliance with osteoporosis treatment guidelines after initiation of a pharmacist-run osteoporosis service at a family medicine clinic.

PubMed

Hall, Larissa N; Shrader, Sarah P; Ragucci, Kelly R

2009-11-01

Osteoporosis affects more than 10 million Americans, and fracture complications are devastating to patients and society. Despite the availability of guidelines and performance measures, osteoporosis is not optimally managed. Pharmacists have been pivotal in management of other disease states, and a multidisciplinary approach to osteoporosis management may improve patient outcomes. To establish a pharmacist-run osteoporosis service at a family medicine clinic and to evaluate short-term compliance with osteoporosis treatment guidelines before and after initiation of the service. A pharmacist-run osteoporosis service was established in October 2008. Adults with the diagnosis of osteoporosis before initiation of the service were included in evaluation of short-term compliance with treatment guidelines, including appropriate dual-energy X-ray absorptiometry (DEXA) scan frequency, pharmacotherapy, calcium and vitamin D supplementation, and nonpharmacologic education. Of 42 referred patients, 22 were eligible for inclusion. A retrospective chart review was conducted, and patients served as their own controls, with data from before and after establishment of the service evaluated. Of the 22 patients evaluated, 8 (36%) received DEXA scans at the appropriate frequency before the service was established, versus 18 (82%) after the service was initiated. Seven (32%) patients were taking appropriate pharmacotherapy before the service, versus 17 (77%) after the service. Nine (41%) patients were taking calcium and vitamin D before the service, versus 22 (100%) after the service. Three (33%) of these patients were taking the appropriate dose and salt of calcium before the service, versus 20 (91%) after the service. Five (56%) of the 9 patients were taking the appropriate vitamin D dose before the service, versus 21 (95%) after the service. No patient had documented nonpharmacologic education prior to the service, compared with all patients after the service. All differences were

Osteoporosis in men: epidemiology and treatment with denosumab

PubMed Central

Sidlauskas, Kristel M; Sutton, Emily E; Biddle, Michael A

2014-01-01

Osteoporosis is a major public health care concern. Although often described as a disease affecting postmenopausal women, researchers and clinicians have emphasized its prevalence in men in recent years. The National Osteoporosis Foundation has stated that up to 25% of men over the age of 50 years will experience a fracture due to osteoporosis. Men who suffer from a major fracture have higher mortality rates than women. Pharmacologic therapy options for treating osteoporosis are limited for men as compared with women, so each medication approved for use in this population represents an important clinical option. In September 2012, the US Food and Drug Administration approved a new indication for denosumab to increase bone mass in men with osteoporosis at high risk for fracture. Denosumab is a fully human monoclonal antibody and novel antiresorptive agent that works by binding receptor activator of nuclear factor kappa-β ligand (RANKL) and inhibiting the signaling cascade that causes osteoclast maturation, activity, and survival. Ultimately, denosumab suppresses bone turnover and increases bone mineral density in both trabecular and cortical bone. Approval for treating osteoporosis in men was based on data from the ADAMO trial which displayed efficacy in increasing bone mineral density at the lumbar spine, total hip, femoral neck, hip trochanter, and one-third radius. Studies indicate that denosumab is effective and safe, and has superior adherence rates and patient satisfaction. Although long-term data and further research on fracture reduction rates in men should be explored, at this time denosumab is one of several appropriate first-line treatment options for men with osteoporosis. PMID:24748777

Osteoporosis in men: epidemiology and treatment with denosumab.

PubMed

Sidlauskas, Kristel M; Sutton, Emily E; Biddle, Michael A

2014-01-01

Osteoporosis is a major public health care concern. Although often described as a disease affecting postmenopausal women, researchers and clinicians have emphasized its prevalence in men in recent years. The National Osteoporosis Foundation has stated that up to 25% of men over the age of 50 years will experience a fracture due to osteoporosis. Men who suffer from a major fracture have higher mortality rates than women. Pharmacologic therapy options for treating osteoporosis are limited for men as compared with women, so each medication approved for use in this population represents an important clinical option. In September 2012, the US Food and Drug Administration approved a new indication for denosumab to increase bone mass in men with osteoporosis at high risk for fracture. Denosumab is a fully human monoclonal antibody and novel antiresorptive agent that works by binding receptor activator of nuclear factor kappa-β ligand (RANKL) and inhibiting the signaling cascade that causes osteoclast maturation, activity, and survival. Ultimately, denosumab suppresses bone turnover and increases bone mineral density in both trabecular and cortical bone. Approval for treating osteoporosis in men was based on data from the ADAMO trial which displayed efficacy in increasing bone mineral density at the lumbar spine, total hip, femoral neck, hip trochanter, and one-third radius. Studies indicate that denosumab is effective and safe, and has superior adherence rates and patient satisfaction. Although long-term data and further research on fracture reduction rates in men should be explored, at this time denosumab is one of several appropriate first-line treatment options for men with osteoporosis.

Genetic and environmental factors in human osteoporosis.

