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Sample records for idiopathic juvenile osteoporosis

  1. Idiopathic juvenile osteoporosis: experience of twenty-one patients.

    PubMed

    Smith, R

    1995-01-01

    The features and outcome of 21 children (12 boys, nine girls) with idopathic juvenile osteoporosis (IJO) followed for up to 23 yr are described. The mean age of onset was 7 yr (range 1-13 yr) with no sex difference; the main presenting symptoms were long bone fractures, pain in the back and difficulty in walking. Typically, radiographs demonstrated compression of the vertebrae and metaphyses of the long bones; bone histology sometimes showed an excess of osteocytes associated with woven bone; routine biochemistry was normal for age; and with three exceptions no abnormalities of extracted dermal collagen or collagen synthesized by fibroblasts were detected. Where circulating vitamin D metabolites were measured they were within the normal range; and hip and spine bone mineral density (measured by DXA) was strikingly low. Five patients are still growing and two are currently untraceable. Of the remaining 14 who are now adults, 11 have substantially or completely recovered and three are disabled. Since spontaneous recovery occurs it remains impossible to assess the many forms of treatment given.

  2. Evidence of Altered Matrix Composition in Iliac Crest Biopsies from Patients with Idiopathic Juvenile Osteoporosis

    PubMed Central

    Garcia, IJ; Chiodo, V; Ma, Y; Boskey, AL

    2015-01-01

    Purpose Idiopathic juvenile osteoporosis (IJO) is a rare condition in children, characterized by bone pain, long bone and vertebral fractures. Previously, IJO bone was solely characterized by histomorphometry and quantitative computed tomography. The goal of this study is to describe IJO bone composition. Materials and Methods Fourier transform infrared imaging (FTIRI), a vibrational spectroscopic technique providing spatially resolved images of chemical composition, was used to determine whether iliac crest biopsies from children with IJO differed in composition from and age- and sex-matched controls, and, as a secondary analysis, whether IJO-bone showed the same disease dependent change in composition as do iliac crest bone biopsies from women with postmenopausal osteoporosis (PMO). Wilcoxon rank-tests and linear regressions were used to analyze FTIRI variables (mineral-to-matrix ratio, carbonate-to-phosphate ratio, crystallinity, acid-phosphate substitution, collagen maturity) and their individual pixel distributions (Heterogeneity). Results Mineral-to-matrix ratio was comparable in IJO and age-matched controls. Contrastingly, collagen maturity (also known as collagen cross-link ratio) was higher in cortical and cancellous IJO bone compared to juvenile controls. Acid-phosphate substitution was greater in IJO cancellous bone than in age-matched controls, suggesting IJO bone mineral is formed more recently, reflecting a slower mineralization process. This agrees with findings of increased heterogeneity for mineral-to-matrix and collagen maturity ratios in IJO cancellous bone. There were negative correlations between cancellous collagen maturity and previously reported histomorphometric bone formation markers. There were no correlations with indices of remodeling. Conclusions IJO bone, similar to PMO bone, had elevated collagen maturity relative to its age-matched controls. This emphasizes the importance of the collagen matrix for bone health. IJO bone differed

  3. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... for You Healthy School Lunch Planner Juvenile Idiopathic Arthritis (JIA) KidsHealth > For Teens > Juvenile Idiopathic Arthritis (JIA) ... people under age 17. What Is Juvenile Idiopathic Arthritis? Arthritis doesn't affect young people as much ...

  4. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Juvenile Idiopathic Arthritis (JIA) KidsHealth > For Teens > Juvenile Idiopathic ... can affect people under age 17. What Is Juvenile Idiopathic Arthritis? Arthritis doesn't affect young people ...

  5. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...

  6. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... rule out other conditions or infections, such as Lyme disease , that may cause similar symptoms or occur along ... ESR) Bones, Muscles, and Joints Evaluate Your Child's Lyme Disease Risk Word! Arthritis Arthritis Lupus Juvenile Idiopathic Arthritis ( ...

  7. Idiopathic juvenile osteoporosis: a cross-sectional single-centre experience with bone histomorphometry and quantitative computed tomography

    PubMed Central

    2013-01-01

    Background Idiopathic juvenile osteoporosis (IJO) is a rare condition of poorly understood etiology and pathophysiology that affects otherwise healthy children. This condition is characterized clinically by bone pain and vertebral fractures; spontaneous recovery is observed after puberty in the majority of cases. Although decreased trabecular bone turnover has been noted previously, cortical and trabecular bone characteristics as determined by quantitative computed tomography (QCT) and their relationship to bone histomorphometry are unknown. Methods All children with a clinical diagnosis of IJO who were followed in our center since 1995 and who had undergone at least one diagnostic bone biopsy were included in this cross-sectional analysis. Results Fifteen patients (11 males/4 females) with median ages of 5.8 and 10.2 years at first symptoms and at referral, respectively, were included in the analysis. Histomorphometric analysis demonstrated decreased trabecular bone turnover (BFR/BS) in the majority of patients with heterogeneous parameters of trabecular mineralization and volume. QCTresults demonstrated that bone mineral density (BMD) was reduced in both trabecular/lumbar and cortical/femoral bone: Z score: -2.1 (−3.6;–1.0) and −0.9 (−8.2;1.4)in the two compartments, respectively. In the eight patients who underwent both bone biopsy and QCT, cortical BMD was associated with trabecular separation and with trabecular bone formation rate (r = 0.898 and −0.881, respectively, both p < 0.05). Conclusions This series confirms that IJO is characterized by impaired trabecular architecture that can be detected by both bone biopsy and QCT. The association between bone biopsy and QCT results may have implications for diagnosis, treatment, and follow-up of these children. PMID:23418950

  8. Genetics Home Reference: juvenile primary osteoporosis

    MedlinePlus

    ... Home Health Conditions juvenile primary osteoporosis juvenile primary osteoporosis Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Juvenile primary osteoporosis is a skeletal disorder characterized by thinning of ...

  9. Genetics Home Reference: juvenile idiopathic arthritis

    MedlinePlus

    ... Home Health Conditions juvenile idiopathic arthritis juvenile idiopathic arthritis Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  10. Juvenile idiopathic arthritis.

    PubMed

    Bhatt, Krupa H; Karjodkar, Freny R; Sansare, Kaustubh; Patil, Darshana

    2014-01-01

    Juvenile Idiopathic Arthritis (JIA) is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential.

  11. [Juvenile idiopathic systemic arthritis].

    PubMed

    Baksiene, Dalia; Kasparaviciene, Jūrate; Zebiene, Migla; Puteliene, Brigita

    2003-01-01

    THE PURPOSE OF THE STUDY was to evaluate the peculiarities of the clinical features, laboratory parameters and tactics of treatment in juvenile idiopatic systematic arthritis. A retrospective data review of 41 children (26 boys and 15 girls) who underwent treatment for systemic arthritis (according to ILAR criteria) in our institution between 1992 and 2002 was performed. The disease started with fever of unknown origin in all cases. In 73% of patients it lasted longer than one month, in 54% fever was with twice daily spikers in the morning and in the evening. The rash during the rise of temperature appeared in 49%, in most cases (70%) there was a maculo-papular rash. Lymphadenopathy and serositis were observed in 32%, hepatomegaly in 29%, and splenomegaly in 24%. Arthritis coincided with the fever in 29% of patients, in majority of cases it was progressing to a severe persistent arthritis after the systematic phase. There was no specific laboratory findings: neutrophilic leucocytosis was found in 73%, anemia - in 80.5%, trombocytosis - in 36.6%, elevated CRP - in 63.4%, dysproteinemia - in 79% of patients. Antinuclear factors were absent in all examined children. For all patients intravenous methylprednisolone pulses have been administered (10-22 mg/kg/infusion). Prednisolone was also continued orally (1-2 mg/kg/day). 24.4% of patients required in addition immunosupressive agents such as methotrexate, azathioprine and cyclophosphamide. Puls-therapy of methylprednisolone is a safe and sufficiently effective method of treatment in most cases of the systematic juvenile arthritis.

  12. Pamidronate treatment stimulates the onset of recovery phase reducing fracture rate and skeletal deformities in patients with idiopathic juvenile osteoporosis: comparison with untreated patients.

    PubMed

    Baroncelli, Giampiero I; Vierucci, Francesco; Bertelloni, Silvano; Erba, Paola; Zampollo, Elisa; Giuca, Maria Rita

    2013-09-01

    Although spontaneous remission occurs in patients with idiopathic juvenile osteoporosis (IJO), permanent bone deformities may occur. The effects of long-term pamidronate treatment on clinical findings, bone mineral status, and fracture rate were evaluated. Nine patients (age 9.8 ± 1.1 years, 7 males) with IJO were randomized to intravenous pamidronate (0.8 ± 0.1 mg/kg per day for 3 days; cycles per year 2.0 ± 0.1; duration 7.3 ± 1.1 years; n = 5) or no treatment (n = 4). Fracture rate, phalangeal quantitative ultrasound, and lumbar bone mineral density (BMD) by dual energy X-ray absorptiometry at entry and during follow-up (range 6.3-9.4 years) were assessed. Bone pain improved in treated patients. Difficulty walking continued for 3-5 years in untreated patients, and vertebral collapses occurred in three of them. During follow-up, phalangeal amplitude-dependent speed of sound (AD-SoS), bone transmission time (BTT), and lumbar BMDarea and BMDvolume progressively increased in treated patients (P < 0.05-P < 0.0001). In untreated patients AD-SoS and BTT decreased during the first 2-4 years of follow-up (P < 0.05-P < 0.01); lumbar BMDarea increased after 6 years (P < 0.001) whereas BTT and lumbar BMDvolume increased after 7 years of follow-up (P < 0.05 and P < 0.001, respectively). At the end of follow-up, AD-SoS, BTT, lumbar BMDarea, and BMDvolume Z-scores were lower in untreated patients than in treated patients (-2.2 ± 0.3 and -0.5 ± 0.2; -1.9 ± 0.2 and -0.6 ± 0.2; -2.3 ± 0.3 and -0.7 ± 0.3; -2.4 ± 0.2 and -0.7 ± 0.3, P < 0.0001, respectively). Fracture rate was higher in untreated patients than in treated patients during the first 3 years of follow-up (P < 0.02). Our study showed that spontaneous recovery of bone mineral status is unsatisfactory in patients with IJO. Pamidronate treatment stimulated the onset of recovery phase reducing fracture rate and permanent disabilities without evidence of

  13. [Idiopathic and secondary osteoporosis in childhood].

    PubMed

    Rossi, F; Perrotta, S; Falcone, E; Gimigliano, F; Iodice, M; Vetrella, S; Iolascon, G

    2005-10-01

    Osteoporosis is a common disease characterized by reduced bone mass, with a consequent increase in bone fragility and susceptibility to fracture risk. Bone mineral density (BMD) measurement is used to make the diagnosis of osteoporosis prior to incident fracture, and to predict fracture risk. BMD is determined by the peak bone mass achieved, and the rate and timing of subsequent bone loss. Dual-energy X-ray absorptiometry (DEXA) is the most popular and effective method utilized for osteoporosis screening. Bone disease is a side effect of concern regarding chronic glucocorticoid (GC) administration. Most GC-treated patients exhibit a process of bone loss, frequently leading to osteoporosis, with increased fracture risk, especially in spinal vertebrae. Osteogenesis imperfecta is an inherited and generalized connective tissue disorder characterized mainly by bone fragility. Idiopathic osteoporosis of childhood or adolescence without blue sclerae and other stigmata of osteogenesis imperfecta is occasionally observed and sometimes more than one sib is affected. Beta-thalassemia major is associated with significant bone disease. The etiology of the bone disease is still debatable, many factors can adversely affect bone accretion in thalassemic patients. These include delayed puberty, bone marrow expansion, the deleterious effects of desferrioxamine, iron overload and genetic factors. Current treatment alternatives of osteoporosis include bisphosphonates, calcitonin, and selective estrogen receptor modulators.