PubMed

Özbaş, Halil; Tutgun Onrat, Serap; Özdamar, Kazım

2012-12-01

Osteoporosis is a common disorder, with prolongation of the average life span it has become a major public health problem. On the formation of osteoporosis genetic factors and environmental influences could play a role then it is considered as multi-factorial. Because a variety of functions to affect susceptibility to the formation of osteoporosis VDR-F, VDR-B, COL1A1, ESR1X, ESR1P and CTR are thought to be candidate genes. In this study, the aim is to investigate the relationship between these genes polymorphism and bone mineral density (BMD) values of lumbar vertebra and femoral neck in 188 Turkish people. Lumbar spine and femoral neck BMD of the individuals included in the study were measured by the dual X-ray absorptiometry method. The genotyped polymorphisms by simultaneous amplification of five regions of the genome, containing six SNPs of interest and detecting the amplified product, using the kit MetaBone Clinical Arrays(®). Statistical analyses indicated that; VDR-B gene polymorphisms major (P = 0.013), VDR-F polymorphisms have minor (P = 0.082) effect on femur BMD. None of the other genes has any significant effect on spinal BMD. Patient age, body mass index and diet has significant effect on femoral and spinal BMD. Osteoporosis is a multi-factorial disease and many genetic and non-genetic risk factors contribute to the development of osteoporosis. Early detection of a genetic predisposition to osteoporosis should allow delay and/or limit unfavorable changes in the bone tissue.

Regulatory element-based prediction identifies new susceptibility regulatory variants for osteoporosis.

PubMed

Yao, Shi; Guo, Yan; Dong, Shan-Shan; Hao, Ruo-Han; Chen, Xiao-Feng; Chen, Yi-Xiao; Chen, Jia-Bin; Tian, Qing; Deng, Hong-Wen; Yang, Tie-Lin

2017-08-01

Despite genome-wide association studies (GWASs) have identified many susceptibility genes for osteoporosis, it still leaves a large part of missing heritability to be discovered. Integrating regulatory information and GWASs could offer new insights into the biological link between the susceptibility SNPs and osteoporosis. We generated five machine learning classifiers with osteoporosis-associated variants and regulatory features data. We gained the optimal classifier and predicted genome-wide SNPs to discover susceptibility regulatory variants. We further utilized Genetic Factors for Osteoporosis Consortium (GEFOS) and three in-house GWASs samples to validate the associations for predicted positive SNPs. The random forest classifier performed best among all machine learning methods with the F1 score of 0.8871. Using the optimized model, we predicted 37,584 candidate SNPs for osteoporosis. According to the meta-analysis results, a list of regulatory variants was significantly associated with osteoporosis after multiple testing corrections and contributed to the expression of known osteoporosis-associated protein-coding genes. In summary, combining GWASs and regulatory elements through machine learning could provide additional information for understanding the mechanism of osteoporosis. The regulatory variants we predicted will provide novel targets for etiology research and treatment of osteoporosis.

Osteoporosis in older persons: current pharmacotherapy and future directions.

PubMed

Duque, Gustavo

2013-10-01

Osteopororic fractures are highly prevalent in older persons having catastrophic consequences in their quality of life and increasing disability and mortality in this population. The mechanisms of osteoporosis in older persons are unique in terms of cellular changes and response to osteoporosis treatment. Therefore, specifically targeted treatments are required in this particular population. This paper provides an overview on the particular mechanisms of osteoporosis in older persons and the current and future therapeutic strategies to improve bone mass and prevent fractures in this population. Osteoporosis in older persons (especially in the old-old) has a unique pathophysiology that predisposes them to fractures thus having catastrophic consequences. Identification of patients at risk followed by therapies targeted to their cellular changes is pivotal to close the care gap observed in osteoporosis, predominantly in the older population. The treatment of osteoporosis has evolved from daily to yearly dosing thus facilitating compliance and effectiveness. It is expected that future biologically targeted treatments will have a similar separate dosing regime with better anti-fracture efficacy and lower incidence of side effects.

Osteoporosis screening is unjustifiably low in older African-American women.

PubMed Central

Wilkins, Consuelo H.; Goldfeder, Jason S.

2004-01-01

BACKGROUND: More than one million Americans suffer osteoporotic fractures yearly, resulting in a marked increase in morbidity and mortality. Despite a decrease in bone mineral density with increasing age in all ethnic groups and both genders, preventative and therapeutics efforts in osteoporosis have been focused on caucasian and Asian women. This study assesses the osteoporosis screening practices and the frequency of low bone density in a primarily African-American population of older women. METHODS: Medical records of 252 women at risk for osteoporosis were reviewed for the diagnosis of osteoporosis, prior osteoporosis screening, prior breast cancer screening, and the use of calcium, vitamin D or estrogen. Subsequently, 128 women were assessed for risk factors for osteoporosis, and their bone mineral density was measured using a peripheral bone densitometer. RESULTS: Osteoporosis screening had been performed in 11.5% of the subjects. Of the women evaluated by peripheral bone densitometry, 44.5% of all women, 40.4% of African-American women, and 53.3% of caucasian women had abnormally low bone density measurements. The frequency of abnormal bone density increased with both increasing age and decreasing body mass index. CONCLUSIONS: Although few women in this population were previously screened for osteoporosis, low bone density occurred in African-American women at substantial rates. Increasing age and low body mass are important risk factors for low bone density in African-American women. Ethnicity should not be used as an exclusion criterion for screening for osteoporosis. PMID:15101666

Management of postmenopausal osteoporosis for primary care.

PubMed

Miller, P; Lukert, B; Broy, S; Civitelli, R; Fleischmann, R; Gagel, R; Khosla, S; Lucas, M; Maricic, M; Pacifici, R; Recker, R; Sarran, H S; Short, B; Short, M J

1998-01-01

The shift in health care delivery from a subspecialty to primary care system has transferred the responsibility of preventing osteoporotic fractures from specialists in metabolic bone disease to the web of physicians--family practitioners, general internists, pediatricians, and gynecologists--who provide the bulk of primary care. The challenge for this group of physicians is to decrease the rising prevalence of osteoporotic hip and vertebral fractures while operating within the cost parameters. It is the goal of this brief summary to provide primary practitioners with focused guidelines for the management of postmenopausal osteoporosis based on new and exciting developments. Prevention and treatment will change rapidly over the next decade and these advances will require changes in these recommendations. We identified patients at risk for osteoporosis and provided indications for bone mass measurement, criteria for diagnosis of osteoporosis, therapeutic interventions, and biochemical markers of the disease. Prevention and treatment are discussed, including hormone replacement therapy and use of calcitonin, sodium fluoride, bisphosphonates, and serum estrogen receptor modulators. Postmenopausal osteoporosis should no longer be an accepted process of aging. It is both preventable and treatable. Primary care physicians must proactively prevent and treat osteoporosis in their daily practice, and combination therapies are suggested.

Prevalence of osteoporosis in men aged 65-75 in a primary care setting. A practice audit after application of the Canadian 2010 guidelines for osteoporosis screening.

PubMed

Ferrari, Robert

2015-03-01

Current Canadian osteoporosis guidelines recommend routine bone density screening of men at age 65. The purpose of this study is to determine the prevalence of osteoporosis in men aged 65-75 in after application of screening guidelines. All males aged 65-75 years who attended a large primary care clinic were advised of the 2010 Canadian osteoporosis guidelines and advised to obtain a bone density scan at or after their 65th birthday. Those who did not have a bone density scan since their 65th birthday were advised to obtain a scan, unless there was obvious reason not to do so (i.e. known osteoporosis). A record of the results for each patient were kept and tallied to determine the prevalence of osteoporosis. Osteoporosis was defined as a T-score of ≤ -2.5 in either the hip or lumbar spine. Of 574 male subjects in this clinic, between the ages of 65-75, 557 had a bone density scan, either already having done so at the time of being informed of the guidelines or obtaining a scan in the subsequent year after being informed of the guidelines. The prevalence of osteoporosis was 1.6% (9/557, 95% confidence interval 0.8-3.1%) in this sample. The average age of subjects with osteoporosis was 70.5 ± 1.4 years (range 68-75). None of the subjects under 68 years of age were found to have osteoporosis. The prevalence of osteoporosis in unselected male cohorts aged 65 may be too low to justify the routine bone density screening recommended in the 2010 Canadian osteoporosis guidelines.

Management of postmenopausal osteoporosis and the prevention of fractures.

PubMed

Gambacciani, M; Levancini, M

2014-06-01

Postmenopausal osteoporosis affects millions of women, being estrogen deficiency the key factor in the pathogenesis of involutional osteoporosis. Fracture prevention is one of the public health priorities worldwide. Different treatments for osteoporosis are available. The various options are aimed to maintain bone health and decrease the risk of fractures. The majority of these drugs are antiresorptive agents, i.e., drugs that lower bone turnover, inhibiting osteoclastic bone resorption. Dietary sources of calcium intake and vitamin D are ideal, while pharmachological supplements should be used if diet alone cannot provide the recommended daily intake. Bisphosphonates are first-line therapy for patients with established osteoporosis at high risk of fracture. Some serious, but rare, adverse events have been associated with their long-term administration. The monoclonal antibody to RANKL, named denosumab, administered as a 60-mg subcutaneous injection every 6 months, is a valuable option for the treatment of postmenopausal osteoporosis in women at increased or high risk of fractures, who are unable to take other osteoporosis treatments. Teriparatide (PTH 1-34) is the only available osteoanabolic drugs for osteoporosis treatment at present. Its use is limited to severe osteoporosis because of the high cost of the treatment. In climacteric women, in different stages of menopausal transition, and beyond, hormone replacement therapy at different doses (HRT) rapidly normalizes turnover, preventing and/or treating osteoporosis. HRT is able to preserve and even increase BMD at all skeletal sites, leading to a significant reduction in vertebral and non-vertebral fractures. Selective estrogen modulators (SERMs) as raloxifene and bazedoxifene reduce bone turnover and maintains or increases vertebral and femoral BMDs in comparison to placebo and reduces the risk of vertebral and new vertebral fractures, in high risk women. The combination of a SERM with an estrogen has been

Linkage analysis of idiopathic generalized epilepsy (IGE) and marker loci on chromosome 6p in families of patients with juvenile myocloni epilepsy: No evidence for an epilepsy locus in the HLA region

DOE Office of Scientific and Technical Information (OSTI.GOV)

Whitehouse, W.P.; Rees, M.; Curtis, D.

1993-09-01

Evidence for a locus (EJM1) in the HLA region of chromosome 6p predisposing to idiopathic generalized epilepsy (IGE) in the families of patients with juvenile myoclonic epilepsy (JME) has been obtained in two previous studies of separately ascertained groups of kindreds. Linkage analysis has been undertaken in a third set of 25 families including a patient with JME and at least one first-degree relative with IGE. Family members were typed for eight polymorphic loci on chromosome 6p: F13A, D6889, D6S109, D6S105, D6S10, C4B, DQA1/A2, and TCTE1. Pairwise and multipoint linkage analysis was carried out assuming autosomal dominant and autosomal recessivemore » inheritance and age-dependent high or low penetrance. No significant evidence in favor of linkage was obtained at any locus. Multipoint linkage analysis generated significant exclusion data (lod score < -2.0) at HLA and for a region 10-30 cM telomeric to HLA, the extent of which varied with the level of penetrance assumed. These observations indicate that genetic heterogeneity exists within this epilepsy phenotype. 39 refs., 4 figs., 2 tabs.« less

Associated Factors of Bone Mineral Density and Osteoporosis in Elderly Males

PubMed Central

Heidari, Behzad; Muhammadi, Abdollah; Javadian, Yahya; Bijani, Ali; Hosseini, Reza; Babaei, Mansour