  14. [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.

  15. Kienbock's disease and juvenile idiopathic arthritis.

    PubMed

    Desy, Nicholas M; Bernstein, Mitchell; Harvey, Edward J; Hazel, Hazel

    2011-06-01

    Kienbock's disease or osteonecrosis of the lunate is an uncommon cause of wrist pain. . Though there have been several reports of cases in patients with various rheumatologic diseases, the precise etiology has currently not been established. We report a case of Kienbock's disease that occurred in a patient with juvenile idiopathic arthritis. To our knowledge, this is the first case report with an association between these two conditions.

  16. Anakinra for myocarditis in juvenile idiopathic arthritis.

    PubMed

    Movva, Rajesh; Brown, Suzanne B; Morris, D Lynn; Figueredo, Vincent M

    2013-01-01

    A 20-year-old pregnant woman with a history of juvenile idiopathic arthritis presented with flu-like symptoms, systemic inflammation with myocarditis, and severe cardiomyopathy. Six weeks earlier, her chronic-arthritis therapy had been changed from anakinra, an interleukin-1β receptor antagonist, to etanercept. When she resumed taking anakinra, her condition improved dramatically, including a complete recovery of ventricular function. Myocarditis is a well-recognized complication of systemic vasculitides. This unusual case emphasizes the important pathophysiologic role of interleukin receptors in the successful treatment of myocarditis. We suggest that clinical cardiologists be aware of the therapeutic usefulness of biological agents such as anakinra in patients with rheumatic conditions.

  17. Remission in juvenile idiopathic arthritis: current facts.

    PubMed

    Shenoi, Susan; Wallace, Carol A

    2010-04-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthritic disease affecting children. Despite the availability of potent disease-modifying antirheumatic medications, most children still experience a chronic course with long periods of active disease. Goals of treatment should include achievement of disease remission with optimal physical functioning that allows children to lead normal lives with no structural joint damage. The term remission implies a complete lack of disease activity. This article focuses on recently developed preliminary criteria for inactive disease and remission in JIA. Recent studies using these new definitions demonstrate only modest rates of achievement of remission favoring children with persistent oligoarticular JIA. Children with rheumatoid factor-positive polyarticular JIA are least likely to achieve remission. Therapeutic strategies to achieve remission are also discussed.

  18. Use of methotrexate in juvenile idiopathic arthritis

    PubMed Central

    Ramanan, A; Whitworth, P; Baildam, E

    2003-01-01

    Methotrexate (MTX) has transformed the outlook for children with juvenile idiopathic arthritis (JIA). Most of the evidence from uncontrolled clinical trials suggests that MTX is an effective agent for treating active JIA. Data from controlled clinical trials suggests that MTX has statistically significant effects on patient centred disability measures in JIA patients with active arthritis. Although we would like a much larger study directed evidence base for our use of the drug, the studies that have been done are sound and have been followed by a change in clinical expectations and advice that speak of qualitative evidence from clinical practice, confirming the scientifically acquired data. Randomised controlled multicentre trials using sufficient numbers of patients, including functional assessment and quality of life measures, are needed to confirm the long term efficacy and safety of MTX in JIA. PMID:12598376

  19. Pulmonary haemosiderosis with juvenile idiopathic arthritis in a Malaysian child.

    PubMed

    Wong, A R; Noor, A S Siti; Rasool, A H G; Quah, B S; Roberton, D

    2007-10-01

    A rare case of childhood pulmonary haemosiderosis with juvenile idiopathic arthritis is discussed, with particular reference to treatment with hydroxychloroquine and sildenafil for pulmonary hypertension which occurs secondary to this disease.

  20. Clinical outcome measures in juvenile idiopathic arthritis.

    PubMed

    Consolaro, Alessandro; Giancane, Gabriella; Schiappapietra, Benedetta; Davì, Sergio; Calandra, Serena; Lanni, Stefano; Ravelli, Angelo

    2016-04-18

    Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with significant disease- and treatment-related morbidity, thus impacting children's quality of life. In order to optimize JIA management, the paediatric rheumatologist has begun to regularly use measurements of disease activity developed, validated and endorsed by international paediatric rheumatology professional societies in an effort to monitor the disease course over time and assess the efficacy of therapeutic interventions in JIA patients.A literature review was performed to describe the main outcome measures currently used in JIA patients to determine disease activity status.The Juvenile Disease Activity Score (JADAS), in its different versions (classic JADAS, JADAS-CRP and cJADAS) and the validated definitions of disease activity and response to treatment represent an important tool for the assessment of clinically relevant changes in disease activity, leading more and more to a treat-to-target strategy, based on a tight and thorough control of the patient condition. Moreover, in recent years, increasing attention on the incorporation of patient-reported or parent-reported outcomes (PRCOs), when measuring the health state of patients with paediatric rheumatic diseases has emerged.We think that the care of JIA patients cannot be possible without taking into account clinical outcome measures and, in this regard, further work is required.

  1. [Unusual presentation of juvenile idiopathic arthritis and autoimmune hepatitis].

    PubMed

    Moreno Prieto, M; Carbonero Celis, M J; Cuadrado Caballero, M C

    2015-01-01

    The coexistence of autoimmune hepatitis and juvenile idiopathic arthritis is very rare. This is the case of an 18 month old female patient whose first sign of disease was torticollis due to an underlying atlanto-axial subluxation. Three months later, bilateral knee arthritis developed and she was diagnosed with Juvenile Idiopathic Arthritis. Throughout the disease a persistent elevation of liver enzymes was noted, combined with positive antinuclear antibodies and hypergammaglobulinemia, reaching the diagnosis of concomitant autoimmune hepatitis.

  2. The human microbiome and juvenile idiopathic arthritis.

    PubMed

    Verwoerd, Anouk; Ter Haar, Nienke M; de Roock, Sytze; Vastert, Sebastiaan J; Bogaert, Debby

    2016-09-20

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The microbiome has received increasing attention as a potential contributing factor to the development of a wide array of immune-mediated diseases, including inflammatory bowel disease, type 1 diabetes and rheumatoid arthritis. Also in JIA, there is accumulating evidence that the composition of the microbiome is different from healthy individuals. A growing body of evidence indeed suggests that, among others, the microbiome may influence the development of the immune system, the integrity of the intestinal mucosal barrier, and the differentiation of T cell subsets. In turn, this might lead to dysregulation of the immune system, thereby possibly playing a role in the development of JIA. The potential to manipulate the microbiome, for example by faecal microbial transplantation, might then offer perspectives for future therapeutic interventions. Before we can think of such interventions, we need to first obtain a deeper understanding of the cause and effect relationship between JIA and the microbiome. In this review, we discuss the existing evidence for the involvement of the microbiome in JIA pathogenesis and explore the potential mechanisms through which the microbiome may influence the development of autoimmunity in general and JIA specifically.

  3. [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.

  4. [Optic neuritis in juvenile idiopathic arthritis patient].

    PubMed

    Lourenço, Daniela M R; Buscatti, Izabel M; Lourenço, Benito; Monti, Fernanda C; Paz, José Albino; Silva, Clovis A

    2014-01-01

    Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5,793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  5. 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.

  6. Osteoporosis in men with idiopathic hypogonadotropic hypogonadism

    SciTech Connect

    Finkelstein, J.S.; Klibanski, A.; Neer, R.M.; Greenspan, S.L.; Rosenthal, D.I.; Crowley, W.F. Jr.

    1987-03-01

    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 similarly 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.

  7. Juvenile idiopathic arthritis: review of the literature and case report.

    PubMed

    Beena, J P

    2013-01-01

    Juvenile idiopathic arthritis (JIA), previously known as juvenile chronic arthritis or juvenile rheumatoid arthritis, is a chronic disease of childhood with a spectrum of joint involvement and associated systemic involvement. The cause of JIA is poorly understood, and no drugs can cure the disease currently. Pediatric dentists should be familiar with the symptoms, complications, and oral manifestations of JIA to help manage the disease and provide quality care to these patients. The purpose of this case report is to review the condition and to describe the case of an adolescent with polyarticular juvenile rheumatoid arthritis, focusing on specific recommendations for dental management.

  8. Muscle involvement in juvenile idiopathic arthritis.

    PubMed

    Lindehammar, H; Lindvall, B

    2004-12-01

    An observational study of changes in muscle structure and the relation to muscle strength in juvenile idiopathic arthritis (JIA). Fifteen children and teenagers (eight girls and seven boys) with JIA, aged 9-19 yr (mean age 16.1), were studied. Muscle biopsies were obtained from the anterior tibial muscle and were examined using histopathological and immunohistochemical methods. Muscle fibre types were classified and fibre areas measured. As markers of inflammation, the major histocompatibility complex (MHC) class I and class II and the membrane attack complex (MAC) were analysed. Results were compared with biopsies from the gastrocnemius muscle in 33 young (19-23 yr) healthy controls. Isometric and isokinetic muscle strengths were measured in ankle dorsiflexion. Strength was compared with reference values for healthy age-matched controls. Nerve conduction velocities were recorded in the peroneal and sural nerves. Four of the 15 muscle biopsies were morphologically normal. Eleven biopsies showed minor unspecific changes. Two of these also showed minor signs of inflammation. MHC class II expression was found in 4/15 patients, which was significantly more than in the healthy controls (P = 0.0143). The expression of MHC class I and MAC did not differ from that in the controls. The mean area of type I fibres was lower than that of type IIA fibres in 12/13 biopsies. Muscle strength was significantly reduced in the patient group. There was a significant positive correlation between muscle fibre area and muscle strength. Nerve conduction studies were normal in all cases. Changes in leg muscle biopsies appear to be common in children and teenagers with JIA. The presence of inflammatory cells in the muscle and expression of MHC class II on muscle fibres may be a sign of inflammatory myopathy. There are no findings of type II muscle fibre hypotrophy or neuropathy, as in adults with RA.

  9. Immune Complexes in Juvenile Idiopathic Arthritis

    PubMed Central

    Moore, Terry L.

    2016-01-01

    Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA. PMID:27242784

  10. Immune Complexes in Juvenile Idiopathic Arthritis.

    PubMed

    Moore, Terry L

    2016-01-01

    Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients' sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA.

  11. AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis.

    PubMed

    Saha, Abhijeet; Chopra, Yogiraj; Theis, Jason D; Vrana, Julie A; Sethi, Sanjeev

    2013-10-01

    We report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive management of the systemic-onset juvenile idiopathic arthritis resulted in improvement in clinical and laboratory parameters. The case represents an unusual cause of nephrotic syndrome in children. Early diagnosis of renal amyloidosis and management of systemic-onset juvenile idiopathic arthritis is paramount to preventing progression of kidney disease.