2016-01-01

Background Low bone mineral density and osteoporosis is prevalent in elderly subjects. This study aimed to determine the associated factors of bone mineral density and osteoporosis in elderly males. Methods All participants of the Amirkola health and ageing project cohort aged 60 years and older entered the study. Bone mineral density at femoral neck and lumbar spine was assessed by the dual energy X-ray absorptiometry (DXA) method. Osteoporosis was diagnosed by the international society for clinical densitometry criteria and the association of bone mineral density and osteoporosis with several clinical, demographic and biochemical parameters. Multiple logistic regression analysis was used to determine independent associations. Results A total of 553 patients were studied and 90 patients (16.2%) had osteoporosis at either femoral neck or lumbar spine. Diabetes, obesity, metabolic syndrome, overweight, and quadriceps muscle strength > 30 kg, metabolic syndrome, abdominal obesity and education level were associated with higher bone mineral density and lower prevalence of osteoporosis, whereas age, anemia, inhaled corticosteroids and fracture history were associated with lower bone mineral density and higher prevalence of osteoporosis (P = 0.001). After adjustment for all covariates, osteoporosis was negatively associated only with diabetes, obesity, overweight, and QMS > 30 kg and positively associated with anemia and fracture history. The association of osteoporosis with other parameters did not reach a statistical level. Conclusions The findings of the study indicate that in elderly males, diabetes, obesity and higher muscle strength was associated with lower prevalence of osteoporosis and anemia, and prior fracture with higher risk of osteoporosis. This issue needs further longitudinal studies. PMID:28835759

Prolonged breast-feeding is an independent risk factor for postmenopausal osteoporosis.

PubMed

Okyay, Duygu Ozkale; Okyay, Emre; Dogan, Erbil; Kurtulmus, Secil; Acet, Ferruh; Taner, Cuneyt Eftal

2013-03-01

This study investigated the effects of parity and age at first pregnancy and breast-feeding, as well as duration of BF for total and per child on postmenopausal osteoporosis. The study was conducted among 542 cases who were divided based on the presence or absence of osteoporosis. Patients were separated according to their first pregnancy and breast-feeding age as before or after 27 years. Osteoporosis was defined as a T score of -2.5 or lower. Parity, age at first pregnancy and breast-feeding, breast-feeding period for total and average duration per child according to a questionnaire were assessed. Osteoporosis group had significantly lower parity compared to non-osteoporosis group. The age at first pregnancy and breast-feeding<27 age were significantly more frequent in osteoporosis group. They also had prolonged breast-feeding period. Women who had a breast-feeding period per child>1 year under age 27 was higher in osteoporosis group. In multivariate analysis, women who breast-fed>1 year per child had the highest risk for osteoporosis (odds ratio: 12.92; 95% confidence interval, 3.1-52.6) and osteoporosis risk for women who breast-fed>1 year per child under age 27 was 7.1. Increased parity was associated with a significant protective effect for osteoporosis. Extended breast-feeding period per child>1 year is the highest risk factor for osteoporosis independent of first breast-feeding age. However, high parity has a protective effect. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Family history and its association to curve size and treatment in 1,463 patients with idiopathic scoliosis.

PubMed

Grauers, Anna; Danielsson, Aina; Karlsson, Magnus; Ohlin, Acke; Gerdhem, Paul

2013-11-01

To study family history in relation to curve severity, gender, age at diagnosis and treatment in idiopathic scoliosis. A self-assessment questionnaire on family history of scoliosis was administered to 1,463 untreated, brace or surgically treated idiopathic scoliosis patients. Out of the 1,463 patients, 51 % had one or more relatives with scoliosis. There was no significant difference between females and males, nor between juvenile and adolescent study participants in this respect (p = 0.939 and 0.110, respectively). There was a significant difference in maximum curve size between patients with one or more relatives with scoliosis (median 35°, interquartile range 25) and patients without any relative with scoliosis (median 32°, interquartile range 23) (p = 0.022). When stratifying patients according to treatment (observation, brace treatment or surgery), we found that it was more common to have a relative with scoliosis among the treated patients (p = 0.011). The OR for being treated was 1.32 (95% CI 1.06-1.64) when the patient had a relative with scoliosis, compared to not having. Larger curve sizes were found in patients with a family history of scoliosis than in the ones without. No relation between family history and gender or between family history and age at onset of idiopathic scoliosis was found. Although the presence of a family history of scoliosis may not be a strong prognostic risk factor, it indicates that these patients are at higher risk of developing a more severe curve.

Anti-NT5C1A autoantibodies are associated with more severe disease in patients with juvenile myositis.

PubMed

Yeker, Richard M; Pinal-Fernandez, Iago; Kishi, Takayuki; Pak, Katherine; Targoff, Ira N; Miller, Frederick W; Rider, Lisa G; Mammen, Andrew L