  12. Exercise therapy in juvenile idiopathic arthritis.

    PubMed

    Takken, T; van Brussel, M; Engelbert, R H H; Van der Net, J; Kuis, W; Helders, P J M

    2008-04-16

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability, quality of life and aerobic capacity in children with JIA. The Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Database of Systematic Reviews (The Cochrane Library), MEDLINE (January 1966 to April 2007), CINAHL (January 1982 to April 2007), EMBASE (January 1966 to October 2007), PEDro (January 1966 to October 2007), SportDiscus (January 1966 to October 2007), Google Scholar (to October 2007), AMED (Allied and Alternative Medicine) (January 1985 to October 2007), Health Technologies Assessment database (January 1988 to October 2007), ISI Web Science Index to Scientific and Technical Proceedings (January 1966 to October 2007) and the Chartered Society of Physiotherapy website (http://www.cps.uk.org) were searched and references tracked. Randomised controlled trials (RCTs) of exercise treatment in JIA. Potentially relevant references were evaluated and all data were extracted by two review authors working independently. Three out of 16 identified studies met the inclusion criteria, with a total of 212 participants. All the included studies fulfilled at least seven of 10 methodological criteria. The outcome data of the following measures were homogenous and were pooled in a meta-analysis: functional ability (n = 198; WMD -0.07, 95% CI -0.22 to 0.08), quality of life (CHQ-PhS: n = 115; WMD -3.96, 95% CI -8.91 to 1.00) and aerobic capacity (n = 124; WMD 0.04, 95% CI -0.11 to 0.19). The results suggest that the outcome measures all favoured the exercise therapy but none were statistically significant. None of the studies reported negative effects of the exercise therapy. Overall, based on 'silver-level' evidence (www.cochranemsk.org) there was no clinically

  13. [Current therapy of polyarticular forms of juvenile idiopathic arthritis].

    PubMed

    Hospach, A; Rühlmann, J M; Weller-Heinemann, F

    2016-04-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in infancy and childhood. Approximately 20 % of patients with JIA suffer from the polyarticular form of the disease, which causes a substantial disease burden and long-term sequelae. Therapeutic approaches have used steroids and conventional disease modifying antirheumatic drugs (DMARD) but over the last decade new drugs have become available for the treatment of JIA, in particular biologic DMARD. This article summarizes the current therapy options for polyarticular JIA.

  14. Primary osteoporosis in children.

    PubMed

    Tan, Lay Ong; Lim, Soo Yen; Vasanwala, Rashida Farhad

    2017-09-01

    Osteoporosis in childhood is uncommon, and it may be secondary to a spectrum of diverse conditions. Idiopathic juvenile osteoporosis is a primary osteoporosis of unknown aetiology present in previously well children and is a diagnosis of exclusion. We describe a 10-year-old prepubertal boy who presented with back pain of 1-week duration. His spinal X-ray showed generalised loss of vertebral body heights in keeping with osteoporosis. Endocrine and haematological work-up were normal. He was treated with vitamin D supplement and intravenous pamidronate. This case illustrates the general work-up and causes for paediatric osteoporosis, and the management for idiopathic juvenile osteoporosis. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  15. [Pain and coping strategies in juvenile idiopathic arthritis].

    PubMed

    Herlin, Troels; Thastum, Mikael

    2008-02-18

    Pain is one of the primary symptoms of juvenile idiopathic arthritis (JIA). JIA patients have reduced pain tolerance and pain threshold compared to healthy controls. In children with JIA the greater use of coping strategies such as problem-solving, positive self-statements and distraction consistently have predicted less arthritis-related pain, even after controlling for relevant medical and demographic variables. Interventions specifically designed to modify maladaptive pain coping strategies and pain-related health beliefs may be effective in reducing pain in children with JIA.

  16. 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.

  17. 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

  18. 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

  19. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    PubMed Central

    Barut, Kenan; Sahin, Sezgin; Adrovic, Amra

    2017-01-01

    Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis. PMID:28251009

  20. Bone Density After Teriparatide Discontinuation in Premenopausal Idiopathic Osteoporosis

    PubMed Central

    Kamanda-Kosseh, Mafo; Recker, Robert. R.; Lappe, Joan M.; Dempster, David W.; Zhou, Hua; Cremers, Serge; Bucovsky, Mariana; Stubby, Julie; Shane, Elizabeth

    2015-01-01

    Context: Without antiresorptive therapy, postmenopausal women lose bone mass after teriparatide (TPTD) discontinuation; estrogen treatment prevents bone loss in this setting. It is not known whether premenopausal women with regular menses lose bone mass after teriparatide discontinuation. Objective: This study aimed to test the hypothesis that normally menstruating premenopausal women with idiopathic osteoporosis (IOP) will maintain teriparatide-associated bone mineral density (BMD) gains after medication cessation. Design: Twenty-one premenopausal IOP women previously enrolled in an open-label pilot study of teriparatide (20 mcg for 18–24 mo), had substantial BMD increases at the lumbar spine (LS; 10.8 ± 8.3%), total hip (TH; 6.2 ± 5.6%), and femoral neck (7.6 ± 3.4%). For this study, BMD was remeasured 2.0 ± 0.6 years after teriparatide cessation. Participants: Fifteen women, who had gained 11.1 ± 7.2% at LS and 6.1 ± 6.5% at TH and were premenopausal at teriparatide completion, were followed without antiresorptive treatment. Results: Two years after completing teriparatide, BMD declined by 4.8 ± 4.3% (P = .0007) at the LS. In contrast, BMD remained stable at the femoral neck (−1.5 ± 4.2%) and TH (−1.1 ± 3.7%). Those who sustained LS bone loss >3% (−7.3 ± 2.9%; n = 10), did not differ from those with stable LS BMD (0.1 ± 1.1%; n=5) with regard to baseline body mass index, BMD at any site, or duration of followup, but were significantly older at re-evaluation (46 ± 3 vs 38 ± 7; P = .046), had larger increases in LS BMD during teriparatide treatment and higher cancellous bone remodeling on transiliac biopsy at baseline and completion of teriparatide treatment. Serum bone turnover markers did not differ at baseline or teriparatide completion, but tended to be higher at the re-evaluation timepoint in those with post-teriparatide bone loss. Conclusions: These findings lead us to conclude that premenopausal women with IOP, particularly those over

  1. Skin Manifestations of Rheumatoid Arthritis, Juvenile Idiopathic Arthritis, and Spondyloarthritides.

    PubMed

    Chua-Aguilera, Carolyn Jean; Möller, Burkhard; Yawalkar, Nikhil

    2017-07-27

    Extra-articular manifestations of rheumatoid arthritis, juvenile idiopathic arthritis, and various spondyloarthritides including psoriatic arthritis, ankylosing spondylitis, reactive arthritis, and inflammatory bowel disease-associated spondyloarthritis often involve the skin and may occur before or after diagnosis of these rheumatic diseases. Cutaneous manifestations encompass a wide range of reactions that may have a notable negative impact not only on the physical but especially on the emotional and psychosocial well-being of these patients. Several cutaneous manifestations have been related to rheumatoid arthritis such as subcutaneous nodules including classical rheumatoid nodules, accelerated rheumatoid nodulosis, and rheumatoid nodulosis; vascular disorders like rheumatoid vasculitis, livedo racemosa, and Raynaud's phenomenon; and neutrophilic and/or granulomatous diseases like pyoderma gangrenosum, Sweet's syndrome, rheumatoid neutrophilic dermatitis, interstitial granulomatous dermatitis with arthritis, as well as palisaded neutrophilic and granulomatous dermatitis. In juvenile idiopathic arthritis, the main cutaneous manifestations include an evanescent rash, rheumatoid nodules, as well as plaque and guttate psoriasis. Plaque psoriasis is also the main skin disease involved in spondyloarthritides. Furthermore, other forms of psoriasis including guttate, inverse, erythrodermic, pustular, and particularly nail psoriasis may also occur. In addition, a variety of drug-induced skin reactions may also appear in these diseases. Early recognition and understanding of these different dermatologic manifestations together with an interdisciplinary approach are often needed to optimize management of these diseases.

  2. Imaging of juvenile idiopathic arthritis. Part II: Ultrasonography and MRI

    PubMed Central

    Grochowska, Elżbieta; Gietka, Piotr; Płaza, Mateusz; Pracoń, Grzegorz; Saied, Fadhil; Walentowska-Janowicz, Marta

    2016-01-01

    Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals in the developmental age. Radiography, which was described in the first part of this publication, is the standard modality in the assessment of this condition. Ultrasound and magnetic resonance imaging enable early detection of the disease which affects soft tissues, as well as bones. Ultrasound assessment involves: joint cavities, tendon sheaths and bursae for the presence of synovitis, intraand extraarticular fat tissue to visualize signs of inflammation, hyaline cartilage, cartilaginous epiphysis and subchondral bone to detect cysts and erosions, and ligaments, tendons and their entheses for signs of enthesopathies and tendinopathies. Magnetic resonance imaging is indicated in children with juvenile idiopathic arthritis for assessment of inflammation in peripheral joints, tendon sheaths and bursae, bone marrow involvement and identification of inflammatory lesions in whole-body MRI, particularly when the clinical picture is unclear. Also, MRI of the spine and spinal cord is used in order to diagnose synovial joint inflammation, bone marrow edema and spondylodiscitis as well as to assess their activity, location, and complications (spinal canal stenosis, subluxation, e.g. in the atlantoaxial region). This article discusses typical pathological changes seen on ultrasound and magnetic resonance imaging. The role of these two methods for disease monitoring, its identification in the pre-clinical stage and establishing its remission are also highlighted. PMID:27679727

  3. The Clinical Phenotypes of the Juvenile Idiopathic Inflammatory Myopathies

    PubMed Central

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

    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. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical

  4. The clinical phenotypes of the juvenile idiopathic inflammatory myopathies.

    PubMed

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

    2013-01-01

    The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with another autoimmune or connective tissue disease (JCTM), have not been well characterized, and their similarity to the adult clinical subgroups is unknown. We enrolled 436 patients with JIIM, including 354 classified as JDM, 33 as JPM, and 49 as JCTM, in a nationwide registry study. The aim of the study was to compare demographics; clinical features; laboratory measures, including myositis autoantibodies; and outcomes among these clinical subgroups, as well as with published data on adult patients with idiopathic inflammatory myopathies (IIM) enrolled in a separate natural history study. We used random forest classification and logistic regression modeling to compare clinical subgroups, following univariate analysis. JDM was characterized by typical rashes, including Gottron papules, heliotrope rash, malar rash, periungual capillary changes, and other photosensitive and vasculopathic skin rashes. JPM was characterized by more severe weakness, higher creatine kinase levels, falling episodes, and more frequent cardiac disease. JCTM had more frequent interstitial lung disease, Raynaud phenomenon, arthralgia, and malar rash. Differences in autoantibody frequency were also evident, with anti-p155/140, anti-MJ, and anti-Mi-2 seen more frequently in patients with JDM, anti-signal recognition particle and anti-Jo-1 in JPM, and anti-U1-RNP, PM-Scl, and other myositis-associated autoantibodies more commonly present in JCTM. Mortality was highest in patients with JCTM, whereas hospitalizations and wheelchair use were highest in JPM patients. Several demographic and clinical features

  5. Systemic juvenile idiopathic arthritis as a fever of unknown origin.

    PubMed

    Hardal, Cigdem; Erguven, Muferet; Saglam, Zuhal Aydan

    2017-01-01

    Juvenile idiopathic arthritis (JIA) is a rare inflammation with still unidentified cause. It can also be cause of fever of unknown origin. Diagnosis is made by eliminating infection, malignancy, and rheumatological diseases. In this report, case of a 5-year-old patient with symptoms of intermittent fever, areas of rash on the body, itching, and swelling, redness, and pain in the right and left ankle is described. Serological test results were negative for infectious agents, and malignancy was excluded. Patient was diagnosed with systemic JIA associated with intermittent fever, negative rheumatological markers and negative serology test results. Treatment with methylprednisolone and methotrexate yielded positive clinical response. Diagnosis of systemic JIA can be challenging, and must be made by eliminating other diseases.

  6. Idiopathic CD4+ lymphocytopenia and juvenile laryngeal papillomatosis.

    PubMed

    Pasic, Srdjan; Minic, Predrag; Dzudovic, Slobodan; Minic, Aleksandra; Slavkovic, Bojana

    2005-03-01

    We report on an association of idiopathic CD4+ lymphocytopenia (ICL) and juvenile laryngeal papillomatosis (JLP) in a pediatric-aged patient. Because of a past medical history of recurrent lung infections and severe chickenpox in infancy, immunologic investigations were done at age 6 years. On several occasions, a CD4+lymphocyte count of <300 cells/mm3 was detected, supporting the diagnosis of ICL. During follow-up, both medical (interferon-alpha) and surgical treatments of JLP were only partially efficient. Our patient developed disseminated infection with Mycobacterium avium and died at 10 years of age. Human papillomavirus is an important pathogen in pediatric and adult patients with ICL. In pediatric patients with JLP who develop other unusually severe viral or opportunistic infections, immunological investigations should be considered.

  7. Clinical patterns of juvenile idiopathic arthritis in Zambia

    PubMed Central

    2013-01-01

    Background Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders with different disease manifestations among various populations. There are few reports of JIA among indigenous Africans especially sub-Saharan Africa. We present herein the clinical patterns of JIA encountered at a tertiary hospital in Lusaka, Zambia. Method Hospital records of patients with a diagnosis of chronic arthritis with onset at the age of 16 years or less presenting to University Teaching Hospital, Lusaka, Zambia for the periods 1994–98 and 2006–2010 were retrospectively reviewed and reclassified as Juvenile Idiopathic Arthritis (JIA) based on the International League of Associations for Rheumatology (ILA R) JIA diagnostic criteria. Results In total, 126 patients with chronic arthritis of onset at age 16 years or less were evaluated over these periods at the hospital. Of these, 85 could further be analyzed by ILAR JIA criteria but 7 (8.24%) were HIV seropositive and were assessed separately. The average age at disease onset among the 78 JIA patients was 8.70 years (range: 1–15 years) with average age at first visit to hospital being 11.3 years (range: 2 to 25 years) and with a female to male ratio of 1.2:1. Polyarticular rheumatoid factor negative JIA, at 34.62%, was the most frequent type of chronic arthritis encountered. Oligoarthritis was found in 32.05% while 11.54% and 14.10% were polyarticular rheumatoid factor positive and systemic JIA, respectively. Enthesitis-related arthritis was found in 6.41% and only 1.28% were determined to have psoriatic arthritis among this population. Conclusion JIA is predominantly a polyarticular rheumatoid factor negative disease in Zambia. Late presentation is an issue with major implications for educational input and resource acquisition. There is need to elucidate the genetics and environmental factors of JIA in this region. PMID:24034206

  8. 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

  9. Optimisation of disease assessments in juvenile idiopathic arthritis.

    PubMed

    Consolaro, A; Schiappapietra, B; Dalprà, S; Calandra, S; Martini, A; Ravelli, A

    2014-01-01

    A variety of clinical measures are available for assessment of disease status of children with juvenile idiopathic arthritis (JIA) in clinical trials, clinical care and long-term outcome surveys. The American College of Rheumatology (ACR) Pediatric 30 remains the preferred primary outcome measure for registrative trials, although in most therapeutic studies performed in the 2000s patients were also evaluated for more stringent levels of improvement, that is, applying the ACR Pediatric 50, 70, 90, and 100 response criteria. Because the recent therapeutic advances have made inactive disease an achievable goal in most patients, it has been suggested that endpoints for future clinical trials incorporate the evaluation of disease activity state, namely the assessment of inactive disease and low disease activity. The introduction of the Juvenile Arthritis Disease Activity Score (JADAS) and the establishment of its cut-offs for various disease activity states may foster the implementation of the treat-to-target strategy in both clinical trials and routine practice. In recent years, there has been an increased focus on the inclusion of patient and child perspectives in health outcome measures through the use of parent/child-reported outcomes. Integration of these measures in the clinical evaluation is considered important as they reflect the parent's and child's perception of the disease course and effectiveness of therapeutic interventions. Future studies will show whether the newer imaging modalities, namely magnetic resonance imaging and ultrasound, can replace conventional radiography for the assessment of structural joint damage and its progression.

  10. Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies

    PubMed Central

    Schwartz, Thomas; Diederichsen, Louise Pyndt; Lundberg, Ingrid E; Sanner, Helga

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention. The risk of developing atherosclerotic coronary artery disease is increased twofold to fourfold in DM/PM. New and improved diagnostic methods have in recent studies in PM/DM and JDM demonstrated a high prevalence of subclinical cardiac involvement, especially diastolic dysfunction. Interactions between proinflammatory cytokines and traditional risk factors might contribute to the pathogenesis of cardiac dysfunction. Heart involvement could also be related to myocarditis and/or myocardial fibrosis, leading to arrhythmias and congestive heart failure, demonstrated both in adult and juvenile IIM. Also, reduced heart rate variability (a known risk factor for cardiac morbidity and mortality) has been shown in long-standing JDM. Until more information is available, patients with IIM should follow the same recommendations for cardiovascular risk stratification and prevention as for the corresponding general population, but be aware that statins might worsen muscle symptoms mimicking myositis relapse. On the basis of recent studies, we recommend a low threshold for cardiac workup and follow-up in patients with IIM. PMID:27752355

  11. Osteochondral lesions in children with juvenile idiopathic arthritis.

    PubMed

    Kröger, Liisa; Piippo-Savolainen, Eija; Tyrväinen, Erja; Penttilä, Pekko; Kröger, Heikki

    2013-05-01

    Joint pain and swelling are typical symptoms in children with juvenile idiopathic arthritis (JIA) and these are often related to inflammation of the joint. Juvenile osteochondritis dissecans (JOCD), that is separation of a bone-cartilage segment from the articular surface, can manifest with similar symptoms. We studied thirteen cases of osteochondritis dissecans lesions (OCD) in children with JIA. There were nine girls and four boys with a mean age of 6.5 (2-12) years at the time of diagnosis of JIA. Mean time between diagnosis of JIA and manifestation of OCD was 5.5 (1-11) years. Indications for MRI were the presence of pain or discomfort in the joint, despite otherwise effective treatment, with no evidence from ultrasound examination of any obvious signs of active inflammation. The most common location of osteochondral lesion was the knee, although the ankle joint was affected in one case. Five patients had lesions in both knees. Operative treatment was needed in eight cases (joints). Pain, and minor dysfunction of the joint are common complaints of children suffering from JIA. Earlier research has discounted the possibility of children who were not athletes presenting with this condition. However, this study demonstrates that these lesions also seem to be relatively common in patients with JIA. When there is no sign of inflammation, the possibility of OCD must therefore be considered in these children.

  12. Why we need a process on breaking news of Juvenile Idiopathic Arthritis: a mixed methods study.

    PubMed

    Chausset, Aurélie; Gominon, Anne-Laure; Montmaneix, Nathalie; Echaubard, Stéphane; Guillaume-Czitrom, Séverine; Cambon, Benoit; Miele, Cécile; Rochette, Emmanuelle; Merlin, Etienne

    2016-05-21

    Juvenile Idiopathic Arthritis is the most common chronic pediatric rheumatic disease. The announcement of Juvenile Idiopathic Arthritis poses for parents a number of challenges that make it hard to accept a diagnosis of the disease for their child; yet to our knowledge, no study to date has focused on the time period immediately surrounding the diagnosis. This study sets out to describe parents' experiences in engaging with their child's diagnosis of Juvenile Idiopathic Arthritis. This is a mixed methods study. Semi-structured interviews of families with a Juvenile Idiopathic Arthritis child were conducted. A grounded-theory thematic analysis was performed. Items that emerged in the interviews were compiled into a self-administered questionnaire. Eleven families participated in the qualitative study. Sixty families responded to the questionnaire. The path of parents was characterized by doubt (before, during and after diagnosis) while the disease tended to take center stage. Doubt was generated through mismatches in perspectives between the parents' circle of acquaintances, physicians, and the parents' own subjective experiences of symptoms. This study also found that social support and parent associations occupied an ambiguous position between help and stigmatization. Doubt fuels self-energizing spirals that take root as parents learn the news that their child has Juvenile Idiopathic Arthritis. These spirals of doubt may influence parents' experiences at every stage throughout the course of disease. Our data support the implementation of a specific process dedicated to breaking the news of Juvenile Idiopathic Arthritis to parents.

  13. Reduced proliferation and osteocalcin expression in osteoblasts of male idiopathic osteoporosis.

    PubMed

    Ruiz-Gaspà, Sílvia; Blanch-Rubió, Josep; Ciria-Recasens, Manuel; Monfort, Jordi; Tío, Laura; Garcia-Giralt, Natàlia; Nogués, Xavier; Monllau, Joan C; Carbonell-Abelló, Jordi; Pérez-Edo, Lluis

    2010-03-01

    Osteoporosis is characterized by low bone mineral density (BMD), resulting in increasing susceptibility to bone fractures. In men, it has been related to some diseases and toxic habits, but in some instances the cause of the primary--or idiopathic--osteoporosis is not apparent. In a previous study, our group compared histomorphometric measurements in cortical and cancellous bones from male idiopathic osteoporosis (MIO) patients to those of control subjects and found reduced bone formation without major differences in bone resorption. To confirm these results, this study analyzed the etiology of this pathology, examining the osteoblast behavior in vitro. We compared two parameters of osteoblast activity in MIO patients and controls: osteoblastic proliferation and gene expression of COL1A1 and osteocalcin, in basal conditions and with vitamin D(3) added. All these experiments were performed from a first-passage osteoblastic culture, obtained from osteoblasts that had migrated from the transiliac explants to the plate. The results suggested that the MIO osteoblast has a slower proliferation rate and decreased expression of genes related to matrix formation, probably due to a lesser or slower response to some stimulus. We concluded that, contrary to female osteoporosis, in which loss of BMD is predominantly due to increased resorption, low BMD in MIO seems to be due to an osteoblastic defect.

  14. Immunogenetics of juvenile idiopathic arthritis: A comprehensive review

    PubMed Central

    Hersh, Aimee O.; Prahalad, Sampath

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthropathy of childhood. Juvenile idiopathic arthritis is believed to be a complex genetic trait influenced by both genetic and environmental factors. Twin and family studies suggest a substantial role for genetic factors in the predisposition to JIA. Describing the genetics is complicated by the heterogeneity of JIA; the International League of Associations for Rheumatology (ILAR) has defined seven categories of JIA based on distinct clinical and laboratory features. Utilizing a variety of techniques including candidate gene studies, the use of genotyping arrays such as Immunochip, and genome wide association studies (GWAS), both human leukocyte antigen (HLA) and non-HLA susceptibility loci associated with JIA have been described. Several of these polymorphisms (e.g. HLA class II, PTPN22, STAT4) are shared with other common autoimmune conditions; other novel polymorphisms that have been identified may be unique to JIA. Associations with oligoarticular and RF-negative polyarticular JIA are the best characterized. A strong association between HLA DRB1:11:03/04 and DRB1:08:01, and a protective effect of DRB1:15:01 have been described. HLA DPB1:02:01 has also been associated with oligoarticular and RF-negative polyarticular JIA. Besides PTPN22, STAT4 and PTPN2 variants, IL2, IL2RA, IL2RB, as well as IL6 and IL6R loci also harbor variants associated with oligoarticular and RF-negative polyarticular JIA. RF-positive polyarticular JIA is associated with many of the shared epitope encoding HLA DRB1 alleles, as well as PTPN22, STAT4 and TNFAIP3 variants. ERA is associated with HLA B27. Most other associations between JIA categories and HLA or non-HLA variants need confirmation. The formation of International Consortia to ascertain and analyze large cohorts of JIA categories, validation of reported findings in independent cohorts, and functional studies will enhance our understanding of the genetic

  15. Immunogenetics of juvenile idiopathic arthritis: A comprehensive review.

    PubMed

    Hersh, Aimee O; Prahalad, Sampath

    2015-11-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthropathy of childhood. Juvenile idiopathic arthritis is believed to be a complex genetic trait influenced by both genetic and environmental factors. Twin and family studies suggest a substantial role for genetic factors in the predisposition to JIA. Describing the genetics is complicated by the heterogeneity of JIA; the International League of Associations for Rheumatology (ILAR) has defined seven categories of JIA based on distinct clinical and laboratory features. Utilizing a variety of techniques including candidate gene studies, the use of genotyping arrays such as Immunochip, and genome wide association studies (GWAS), both human leukocyte antigen (HLA) and non-HLA susceptibility loci associated with JIA have been described. Several of these polymorphisms (e.g. HLA class II, PTPN22, STAT4) are shared with other common autoimmune conditions; other novel polymorphisms that have been identified may be unique to JIA. Associations with oligoarticular and RF-negative polyarticular JIA are the best characterized. A strong association between HLA DRB1:11:03/04 and DRB1:08:01, and a protective effect of DRB1:15:01 have been described. HLA DPB1:02:01 has also been associated with oligoarticular and RF-negative polyarticular JIA. Besides PTPN22, STAT4 and PTPN2 variants, IL2, IL2RA, IL2RB, as well as IL6 and IL6R loci also harbor variants associated with oligoarticular and RF-negative polyarticular JIA. RF-positive polyarticular JIA is associated with many of the shared epitope encoding HLA DRB1 alleles, as well as PTPN22, STAT4 and TNFAIP3 variants. ERA is associated with HLA B27. Most other associations between JIA categories and HLA or non-HLA variants need confirmation. The formation of International Consortia to ascertain and analyze large cohorts of JIA categories, validation of reported findings in independent cohorts, and functional studies will enhance our understanding of the genetic

  16. 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.