2018-05-01

Autoantibodies recognising cytosolic 5'-nucleotidase 1A (NT5C1A) are found in adult patients with myositis and other autoimmune diseases. They are especially prevalent in adults with inclusion body myositis (IBM), in which they are associated with more severe weakness and higher mortality. This study was undertaken to define the prevalence and clinical features associated with anti-NT5C1A autoantibodies in juvenile myositis. We screened sera from 380 patients with juvenile myositis, 30 patients with juvenile idiopathic arthritis (JIA) and 92 healthy control children for anti-NT5C1A autoantibodies. Clinical characteristics were compared between patients with myositis with and without anti-NT5C1A autoantibodies. Anti-NT5C1A autoantibodies were present in 102 of 380 (27%) patients with juvenile myositis and in 11 of 92 (12%) healthy control children (P=0.002) and 27% of children with JIA (P=0.05 vs controls). Sera of 83 of 307 (27%) patients with juvenile dermatomyositis and 16 of 46 (35%) patients with juvenile overlap myositis were anti-NT5C1A autoantibody-positive (P<0.01 vs healthy controls for each), but sera of only 3 of 27 (11%) patients with juvenile polymyositis were anti-NT5C1A-positive. Patients with juvenile myositis with and without anti-NT5C1A autoantibodies had similar clinical phenotypes. However, patients with anti-NT5C1A autoantibody-positive myositis had greater pulmonary symptoms at diagnosis (P=0.005), more frequent hospitalisations (P=0.01) and required a larger number of medications (P<0.001). Anti-NT5C1A autoantibodies are present in more than one-quarter of children with juvenile myositis and JIA compared with only 12% of healthy children, suggesting they are myositis-associated in children. As in adults with IBM, patients with juvenile myositis with anti-NT5C1A autoantibodies have more severe disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use

Association between tea consumption and osteoporosis: A meta-analysis.

PubMed

Sun, Kang; Wang, Le; Ma, Qingping; Cui, Qiaoyun; Lv, Qianru; Zhang, Wenzheng; Li, Xinghui

2017-12-01

Previous reports have suggested a potential association of tea consumption with the risk of osteoporosis. As such association is controversial, we conducted a meta-analysis to assess the relationship between tea consumption and osteoporosis. We systematically searched PubMed, EMBASE and WanFang databases until March 30, 2016, using the keywords "tea and osteoporosis," without limits of language. Odds ratios (ORs) with 95% confidence intervals (95% CIs) were derived by using random-effects models throughout the analyses. We conducted the analysis of the statistical heterogeneity using Cochrane I. The funnel plot was used to speculate the publication bias, while the subgroup analysis and multiround elimination method were employed. Our study was based on 17 journal articles, including 2 prospective cohort studies, 4 case-control studies, and 11 cross-sectional studies. In the present study, the total OR of osteoporosis for the highest versus the lowest categories of tea consumption was 0.62 (95% CI, 0.46-0.83), with significant heterogeneity among studies (I = 94%, P < .01). There was, however, no publication bias of the meta-analysis about tea consumption and osteoporosis. Subgroup analysis showed that tea consumption could reduce the risk of osteoporosis in all examined subgroups. In the present study, it can be concluded from the results that tea consumption can reduce the risk of osteoporosis.

Pathogenesis of systemic inflammatory diseases in childhood: "Lessons from clinical trials of anti-cytokine monoclonal antibodies for Kawasaki disease, systemic onset juvenile idiopathic arthritis, and cryopyrin-associated periodic fever syndrome".

PubMed

Yokota, Shumpei; Kikuchi, Masako; Nozawa, Tomo; Kanetaka, Taichi; Sato, Tomomi; Yamazaki, Kazuko; Sakurai, Nodoka; Hara, Ryoki; Mori, Masaaki

2015-01-01

Inflammation has often been considered to be a nonspecific response and to play a bridging role in the activation of adaptive immunity. However, it is now accepted that inflammation is the product of an independent innate immune system closely linked to the adaptive immune system. The key mediators of inflammation are inflammatory cytokines, as determined by multiple lines of evidence both in vitro and in vivo. Due to the crucial role of inflammatory cytokines in the pathogenesis of autoimmune disorders, anti-cytokine treatment has been developed as a therapy for rheumatoid arthritis, juvenile idiopathic arthritis (JIA), and inflammatory bowel diseases. We recently completed several clinical trials of anti-cytokine treatment for children with systemic inflammatory diseases: anti-IL-6 receptor monoclonal antibody (tocilizumab) for children with two subtypes of JIA (poly-JIA and systemic JIA), anti-TNF-α monoclonal antibody (infliximab) for children with Kawasaki disease, and anti-IL-1-β monoclonal antibody (canakinumab) for children with cryopyrin-associated periodic syndrome. This review summarizes the basis of inflammation in terms of innate immunity and adaptive immunity in these systemic inflammatory diseases, clinical efficacy, and tolerability of these biologic agents, and attempts to determine the roles of individual inflammatory cytokines in disease pathogenesis.

Characteristics associated with anti-osteoporosis medication use: data from the Global Longitudinal Study of Osteoporosis in Women (GLOW) USA cohort

PubMed Central

Guggina, Pamela; Flahive, Julie; Hooven, Frederick H.; Watts, Nelson B.; Siris, Ethel S.; Silverman, Stuart; Roux, Christian; Pfeilschifter, Johannes; Greenspan, Susan L.; Díez-Pérez, Adolfo; Cooper, Cyrus; Compston, Juliet E.; Chapurlat, Roland; Boonen, Steven; Adachi, Jonathan D.; Anderson, Frederick A.; Gehlbach, Stephen