  17. A comprehensive review of the genetics of juvenile idiopathic arthritis

    PubMed Central

    Prahalad, Sampath; Glass, David N

    2008-01-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic arthropathy of childhood which is believed to be influenced by both genetic and environmental factors. The progress in identifying genes underlying JIA susceptibility using candidate gene association studies has been slow. Several associations between JIA and variants in the genes encoding the human leukocyte antigens (HLA) have been confirmed and replicated in independent cohorts. However it is clear that genetic variants outside the HLA also influence susceptibility to JIA. While a large number of non-HLA candidate genes have been tested for associations, only a handful of reported associations such as PTPN22 have been validated. In this review we discuss the principles behind genetic studies of complex traits like JIA, and comprehensively catalogue non-HLA candidate-gene association studies performed in JIA to date and review several validated associations. Most candidate gene studies are underpowered and do not detect associations, and those that do are often not replicated. We also discuss the principles behind genome-wide association studies and discuss possible implications for identifying genes underlying JIA. Finally we discuss several genetic variants underlying multiple clinically distinct autoimmune phenotypes. PMID:18644131

  18. Autoimmune response to transthyretin in juvenile idiopathic arthritis

    PubMed Central

    Clement, Cristina C.; Lele, Aditi; Janow, Ginger; Becerra, Aniuska; Bauli, Francesco; Saad, Fawzy A.; Perino, Giorgio; Montagna, Cristina; Cobelli, Neil; Hardin, John; Stern, Lawrence J.; Ilowite, Norman; Porcelli, Steven A.

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatological condition. Although it has been proposed that JIA has an autoimmune component, the autoantigens are still unknown. Using biochemical and proteomic approaches, we identified the molecular chaperone transthyretin (TTR) as an antigenic target for B and T cell immune responses. TTR was eluted from IgG complexes and affinity purified from 3 JIA patients, and a statistically significant increase in TTR autoantibodies was observed in a group of 43 JIA patients. Three cryptic, HLA-DR1–restricted TTR peptides, which induced CD4+ T cell expansion and IFN-γ and TNF-α production in 3 out of 17 analyzed patients, were also identified. Misfolding, aggregation and oxidation of TTR, as observed in the synovial fluid of all JIA patients, enhanced its immunogenicity in HLA-DR1 transgenic mice. Our data point to TTR as an autoantigen potentially involved in the pathogenesis of JIA and to oxidation and aggregation as a mechanism facilitating TTR autoimmunity. PMID:26973882

  19. Genomic characterization of remission in juvenile idiopathic arthritis

    PubMed Central

    2013-01-01

    Introduction The attainment of remission has become an important end point for clinical trials in juvenile idiopathic arthritis (JIA), although we do not yet have a full understanding of what remission is at the cell and molecular level. Methods Two independent cohorts of patients with JIA and healthy child controls were studied. RNA was prepared separately from peripheral blood mononuclear cells (PBMC) and granulocytes to identify differentially expressed genes using whole genome microarrays. Expression profiling results for selected genes were confirmed by quantitative, real-time polymerase chain reaction (RT-PCR). Results We found that remission in JIA induced by either methotrexate (MTX) or MTX plus a TNF inhibitor (etanercept, Et) (MTX + Et) is characterized by numerous differences in gene expression in peripheral blood mononuclear cells and in granulocytes compared with healthy control children; that is, remission is not a restoration of immunologic normalcy. Network analysis of the differentially expressed genes demonstrated that the steroid hormone receptor superfamily member hepatocyte nuclear factor 4 alpha (HNF4α) is a hub in several of the gene networks that distinguished children with arthritis from controls. Confocal microscopy revealed that HNF4a is present in both T lymphocytes and granulocytes, suggesting a previously unsuspected role for this transcription factor in regulating leukocyte function and therapeutic response in JIA. Conclusions These findings provide a framework from which to understand therapeutic response in JIA and, furthermore, may be used to develop strategies to increase the frequency with which remission is achieved in adult forms of rheumatoid arthritis. PMID:24000795

  20. Altered signaling in systemic juvenile idiopathic arthritis monocytes

    PubMed Central

    Macaubas, Claudia; Wong, Elizabeth; Zhang, Yujuan; Nguyen, Khoa D.; Lee, Justin; Milojevic, Diana; Shenoi, Susan; Stevens, Anne M.; Ilowite, Norman; Saper, Vivian; Lee, Tzielan; Mellins, Elizabeth D.

    2016-01-01

    Systemic juvenile idiopathic arthritis (sJIA) is characterized by systemic inflammation and arthritis. Monocytes are implicated in sJIA pathogenesis, but their role in disease is unclear. The response of sJIA monocytes to IFN may be dysregulated. We examined intracellular signaling in response to IFN type I (IFNα) and type II (IFNγ) in monocytes during sJIA activity and quiescence, in 2 patient groups. Independent of disease activity, monocytes from Group 1 (collected between 2002-2009) showed defective STAT1 phosphorylation downstream of IFNs, and expressed higher transcript levels of SOCS1, an inhibitor of IFN signaling. In the Group 2 (collected between 2011-2014), monocytes of patients with recent disease onset were IFNγ hyporesponsive, but in treated, quiescent subjects, monocytes were hyperresponsive to IFNγ. Recent changes in medication in sJIA may alter the IFN hyporesponsiveness. Impaired IFN/pSTAT1 signaling is consistent with skewing of sJIA monocytes away from an M1 phenotype and may contribute to disease pathology. PMID:26747737

  1. Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

    PubMed Central

    Oray, Merih; Tuğal-Tutkun, İlknur

    2016-01-01

    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. PMID:27800265

  2. IL-1 Inhibition in Systemic Juvenile Idiopathic Arthritis

    PubMed Central

    Giancane, Gabriella; Minoia, Francesca; Davì, Sergio; Bracciolini, Giulia; Consolaro, Alessandro; Ravelli, Angelo

    2016-01-01

    Systemic juvenile idiopathic arthritis (sJIA) is the form of childhood arthritis whose treatment is most challenging. The demonstration of the prominent involvement of interleukin (IL)-1 in disease pathogenesis has provided the rationale for the treatment with biologic medications that antagonize this cytokine. The three IL-1 blockers that have been tested so far (anakinra, canakinumab, and rilonacept) have all been proven effective and safe, although only canakinumab is currently approved for use in sJIA. The studies on IL-1 inhibition in sJIA published in the past few years suggest that children with fewer affected joints, higher neutrophil count, younger age at disease onset, shorter disease duration, or, possibly, higher ferritin level may respond better to anti-IL-1 treatment. In addition, it has been postulated that use of IL-1 blockade as first-line therapy may take advantage of a “window of opportunity,” in which disease pathophysiology can be altered to prevent the occurrence of chronic arthritis. In this review, we analyze the published literature on IL-1 inhibitors in sJIA and discuss the rationale underlying the use of these medications, the results of therapeutic studies, and the controversial issues. PMID:27999545

  3. Laboratory Indicators of Aggrecan Turnover in Juvenile Idiopathic Arthritis

    PubMed Central

    Winsz-Szczotka, Katarzyna; Kuźnik-Trocha, Kornelia; Komosińska-Vassev, Katarzyna; Jura-Półtorak, Agnieszka; Olczyk, Krystyna

    2016-01-01

    Objectives. Evaluation of chondroitin sulfate (CS), as an early marker of aggrecan degradation, and chondroitin sulfate 846 epitope (CS846), as a biomarker of CS synthesis, is an attempt at answering the question whether the therapy used in juvenile idiopathic arthritis (JIA) patients contributes to the normalization of biochemical changes in aggrecan. Methods and Results. Serum levels of CS and CS846 as well as catalase (CT), superoxide dismutase (SOD), and glutathione peroxidase (GPx) activities in erythrocyte were assessed in patients before and after treatment. In the course of JIA, aggrecan metabolism is disturbed, which is reflected by a decrease (p < 0.001) in CS serum level and an increase (p < 0.05) in CS846 concentration. Furthermore, increased (p < 0.001) activities of CT, SOD, and GPx in untreated JIA patients were recorded. The anti-inflammatory treatment resulted in the normalization of CS846 level and SOD and GPx activities. In untreated patients, we have revealed a significant correlation between serum CS and CS846, CT, CRP, ESR, MMP-3, and ADAMTS-4, respectively, as well as between CS846 and CT, GPx, CRP, ESR, and TGF-β1, respectively. Conclusion. The observed changes of CS and CS846 in JIA patients indicate a further need of the therapy continuation aimed at protecting a patient from a possible disability. PMID:26924871

  4. Investigational drugs for treatment of juvenile idiopathic arthritis.

    PubMed

    Mauro, Angela; Rigante, Donato; Cimaz, Rolando

    2017-04-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. The improvement of knowledge about the pathogenetic mechanisms of JIA and advances in the understanding of pathways linking inflammation and autoimmunity and functions of multiple transcription factors have translated into new drug development for a tailored treatment directed to specific subpopulations of JIA patients. Areas covered: This review provides a digest of new investigational drugs which are currently or have been recently tested for treatment of JIA, and highlights some early phase clinical trials on rilonacept, givinostat, daclizumab, tofacitinib, and sarilumab. Expert opinion: Several studies have been focused on multiple complementary pathways driving synovial inflammation in JIA or molecules implicated in the inflammatory signature of JIA to deliver durable effects and prevent long-term complications. Since JIA is a complex disorder with multiple faces, identifying new treatment options for patients nonresponsive to the current drug armamentarium is of great relevance. A number of agents have been developed in the very last years, such as givinostat and tofacitinib, showing promising results in some cases, but trials remain in an early phase and few agents are currently under evaluation in a further phase setting. Longer-term use in possibly high numbers of patients and adequate data collection using large-scale registries are necessary to confirm clinical efficacy and provide a well-balanced overview of safety issues related to the drugs presented in this review.

  5. Report - Recurrent hip arthritis diagnosed as juvenile idiopathic arthritis: A case report.

    PubMed

    Chang, Tung-Ming; Yang, Kuender D; Yong, Su-Boon

    2016-05-01

    Juvenile idiopathic arthritis is the most common rheumatic disease in childhood. It is a chronic inflammatory disease associated with arthritis of unknown etiology that begins before the age of 16 and persists for longer than 6 weeks. In this report, the case of a child who suffered recurrent alternative hip arthritis with bilateral hip arthritis is examined, in which he was finally diagnosed as suffering from Juvenile idiopathic arthritis. A 14-year-old boy of Taiwanese origin presented with a normal birth and developmental history. At the age of 10, right-side hip joint pain was experienced, which later migrated to the left side. On further inspection, synovium hypertrophy, cartilage erosion and hip turbid fluid accumulation were found and aseptic arthritis was presumed to be the primary cause. However, after re-examining both his clinical history and presentation, Juvenile idiopathic arthritis was the final diagnosis. Any child presenting with repeat joint swelling are at risk of Juvenile idiopathic arthritis. This is still to be the case if symptoms recede or heal and no initial diagnosis is made. Therefore, a better understanding of the risk of recurrent arthritis is needed. It cannot be emphasized strongly enough that Juvenile idiopathic arthritis should be suspected at all times when a child suffers from recurrent aseptic arthritis of the hip joint.

  6. Incidence and prevalence of juvenile idiopathic arthritis in Catalonia (Spain).

    PubMed

    Modesto, C; Antón, J; Rodriguez, B; Bou, R; Arnal, C; Ros, J; Tena, X; Rodrigo, C; Rotés, I; Hermosilla, E; Barceló, P

    2010-11-01

    To ascertain the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Catalonia (autonomous region in northeast Spain), examined according to the currently established disease subtypes. Before initiating the study, we conducted an educational programme on paediatric rheumatology, addressed to all general paediatricians in Catalonia. A 2-year (2004-2006), prospective, population-based study was then carried out to determine the incidence of JIA. Prospective and retrospective data retrieval was performed to calculate prevalence. The International League of Associations for Rheumatology (ILAR, Edmonton revision) classification criteria were applied. Over the study period, 145 new cases of JIA were diagnosed. The mean annual incidence was 6.9/10⁵ children aged less than 16 years (range 5.8-8.1 years; 9.0 years for girls and 4.8 years for boys). On separate analysis of patients ≤ 6 and > 6 years, the distribution in younger children was found to be similar for both girls and boys, whereas in older children, most girls belonged to the oligoarthritis and polyarthritis subgroups, and boys to the enthesitis-related arthritis and undifferentiated subgroups. The calculated prevalence of JIA (31 October 2006) was 39.7 (36.1-43.7)/10⁵ children younger than 16. The relative risk of girls having JIA was 2.1 [95% confidence interval (CI) 1.7-2.7, p < 0.001]. In 70% of patients, the diagnosis was established before the age of 7. Subgroup distribution of prevalent cases mirrored that of incident cases. This is the first population-based study on the epidemiology of JIA in Catalonia. Incidence and prevalence rates are lower than those reported for several areas in Nordic countries of Europe. Oligoarthritis was the most common subtype.

  7. 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

  8. Quantifying Temporomandibular Joint Synovitis in Children With Juvenile Idiopathic Arthritis

    PubMed Central

    Vakilian, Pouya M.; Breen, Micheál; Zurakowski, David; Caruso, Paul; Henderson, Lauren; Nigrovic, Peter A.; Kaban, Leonard B.; Peacock, Zachary S.

    2016-01-01

    Objective Juvenile idiopathic arthritis (JIA) frequently affects the temporomandibular joints (TMJs) and is often undetected by history, examination, and plain imaging. Qualitative assessment of gadolinium‐enhanced magnetic resonance images (MRIs) is currently the standard for diagnosis of TMJ synovitis associated with JIA. The purpose of this study is to apply a quantitative analysis of synovial enhancement to MRIs of patients with and without JIA to establish a disease threshold and sensitivity and specificity for the technique. Methods This is a retrospective case–control study of children (age ≤16 years) who had MRIs with gadolinium including the TMJs. Subjects were divided into a JIA group and a control group. From a coronal T1‐weighted image, a ratio (enhancement ratio [ER]) of the average pixel intensity within three 0.2‐mm2 regions of interest (ROIs) in the TMJ synovium to that of a 50‐mm2 ROI of the longus capitis muscle was calculated. Receiver operating characteristic curves were used to determine the sensitivity and specificity. The inter‐ and intraexaminer reliability was evaluated with Bland‐Altman plots and 2‐way mixed, absolute agreement intraclass correlation coefficients. Results There were 187 and 142 TMJs included in the JIA and control groups, respectively. An ER threshold of 1.55 had a sensitivity and specificity for detecting synovitis of 91% and 96%, respectively. The inter‐ and intraexaminer reliability was excellent. Conclusion Calculating a ratio of pixel intensity between the TMJ synovium and the longus capitis muscle is a reliable way to quantify synovial enhancement. An ER of 1.55 differentiates normal TMJs from those affected by inflammatory arthritis. PMID:27110936

  9. Imaging of the temporomandibular joint in juvenile idiopathic arthritis.

    PubMed

    Vaid, Yoginder N; Dunnavant, F Daniel; Royal, Stuart A; Beukelman, Timothy; Stoll, Matthew L; Cron, Randy Q

    2014-01-01

    Temporomandibular joint (TMJ) arthritis in children with juvenile idiopathic arthritis (JIA) is extremely common but frequently asymptomatic. Magnetic resonance imaging (MRI) with contrast remains the gold standard for identifying TMJ arthritis in JIA. A reliable scoring system with published MRI examples of typical acute and chronic TMJ arthritis changes will be invaluable for future prospective treatment trials of TMJ arthritis in JIA. MRIs were collected from routine clinical studies assessing TMJ arthritis in JIA. Representative images were selected for publication to depict acute (synovial fluid, bone marrow edema, and synovial enhancement) and chronic (pannus, disc derangement, and condylar head flattening and erosions) TMJ arthritis findings. A preliminary MRI-based scoring system for assessing degrees of acute and chronic TMJ arthritis was developed and tested for inter- and intrareader reliability. TMJ MRIs representative of acute and chronic TMJ arthritis in JIA were selected from among thousands taken (>500 TMJ MRI studies annually at Children's of Alabama) since September 2007. Moreover, computed tomography scans depicting select bony changes (osteophyte formation, micrognathia) were chosen for publication. A description of the MRI protocol for assessing TMJ arthritis is included. A preliminary scoring system weighted for degree of acute and chronic TMJ arthritis MRI findings was found to have substantial inter- and intrareader reliability. A published set of MRIs depicting representative acute and chronic changes will help establish a standardized scoring system to assess TMJ arthritis in children with JIA. Future validation will aid in assessing improvement during treatment trials of TMJ arthritis. Copyright © 2014 by the American College of Rheumatology.

  10. Comorbidity of type 1 diabetes and juvenile idiopathic arthritis.

    PubMed

    Hermann, Gerhard; Thon, Angelika; Mönkemöller, Kirsten; Lilienthal, Eggert; Klinkert, Christof; Holder, Martin; Hörtenhuber, Thomas; Vogel-Gerlicher, Petra; Haberland, Holger; Schebek, Martin; Holl, Reinhard W

    2015-04-01

    To analyze the prevalence of juvenile idiopathic arthritis (JIA) and diabetes end points in pediatric patients with type 1 diabetes. Patients with type 1 diabetes, recorded from 1995 up to September 2013 in the Diabetes Patienten Verlaufsdokumentation database (n = 54,911, <16 years of age, 47% girls), were analyzed. The patients' height, weight, and body mass index SDS, glycosylated hemoglobin A1c (HbA1c); insulin dose; hypertension and dyslipidemia prevalence; rate of hypoglycemic events; and ketoacidosis were compared between patients with and without JIA. To adjust for age, sex, diabetes duration, and migration background, data were analyzed in hierarchic multivariable regression models. The prevalence of JIA in type 1 diabetes was 106 of 54,911 patients; 66% were girls. Diabetes onset was earlier in children with JIA (7.2 years vs 8.3 years, P = .04). Children with JIA were smaller (SDS: -0.22 vs 0.09, P = .004). Correspondingly, weight SDS was lower in patients with JIA (-0.02 vs 0.22, P = .01). Body mass index SDS did not differ. HbA1c was marginally lower in children with JIA (63 mmol/mol [8.0%] vs 67 mmol/mol [8.3%], P = .06). Insulin requirement was greater in patients with JIA (1.03 vs 0.93 insulin units/weight/day, P = .003). Hypertension and dyslipidemia were comparable in both groups. The JIA-prevalence in patients with type 1 diabetes (0.19%) was considerably greater than in the general population (0.05%). Growth is influenced negatively by JIA. Surprisingly, HbA1c was somewhat lower in children with JIA, possibly because of a more intensive treatment or a latent hemolysis caused by the inflammation. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Sibling Exposure and Risk of Juvenile Idiopathic Arthritis.

    PubMed

    Miller, Jessica; Ponsonby, Anne-Louise; Pezic, Angela; Kemp, Andrew; Piper, Susan E; Akikusa, Jonathan D; Allen, Roger C; Munro, Jane E; Ellis, Justine A

    2015-07-01

    Susceptibility to juvenile idiopathic arthritis (JIA) is presumed to be determined by both genes and environment. However, the environmental factors remain largely unknown. The hygiene hypothesis suggests that exposure to siblings, as a marker of exposure to microbes in early life, may protect against the development of later immune disorders. Some prior evidence suggests this may also be true for JIA. The present study was undertaken to test this hypothesis in detail. We conducted a comprehensive analysis of the role of sibling exposure in JIA risk within the Childhood Arthritis Risk Factor Identification Study JIA case-hospital control sample (302 cases and 676 controls) from Victoria, Australia. We found that, compared to being an only child, having any siblings was protective against JIA, with an adjusted odds ratio (OR) of 0.46 (95% confidence interval [95% CI] 0.28-0.74) (P = 0.001). The protective association appeared to increase with increasing number of siblings (e.g., for ≥3 siblings, adjusted OR 0.25 [95% CI 0.13-0.48], P < 0.001). A protective association of siblings was also observed when we considered cumulative sibling years by age 6 (e.g., for ≥3 years of exposure versus no exposure, adjusted OR 0.49 [95% CI 0.30-0.79], P = 0.003). We also compared cases to a second control sample (n = 341) collected from the community and weighted to represent the child population of Victoria. Data remained supportive of an association between sibling exposure and protection against JIA, particularly for exposure to younger siblings. Increased exposure to siblings is associated with a reduced risk of disease in our sample. This suggests that increased microbial exposure in childhood may confer protection against the development of JIA. © 2015, American College of Rheumatology.

  12. Adalimumab for juvenile idiopathic arthritis-associated uveitis.

    PubMed

    Magli, Adriano; Forte, Raimondo; Navarro, Pasqualina; Russo, Giustina; Orlando, Francesca; Latanza, Loredana; Alessio, Maria

    2013-06-01

    To assess the long-term outcomes and complications of patients with uveitis from juvenile idiopathic arthritis (JIA) treated with adalimumab. Prospective interventional case series. All patients who underwent treatment with adalimumab for JIA and anterior uveitis were prospectively included in the study. The anterior chamber inflammation was evaluated according to the Standardization of Uveitis Nomenclature criteria. Twenty-one patients (16 females, five males, 38 eyes) were included in the study. Mean age of patients at referral was 11.1 ± 3.8 (5-17) years. Before initiation of treatment, mean duration of arthritis was 7.0 ± 5.5 (median, 6) months, mean duration of uveitis was 7.0 ± 4.4 (median, 7) months. Oligoarticular arthritis was present in 15 cases (71 %), polyarticular arthritis in six cases (28 %). After a mean follow-up of 18.2 ± 7.7 (9-41) months, resolution of anterior chamber inflammation was obtained in 29/38 eyes (76 %). The anterior uveitis flare rate during the 12 months prior to enrollment was 1.6 ± 0.4/year, and was reduced during adalimumab treatment to 0.7 ± 0.3/year (p<0.001). A significant decrease of the number of relapses/month was present after onset of treatment with adalimumab (0.18 ± 0.2 before versus 0.02 ± 0.1 after treatment onset, p<0.001). No significant correlation was found between relapse number and age, sex, type of JIA and doses of previous steroid treatment (p>0.05). Adalimumab showed to be effective and relatively safe for treatment of JIA-associated uveitis.