2016-01-01

Introduction Many women at risk of fracture do not receive anti-osteoporosis medication (AOM), while others may be receiving unnecessary treatment. Purpose To examine the characteristics associated with AOM use among women at low and high risk of fracture. Methods The Global Longitudinal Study of Osteoporosis in Women (GLOW) is a prospective cohort study in which data were collected, via self-administered questionnaires, from 60,393 non-institutionalized women aged ≥55 years in 10 countries between October 1, 2006 and April 30, 2008. This is a cross-sectional analysis of baseline USA data, in which women were classified as having low fracture risk (<65 years; no FRAX risk factors) or high fracture risk (≥65 years; prior fracture or ≥2 other FRAX risk factors). Results Of 27,957 women, 3013 were at low risk of fracture and 3699 were at high risk. Only 35.7% of high-risk women reported AOM treatment, rising to 39.5% for those with self-reported osteopenia and 65.4% for those with self-reported osteoporosis. Conversely, 13.4% of low-risk women reported AOM, rising to 28.7% for osteopenia and 62.4% for osteoporosis. Characteristics associated with significantly higher AOM treatment rates among low-and high-risk women were: osteoporosis (odds ratios 75.3 and 18.1, respectively), osteopenia (17.9 and 6.3), concern about osteoporosis (2.0 and 1.8), higher perceived risk of fracture (2.3 and 1.6), and higher vitality score (1.7 and 1.6). Conclusion Use of AOM is frequently inconsistent with published guidelines in both high -and low-risk women. Characteristics other than FRAX fracture risk appear to influence this use, particularly the presence of self-reported osteoporosis. PMID:22964142

Assessment of postmenopausal women and significant risk factors for osteoporosis.

PubMed

Schnatz, Peter F; Marakovits, Kimberly A; O'Sullivan, David M

2010-09-01

The assessment of osteoporosis risk factors can help guide early intervention. The objective of this study was to analyze numerous potential risk factors to see which were associated with postmenopausal osteoporosis. Women aged 49 or greater presenting for dual-energy x-ray absorptiometry bone scans were recruited from radiology sites in the Hartford, Connecticut, area between January 2007 and March 2009, inclusive. Information was collected regarding primary and secondary risk factors for osteoporosis development, as well as family history and history of pregnancy and breast-feeding. Survey results were subsequently correlated with each woman's dual-energy x-ray absorptiometry scan results. In a sample of 619 women, history of fracture (odds ratio [OR], 12.49), weight less than 127 pounds (OR, 3.50), and use of anticoagulants (OR, 5.40) increased the chance of developing osteoporosis. In contrast, multiparity (OR, 0.45) and history of breast-feeding (OR, 0.38) decreased the development of osteoporosis in postmenopausal women. In women aged 49 to 54, breast-feeding was significantly protective, while low body mass index was most indicative of osteoporosis in women ages 55 to 64. Both previous fracture and low body mass index were associated with osteoporosis in women over age 64. The current results are consistent with other studies suggesting that previous fracture, low body weight, and use of anticoagulants increase the risk of osteoporosis. Our results also suggest that a history of pregnancy and breast-feeding protects against the development of postmenopausal osteoporosis, especially in women aged 49 to 54.

The frequency and outcome of uveitis in patients with newly diagnosed juvenile idiopathic arthritis in two 4-year cohorts from 1990-1993 and 2000-2003.

PubMed

Kotaniemi, Kaisu; Sihto-Kauppi, Kristiina; Salomaa, Pirjo; Säilä, Hanna; Ristolainen, Leena; Kauppi, Markku

2014-01-01

To retrospectively compare the frequency and outcome of uveitis between two cohorts of patients with newly-onset juvenile idiopathic arthritis (JIA) separated by a 10 year interval. The diagnosis of JIA was made in 239 patients in 1990-1993 and in 240 patients in 2000-2003 by paediatric rheumatologists at the Rheumatism Foundation Hospital, Heinola, Finland. An ophthalmologist examined all the patients regularly and diagnosed uveitis. The demographics of the patients, type of JIA, frequency, medical treatment and outcome of uveitis were documented. The main outcome measures were the frequency and outcome of uveitis, the number of complications and the best corrected visual acuity (BCVA), need of corticosteroids and other immunosuppressive treatment. The frequency of uveitis was higher (25% vs. 18%) in the earlier cohort. The visual outcome was ≥0.5 in all JIA-uveitis patients except one in the earlier cohort. Complications were fewer (21% vs. 35%) and uveitis was milder according to the Standardisation of Uveitis Nomenclature (SUN) criteria in the later cohort. Remission of uveitis (33% vs. 42%) and arthritis (20% vs. 23%) in JIA-uveitis patients was similar in both cohorts after a follow-up of 6.6 and 5.9 years, respectively. Systemic corticosteroids were more commonly used (25% vs. 7%) in JIA-uveitis patients of the earlier cohort but the use of methotrexate was equal in both cohorts (65% vs. 67%). In this study with early and aggressive treatment and close monitoring the outcome of JIA-uveitis patients was favourable and visual loss was avoided in most cases.

Access to Oral Osteoporosis Drugs among Female Medicare Part D Beneficiaries

PubMed Central

Lin, Chia-Wei; Karaca-Mandic, Pinar; McCullough, Jeffrey S.; Weaver, Lesley

2014-01-01

Background For women living with osteoporosis, high out-of-pocket drug costs may prevent drug therapy initiation. We investigate the association between oral osteoporosis out-of-pocket medication costs and female Medicare beneficiaries’ initiation of osteoporosis drug therapy. Methods We used 2007 and 2008 administrative claims and enrollment data for a 5% random sample of Medicare beneficiaries. Our study sample included age-qualified, female beneficiaries who had no prior history of osteoporosis but were diagnosed with osteoporosis in 2007 or 2008. Additionally, we only included beneficiaries continuously enrolled in standalone prescription drug plans. We excluded beneficiaries who had a chronic condition that was contraindicated with osteoporosis drug utilization. Our final sample included 25,069 beneficiaries. Logistic regression analysis was used to examine the association between the out-of-pocket costs and initiation of oral osteoporosis drug therapy during the year of diagnosis. Findings Twenty-six percent of female Medicare beneficiaries newly diagnosed with osteoporosis initiated oral osteoporosis drug therapy. Beneficiaries’ out-of-pocket costs were not associated with the initiation of drug therapy for osteoporosis. However, there were statistically significant racial disparities in beneficiaries’ initiation of drug therapy. African Americans were 3 percentage points less likely to initiate drug therapy than whites. In contrast, Asian/Pacific Islander and Hispanic beneficiaries were 8 and 18 percentage points respectively more likely to initiate drug therapy than whites. Additionally, institutionalized beneficiaries were 11 percentage points less likely to initiate drug therapy than other beneficiaries. Conclusions Access barriers for drug therapy initiation may be driven by factors other than patients’ out-of-pocket costs. These results suggest that improved osteoporosis treatment requires a more comprehensive approach that goes beyond payment