  13. Incidence of herpes zoster infections in juvenile idiopathic arthritis patients.

    PubMed

    Nimmrich, S; Horneff, G

    2015-03-01

    The risk of herpes zoster among patients with juvenile idiopathic arthritis (JIA) exposed to biologics has not been evaluated. We determined incidence rates of herpes zoster among children with JIA in correlation with medication at time of occurrence and total drug exposure. The German biologics register database was used to identify patients with herpes zoster. Crude infection rates and incidence ratios (IRR) were compared to published rates. Demographics and overall exposure and particular exposure time to corticosteroids, immunosuppressive drugs and biologics were analyzed. The JIA cohort included 3,042 patients with 5,557.9 person-years of follow-up; 1,628 have used corticosteroids, 2,930 methotrexate and 1,685 etanercept. In total, 17 herpes zoster events have been documented [6/1,000 patients (3.5-9.0); 3.1/1,000 patient-years (1.9-4.9)]. Thus, the incidence rate in JIA patients was higher than expected [IRR 2.9 (1.8-4.5), p < 0.001]. In all patients, the event resolved completely. There were two complications, one patient developed intercostal neuralgia, and one had a recurrent herpes zoster. Compared to the healthy population, a significant higher IRR is observed in JIA patients who received a monotherapy with etanercept or in combination with steroids and methotrexate, but not in JIA patients exposed to methotrexate without biologics. In comparison with our control group of patients treated with methotrexate, the IRR was higher for exposure to etanercept monotherapy and combination of etanercept and corticosteroids irrespective of methotrexate use. A generally higher incidence rate in JIA patients treated with etanercept was observed. No serious or refractory manifestations occurred.

  14. Juvenile Idiopathic Arthritis in the Era of International Cooperation

    PubMed Central

    Uziel, Yosef

    2017-01-01

    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

  15. Phenotypic characterization of juvenile idiopathic arthritis in African American children

    PubMed Central

    Fitzpatrick, Lauren; Broadaway, K Alaine; Ponder, Lori; Angeles-Han, Sheila T.; Jenkins, Kirsten; Rouster-Stevens, Kelly; Pelajo, Christina F.; Conneely, Karen; Epstein, Michael P; Lopez-Benitez, Jorge; Vogler, Larry B.; Prahalad, Sampath

    2016-01-01

    Objectives Juvenile idiopathic arthritis (JIA) affects children of all races. Prior studies suggest that phenotypic features of JIA in African American (AA) children differ from those of Non-Hispanic White (NHW) children. We evaluated the phenotypic differences at presentation between AA and NHW children enrolled in the CARRA Registry, and replicated the findings in a JIA cohort from a large center in South Eastern USA. Methods Children with JIA enrolled in the multi-center CARRA Registry and from Emory University comprised the study and replication cohorts. Phenotypic data on Non-Hispanic AA children were compared with NHW children with JIA using Chi-square, Fisher's exact and Wilcoxon rank sum tests. Results In all, 4177 NHW and 292 AA JIA cases from the CARRA Registry, and 212 NHW and 71 AA cases from Emory were analyzed. AA subjects more often had RF-positive polyarthritis in both CARRA (13.4% vs. 4.7%, p=5.3×10-7) and Emory (26.8% vs. 6.1%, p =1.1×10-5) cohorts. AA children had positive tests for RF and CCP more frequently, but oligoarticular or early onset ANA-positive JIA less frequently in both cohorts. AA children were older at onset in both cohorts and this difference persisted after excluding RF-positive polyarthritis in the CARRA Registry (median age 8.5 vs. 5.0 years; p =1.4×10-8). Conclusions Compared to NHW children, AA children with JIA are more likely to have RF/CCP positive polyarthritis, and are older at disease onset, and less likely to have oligoarticular or ANA-positive early onset JIA, suggesting that the JIA phenotype is different in African American children. PMID:26879356

  16. Phenotypic Characterization of Juvenile Idiopathic Arthritis in African American Children.

    PubMed

    Fitzpatrick, Lauren; Broadaway, K Alaine; Ponder, Lori; Angeles-Han, Sheila T; Jenkins, Kirsten; Rouster-Stevens, Kelly; Pelajo, Christina F; Conneely, Karen; Epstein, Michael P; Lopez-Benitez, Jorge; Vogler, Larry B; Prahalad, Sampath

    2016-04-01

    Juvenile idiopathic arthritis (JIA) affects children of all races. Prior studies suggest that phenotypic features of JIA in African American (AA) children differ from those of non-Hispanic white (NHW) children. We evaluated the phenotypic differences at presentation between AA and NHW children enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, and replicated the findings in a JIA cohort from a large center in the southeastern United States. Children with JIA enrolled in the multicenter CARRA Registry and from Emory University formed the study and replication cohorts. Phenotypic data on non-Hispanic AA children were compared with NHW children with JIA using the chi-square test, Fisher's exact test, and the Wilcoxon signed-rank test. In all, 4177 NHW and 292 AA JIA cases from the CARRA Registry and 212 NHW and 71 AA cases from Emory were analyzed. AA subjects more often had rheumatoid factor (RF)-positive polyarthritis in both the CARRA (13.4% vs 4.7%, p = 5.3 × 10(-7)) and the Emory (26.8% vs 6.1%, p = 1.1 × 10(-5)) cohorts. AA children had positive tests for RF and cyclic citrullinated peptide antibodies (CCP) more frequently, but oligoarticular or early onset antinuclear antibody (ANA)-positive JIA less frequently in both cohorts. AA children were older at onset in both cohorts and this difference persisted after excluding RF-positive polyarthritis in the CARRA Registry (median age 8.5 vs 5.0 yrs, p = 1.4 × 10(-8)). Compared with NHW children, AA children with JIA are more likely to have RF/CCP-positive polyarthritis, are older at disease onset, and less likely to have oligoarticular or ANA-positive, early-onset JIA, suggesting that the JIA phenotype is different in AA children.

  17. 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

  18. Treatment of juvenile idiopathic arthritis: a revolution in care

    PubMed Central

    2014-01-01

    A generation ago, children with arthritis faced a lifetime of pain and disability. Today, there are a multitude of treatment options, including a variety of biologics targeting key cytokines and other inflammatory mediators. While non-steroidal anti-inflammatory drugs and corticosteroids were once the mainstay of therapy, they are now largely used as bridge or adjunctive therapies. Among the conventional disease-modifying anti-rheumatic drugs, methotrexate remains first-line therapy for most children with juvenile idiopathic arthritis (JIA) due to its long track record of safety and effectiveness in the management of peripheral arthritis. Sulfasalazine and leflunomide may also have a secondary role. The tumor necrosis factor inhibitors (TNFi) have shown tremendous benefit in children with polyarticular JIA and likely in enthesitis-related arthritis and psoriatic JIA as well. There may be additional benefit in combining TNFi with methotrexate. Abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear. For the treatment of systemic JIA, while the TNFi are of less benefit, blockade of interleukin-1 or interleukin-6 is highly effective. Additionally, interleukin-1 blockade appears to be effective treatment of macrophage activation syndrome, one of the most dangerous complications of JIA; specifically, anakinra in combination with cyclosporine and corticosteroids may obviate the need for cytotoxic approaches. In contrast, methotrexate along with the TNFi and abatacept are effective agents for the management of uveitis, another complication of JIA. Overall, the biologics have demonstrated an impressive safety record in children with JIA, although children do need to be monitored for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of malignancy. Finally, there may be a window of opportunity

  19. Sleep Fragmentation and Biomarkers in Juvenile Idiopathic Arthritis.

    PubMed

    Ward, Teresa M; Yuwen, Weichao; Voss, Joachim; Foell, Dirk; Gohar, Faekah; Ringold, Sarah

    2016-05-01

    (1) To compare sleep (nighttime sleep duration and sleep efficiency) and sleep fragmentation (movement and fragmentation index), as measured by actigraphy, and symptoms (pain and fatigue) in 8- to 14-year-old children with polyarticular and extended oligoarticular juvenile idiopathic arthritis (JIA) and (2) to examine the associations between sleep fragmentation (movement and fragmentation index) and the calcium-binding protein biomarkers S100A12 and myeloid-related protein (MRP8/14). Participants included 40 children with extended oligoarticular (n = 15) or polyarticular (n = 25) JIA and their parents. Serum protein samples were obtained during routine rheumatology clinic visits. Children completed the PedsQL Multidimensional Fatigue Scale and daily pain and sleep diaries and wore actigraphy monitors for 9 consecutive days. Parents completed the Children's Sleep Habits Questionnaire (CSHQ). Of the 40 children, 68% scored above the CSHQ clinical cutoff score for sleep disturbances. Mean nighttime sleep duration was 7.5 hr, and mean sleep efficiency was 85.3%. Group differences were not found for nighttime sleep duration, sleep efficiency, movement and fragmentation index, or S100A12 and MRP8/14 protein concentrations. In a stepwise regression, medications, joint count, and movement and fragmentation index explained 21% of the variance in MRP8/14 concentration. Decreased nighttime sleep duration, poor sleep efficiency, and fragmented sleep were observed in our sample, regardless of JIA category. Sleep fragmentation was a significant predictor of MRP8/14 protein concentration. Additional research is needed to understand the interrelations among sleep fragmentation, effects of medication, and S100A12 and MRP8/14 protein biomarkers in JIA. © The Author(s) 2015.

  20. Impact of juvenile idiopathic arthritis on quality of life during transition period at the era of biotherapies.

    PubMed

    Wipff, Julien; Sparsa, Laetitia; Lohse, Anne; Quartier, Pierre; Kahan, Andre; Deslandre, Chantal Job

    2016-01-01

    Few studies have assessed Health-Related Quality of Life (HR-QoL) in adults following juvenile idiopathic arthritis, and none since the advent of biotherapies. The aim of our study is to assess the impact of juvenile idiopathic arthritis on quality of life in a large transitional cohort, evaluate which factors influence quality of life in juvenile idiopathic arthritis, and determine which questionnaire should be used in practice. All consecutive juvenile idiopathic arthritis patients followed during adulthood in a transitional care program were included. Demographical, clinical and biological data were collected. The following quality of life questionnaires were administered: SF36 and EuroQoL. Age- and sex-matched controls (without rheumatic disease) were included. One hundred and sixty-one juvenile idiopathic arthritis (120 women and 41 men) and 76 (51/25) controls were included. Out of 161, sixty-five (40%) were considered to be in remission. Juvenile idiopathic arthritis had a large impact on the physical scales of quality of life. Pain seemed to be the most important factor affecting quality of life in cases of juvenile idiopathic arthritis. No significant difference was found between sub-types of juvenile idiopathic arthritis. In this large transitional cohort of patients at the era of biotherapies, juvenile idiopathic arthritis has a larger effect on physical than mental scale of quality of life measures. Pain was the main factor influencing quality of life. Sub-types of juvenile idiopathic arthritis do not seem to influence quality of life. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  1. Mechanism of osteoporosis in adolescent idiopathic scoliosis: experimental scoliosis in pinealectomized chickens.