Clinical practice guidelines for the diagnosis and management of osteoporosis. Scientific Advisory Board, Osteoporosis Society of Canada.

PubMed Central

1996-01-01

OBJECTIVE: To recommend clinical practice guidelines for the assessment of people at risk for osteoporosis, and for effective diagnosis and management of the condition. OPTIONS: Screening and diagnostic methods: risk-factor assessment, clinical evaluation, measurement of bone mineral density, laboratory investigations. Prophylactic and corrective therapies: calcium and vitamin D nutritional supplementation, physical activity and fall-avoidance techniques, ovarian hormone therapy, bisphosphonate drugs, other drug therapies. Pain-management medications and techniques. OUTCOMES: Prevention of loss of bone mineral density and fracture; increased bone mass; and improved quality of life. EVIDENCE: Epidemiologic and clinical studies and reports were examined, with emphasis on recent randomized controlled trials. Clinical practice in Canada and elsewhere was surveyed. Availability of treatment products and diagnostic equipment in Canada was considered. VALUES: Cost-effective methods and products that can be adopted across Canada were considered. A high value was given to accurate assessment of fracture risk and osteoporosis, and to increasing bone mineral density, reducing fractures and fracture risk and minimizing side effects of diagnosis and treatment. BENEFITS, HARMS AND COSTS: Proper diagnosis and management of osteoporosis minimize injury and disability, improve quality of life for patients and reduce costs to society. Rationally targeted methods of screening and diagnosis are safe and cost effective. Harmful side effects and costs of recommended therapies are minimal compared with the harms and costs of untreated osteoporosis. Alternative therapies provide a range of choices for physicians and patients. RECOMMENDATIONS: Population sets at high risk should be identified and then the diagnosis confirmed through bone densitometry. Dual-energy x-ray absorptiometry is the preferred measurement technique. Radiography can be adjunct when indicated. Calcium and vitamin D

Reversal of Sepsis-Like Features of Neutrophils by Interleukin-1 Blockade in Patients With Systemic-Onset Juvenile Idiopathic Arthritis.

PubMed

Ter Haar, Nienke M; Tak, Tamar; Mokry, Michal; Scholman, Rianne C; Meerding, Jenny M; de Jager, Wilco; Verwoerd, Anouk; Foell, Dirk; Vogl, Thomas; Roth, Johannes; Leliefeld, Pieter H C; van Loosdregt, Jorg; Koenderman, Leo; Vastert, Sebastiaan J; de Roock, Sytze

2018-06-01

Neutrophils are the most abundant innate immune cells in the blood, but little is known about their role in (acquired) chronic autoinflammatory diseases. This study was undertaken to investigate the role of neutrophils in systemic-onset juvenile idiopathic arthritis (JIA), a prototypical multifactorial autoinflammatory disease that is characterized by arthritis and severe systemic inflammation. Fifty patients with systemic-onset JIA who were receiving treatment with recombinant interleukin-1 receptor antagonist (rIL-1Ra; anakinra) were analyzed at disease onset and during remission. RNA sequencing was performed on fluorescence-activated cell-sorted neutrophils from 3 patients with active systemic-onset JIA and 3 healthy controls. Expression of activation markers, apoptosis, production of reactive oxygen species (ROS), and degranulation of secretory vesicles from neutrophils were assessed by flow cytometry in serum samples from 17 patients with systemic-onset JIA and 15 healthy controls. Neutrophil counts were markedly increased at disease onset, and this correlated with the levels of inflammatory mediators. The neutrophil counts normalized within days after the initiation of rIL-1Ra therapy. RNA-sequencing analysis revealed a substantial up-regulation of inflammatory processes in neutrophils from patients with active systemic-onset JIA, significantly overlapping with the transcriptome of sepsis. Correspondingly, neutrophils from patients with active systemic-onset JIA displayed a primed phenotype that was characterized by increased ROS production, CD62L shedding, and secretory vesicle degranulation, which was reversed by rIL-1Ra treatment in patients who had achieved clinical remission. Patients with a short disease duration had high neutrophil counts, more immature neutrophils, and a complete response to rIL-1Ra, whereas patients with symptoms for >1 month had normal neutrophil counts and an unsatisfactory response to rIL-1Ra. In vitro, rIL-1Ra antagonized the

Osteoporosis: Symptoms, Diagnosis, Treatment and Prevention | NIH MedlinePlus the Magazine

MedlinePlus

... of this page please turn Javascript on. Feature: Osteoporosis Osteoporosis: Symptoms, Diagnosis, Treatment and Prevention Past Issues / Winter 2011 Table of Contents Osteoporosis can strike at any age, although the risk ...

Side Effects of HIV Medicines: HIV and Osteoporosis

MedlinePlus

... risk factors for osteoporosis include a poor diet, physical inactivity, and smoking. These risk factors can be managed ... and vitamin D increases the risk of osteoporosis. Physical inactivity : Bones become stronger with exercise, so physical inactivity ...

Diagnosis of Osteoporosis.

ERIC Educational Resources Information Center

Wahner, H. W.

1987-01-01

Early recognition of osteoporosis is difficult because symptoms are lacking and there are no distinct, readily accessible diagnostic features. This article reviews the standard approach, radiographic and laboratory diagnosis, bone mass measurement techniques, and interpretation of bone mineral data. (MT)

Definition of improvement in juvenile idiopathic arthritis using the juvenile arthritis disease activity score.

PubMed

Horneff, Gerd; Becker, Ingrid

2014-07-01

The aim of this study was to define improvement thresholds for the Juvenile Arthritis Disease Activity Score (JADAS). Physicians' and parents' judgements on treatment efficacy, the ACR paediatric response measure (PedACR) and JADAS were extracted from BIKER. Patients were categorized by baseline classes in the 10-joint JADAS (JADAS10) as low (5 to <15), moderate (15 to <25) and high (25 to ≤40). Cut-offs for defining improvement following treatment with biologics or MTX were chosen by calculating the interquartile ranges (IQRs) of the judgement groups and considering the accuracy, sensitivity and specificity of the resulting model. Differences in the change of JADAS10 by JIA category were also analysed by analysis of variance (ANOVA). Sensitivity, specificity and accuracy were calculated. A total of 1315 treatment courses were analysed. The ANOVA of the JIA categories showed no significant differences of the mean JADAS10 in all baseline classes and IQRs also showed good overall limits. Therefore all JIA categories were combined for a collective cut-off. Analysis by baseline class revealed clear cut-off points. Improvement could be defined by the minimal decrease in the JADAS10 in baseline class low by 4 (41%), moderate by 10 (53%) and high by 17 (57%). The model shows values for accuracy from 75.6 to 85.5% and comparable values for sensitivity and specificity. Improvement after 3 months can be defined efficiently by the decrease of the JADAS10, depending on the baseline JADAS10 score, which specifies low, moderate or high disease activity. Our model demonstrates clear cut-off values. The JADAS10 may be used in addition to ACR criteria in clinical trials. Also, since the JADAS10 can easily be calculated at each patient visit, it also can be used for clinical decisions. © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Growth Failure in Children with Systemic Juvenile Idiopathic Arthritis and Prolonged Inflammation despite Treatment with Biologicals: Late Normalization of Height by Combined Hormonal Therapies.

PubMed

de Zegher, Francis; Reynaert, Nele; De Somer, Lien; Wouters, Carine; Roelants, Mathieu

2018-06-25

Biologicals targeting the interleukin (IL)-1β or IL-6 pathway are becoming prime choices for the treatment of children with systemic juvenile idiopathic arthritis (sJIA). Up to 1 in 3 sJIA children receiving such treatment continues to have inflammatory activity and to require supra-physiological glucocorticoid doses which may reduce growth velocity for years and may lead to an extremely short stature for age, if not for life. Currently, there is no long-term proposal to normalize the adult height of these children with sJIA. We present long-term (up to 10 years), proof-of-concept evidence that the adult stature and adipose body composition of short sJIA children can be normalized with a hormonal combination strategy: (i) pubertal onset is postponed with a gonadotropin-releasing hormone analog (triptorelin) until a minimum height is reached, or until prepubertal growth is exhausted, and (ii) height gain is promoted with growth hormone (≈50 μg/kg/day), once inflammation is under control and high glucocorticoid doses are no longer needed. The latter treatment takes advantage of the window of relative glucocorticoid deficiency, which is known to open after prolonged glucocorticoid administration, and to be uniquely favorable to height gain. A long-term combination of biological and hormonal treatments for short sJIA children can be guided by a simple concept that involves (i) postponement of pubertal development and (ii) growth-promoting therapy after the episodes of major inflammation and high-dose glucocorticoid treatment. Limited long-term experience in short sJIA children suggests that this strategy leads consistently - albeit late - to a normal adult stature. © 2018 S. Karger AG, Basel.

Osteoporosis prevention, diagnosis, and therapy.

PubMed

The objective of this NIH Consensus Statement is to inform the biomedical research and clinical practice communities of the results of the NIH Consensus Development Conference on Osteoporosis Prevention, Diagnosis, and Therapy. The statement provides state-of-the-art information and presents the conclusions and recommendations of the consensus panel regarding these issues. In addition, the statement identifies those areas of study that deserve further investigation. The target audience of clinicians for this statement includes, but is not limited to, family practitioners, internists, gerontologists, orthopaedic surgeons, rheumatologists, obstetricians and gynecologists, and preventive medicine specialisits. A nonfederal, nonadvocate, 13-member panel representing the fields of internal medicine, family and community medicine, endocrinology, epidemiology, orthopaedic surgery, gerontology, rheumatology, obstetrics and gynecology, preventive medicine, and cell biology. In addition, 32 experts from these same fields presented data to the panel and a conference audience of approximately 700. The literature was searched using MEDLINE and an extensive bibliography of references was provided to the panel. Experts prepared abstracts for their conference presentations with relevant citations from the literature. Scientific evidence was given precedence over clinical anecdotal experience. The panel, answering predefined questions, developed their conclusions based on the scientific evidence presented in open forum and the scientific literature. The panel composed a draft statement, which was read in its entirety and circulated to the experts and the audience for comment. Thereafter, the panel resolved conflicting recommendations and released a revised statement at the end of the conference. The panel finalized the revisions within a few weeks after the conference. The draft statement was made available on the World Wide Web immediately following its release at the conference