    PubMed

    Kono, Hitoshi; Machida, Masafumi; Saito, Masashi; Nishiwaki, Yuji; Kato, Hiroyuki; Hosogane, Naobumi; Chiba, Kazuhiro; Miyamoto, Takeshi; Matsumoto, Morio; Toyama, Yoshiaki

    2011-11-01

    To clarify the mechanism of osteoporosis in adolescent idiopathic scoliosis (AIS), we investigated radiological and histological changes in the cervical vertebrae of a chicken thoracic scoliosis model. Forty newly hatched broiler chicks were randomly divided into four equal groups: sham-operated chickens serving as control (CON), pinealectomized chickens (PNX), sham-operated (CON + MLT) and pinealectomized chickens (PNX + MLT) that received intraperitoneal administration of melatonin. Pinealectomy was performed at the age of 3 days, and the chickens were killed at 2 months of age. Postmortem X-rays were examined for the presence of scoliosis, and micro-computed tomography (micro-CT) images were taken to evaluate the microstructure of the cervical vertebrae. Histological specimens of the scanned cervical vertebra were prepared, and a midsagittal section was stained with hematoxylin and eosin and tartrate-resistant acid phosphatase to evaluate the numbers of osteoblasts and osteoclasts, respectively. Scoliosis developed at the thoracic spine in all chickens of the PNX and in two of the PNX + MLT group. Micro-CT data revealed that chickens in the PNX group had a greater degree of generalized osteoporosis compared with the other birds. The number of osteoblasts was significantly decreased in the PNX group, while no significant difference was observed among chickens in the numbers of osteoclasts. Our results suggest that melatonin deficiency reduces osteoblast proliferation and leads to the development of scoliosis and osteoporosis. The restoration of melatonin prevented the development of scoliosis and osteoporosis, indicating that melatonin levels may be crucial to the development of deformity and osteoporosis in AIS.

  2. 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

  3. 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.

  4. 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.

  5. Osteoporosis

    MedlinePlus

    ... Emergency Room? What Happens in the Operating Room? Osteoporosis KidsHealth > For Kids > Osteoporosis A A A What's ... you're in your mid-20s. What Is Osteoporosis? If someone has osteoporosis (say: oss-tee-oh- ...

  6. Osteoporosis

    MedlinePlus

    ... Too Tall or Too Short All About Puberty Osteoporosis KidsHealth > For Kids > Osteoporosis Print A A A ... you're in your mid-20s. What Is Osteoporosis? If someone has osteoporosis (say: oss-tee-oh- ...

  7. [Osteoporosis in children and adolescents].

    PubMed

    Marcelli, Christian

    2007-01-01

    There is currently no consensus definition of osteopenia and osteoporosis in children according to bone mineral density (BMD) values measured by dual energy x-ray absorptiometry (DXA); interpretation of BMD measures must take into account the child's weight and pubertal status. In children, primary forms of osteoporosis--juvenile idiopathic osteoporosis and osteogenesis imperfecta--are rare; on the other hand, the frequency of secondary osteoporosis is increasing. Fractures, especially of the forearm, are frequent in children. During the peak growth period, bone growth and mineralization are dissociated; in consequence temporary bone fragility promotes fractures. Several recent studies show that children with fractures have reduced BMD and that the occurrence of fractures in children may constitute a risk factor for osteoporosis and fracture during adulthood. In cases of secondary osteoporosis, close monitoring of the causal disease is the key element of treatment; there are very few controlled studies of the prevention or treatment of osteoporosis in children.

  8. 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 ...

  9. Imaging of juvenile idiopathic arthritis. Part I: Clinical classifications and radiographs

    PubMed Central

    Matuszewska, Genowefa; Gietka, Piotr; Płaza, Mateusz; Walentowska-Janowicz, Marta

    2016-01-01

    Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals at the developmental age. Radiography is the primary modality employed in the diagnostic imaging in order to identify changes typical of this disease entity and rule out other bone-related pathologies, such as neoplasms, posttraumatic changes, developmental defects and other forms of arthritis. The standard procedure involves the performance of comparative joint radiographs in two planes. Radiographic changes in juvenile idiopathic arthritis are detected in later stages of the disease. Bone structures are assessed in the first place. Radiographs can also indirectly indicate the presence of soft tissue inflammation (i.e. in joint cavities, sheaths and bursae) based on swelling and increased density of the soft tissue as well as dislocation of fat folds. Signs of articular cartilage defects are also seen in radiographs indirectly – based on joint space width changes. The first part of the publication presents the classification of juvenile idiopathic arthritis and discusses its radiographic images. The authors list the affected joints as well as explain the spectrum and specificity of radiographic signs resulting from inflammatory changes overlapping with those caused by the maturation of the skeletal system. Moreover, certain dilemmas associated with the monitoring of the disease are reviewed. The second part of the publication will explain issues associated with ultrasonography and magnetic resonance imaging, which are more and more commonly applied in juvenile idiopathic arthritis for early detection of pathological features as well as the disease complications. PMID:27679726

  10. Effects of physical training on bone mineral density in fertile women with idiopathic osteoporosis.

    PubMed

    Bergström, Ingrid; Brinck, Jonas; Sääf, Maria

    2008-08-01

    The aim of this study was to investigate whether moderate physical training can improve the bone mineral density (BMD) in women with idiopathic osteoporosis. Ten pre-menopausal women aged 24-44 years diagnosed with idiopathic osteoporosis were included in the study. The physical training program consisted of three fast 30-min walks plus one or two sessions of 1-h training per week during 1 year at a training centre separate from the hospital. All patients were given supplements of vitamin D and calcium. Bone mineral density was measured in the femoral neck area and the lumbar spine by dual energy X-ray absorptiometry. The measurements were performed at baseline and after 12 months of training and compared with the measurements at the time of diagnosis, 1-3 years before the study. Eight women fulfilled the 12-month training period, and their mean (SD) BMD at start was 0.88 (0.08) g/cm(2) in the spine and 0.76 (0.13) g/cm(2) in the femoral neck. The mean spine BMD increase was 0.031 g/cm(2) (3.5%) after 1 year of training, which was significant (Wilcoxon's non-parametric test, p = 0.018). The mean increment in BMD in the femoral neck was insignificant, 0.007 g/cm(2) (0.9%) after the intervention (p = 0.74). However, the bone loss during the 1- to 3-year period from diagnosis to study start was, on average, 0.045 g/cm(2) or 5.0% in the femoral neck (p = 0.042), thus indicating a positive indirect effect of the intervention. There is no evidence-based therapy for women with idiopathic osteoporosis. It is therefore of importance to elucidate the impact of moderate physical activity in this group of patients. A 1-year training program was sufficient to induce a small but significant change in the spine BMD.

  11. IL-6 blockers in systemic onset juvenile idiopathic arthritis.

    PubMed

    Barone, Patrizia; Pignataro, Rossana; Garozzo, Maria Teresa; Leonardi, Salvatore

    2016-01-01

    IL-6 has a key role in the pathogenesis, clinical manifestations and activity of Systemic Onset Idiopathic Arthritis (sJIA). Tocilizumab (TCZ), the first humanized antihuman IL-6 receptor antibody, inhibits the activity of IL-6. In this review, we summarize the main studies performed, to date, about the use of TCZ in children affected by sJIA refractory to conventional treatment. Nowadays TCZ can be used, alone or in association with Metotrexate, in children older than 2 years. Its use in children younger than 2 years is being investigated. Further study about its use in sJIA and other type of idiopathic arthritis should be done.

  12. Osteoporosis in juvenile-onset diabetes mellitus: morphometric and comparative studies.

    PubMed

    Soejima, K; Landing, B H

    1986-01-01

    Ribs and vertebrae of 8 children and young adults aged from 17 months to 24 years with juvenile-onset diabetes mellitus, 4 with diabetes secondary to cystic fibrosis and 2 with diabetes secondary to thalassemia major, were analyzed for osteoporosis by a point-count morphometric method. The mean ratio of bone spicule to marrow space in cancellous bone of ribs of patients with juvenile-onset diabetes mellitus or with diabetes mellitus secondary to cystic fibrosis or thalassemia was 55% that of 10 control patients. The lengths of the zones of proliferating and mature cartilage cells in costal epiphyses of patients with juvenile-onset diabetes mellitus were also below normal. The ratio of bone spicule to marrow space of vertebrae of the diabetic patients was not significantly different from control values. The data confirm clinical reports that osteoporosis is a regular feature of juvenile-onset diabetes mellitus and suggest that the degree of bone matrix and mineral deficiency in such patients is greater than is usually considered.

  13. How frequent is osteogenesis imperfecta in patients with idiopathic osteoporosis?: Case reports.

    PubMed

    Al Kaissi, Ali; Windpassinger, Christian; Chehida, Farid Ben; Ghachem, Maher Ben; Nassib, Nabil M; Kenis, Vladimir; Melchenko, Eugene; Morenko, Ekatrina; Ryabykh, Sergey; Hofstaetter, Jochen G; Grill, Franz; Ganger, Rudolf; Kircher, Susanne Gerit

    2017-09-01

    The term idiopathic osteoporosis itself is quite a non-specific disease label, which fails to address the etiological understanding. Bone mineral density alone is not a reliable parameter to detect patients at high risk of fracture. The diversity of the clinical phenotypes of discolored teeth, blueness of the sclera, back and joint pain, cardiovascular disease, Diabetes type II, hearing problems and a long list of orthopedic problems are have to be considered. Our study has been designed in accordance with the clinical and radiological phenotype of eleven index cases with the provisional diagnosis of OI, which was followed by genotypic confirmation. This was followed by the invitation of siblings, parents, grandparents and other relatives to participate in the interviews, and to discuss the impact of the diagnosis. Proper collaboration with these families facilitated the process to identify other subjects with a history of fractures and other deformities/disabilities which were seemingly correlated to heritable connective tissue disorder. In total, 63 patients (27 children and 36 parents/grandparents and relatives) were enrolled in the study. Two groups of children were not included in our study. We excluded children with incomplete documentation and children who manifested de novo mutation. The term idiopathic osteoporosis (IOP) has been given to these families in other Institutes and was considered as a definite diagnosis. IOP was solely based on T scores, BMD and certain laboratory tests. Surprisingly, no single adult patient underwent clinical and or radiological phenotypic characterization. A constellation of significant disease associations with osteoporotic fracture risk have been encountered. The index cases showed mutations in COL1A1 (17q21.31.q22) and COL1A2 (7q22.1), the genes encoding collagen type I. The phenotype/genotype confirmation in 11 children was the key factor to boost our research and to re-consult each family. Comprehensive clinical and

  14. Macrophage activation syndrome in a patient with systemic onset of the juvenile idiopathic arthritis.

    PubMed

    Jain, Deepak; Aggarwal, Hari K; Rao, Avinash; Mittal, Anshul; Jain, Promil

    2016-01-01

    Systemic onset juvenile idiopathic arthritis (sJIA) is defined as arthritis affecting one or more joint usually in the juvenile age group (< 16 years of age) with or preceded by fever of at least 2 weeks duration that is documented to be daily ("quotidian") for at least 3 days which may be associated with evanescent (non-fixed) erythematous rash or generalized lymph node enlargement or hepatomegaly/splenomegaly/both or serositis. Macrophage activation syndrome (MAS) is a life-threatening complication of sJIA marked by sudden onset of non-remitting high fever, profound depression in all three blood cell lines (i.e. leukopenia, anemia, and thrombocytopenia), hepatosplenomegaly, lymphadenopathy, and elevated serum liver enzyme levels. In children with systemic juvenile idiopathic arthritis, the clinical picture may mimic sepsis or an exacerbation of the underlying disease. We report a case of a 16-year-old female patient presenting with high grade fever with joint pains and generalized weakness which proved to be systemic onset juvenile idiopathic arthritis with macrophage activation syndrome after ruling out all other differential diagnoses and responded well to intravenous steroids.

  15. Profiling risk factors for chronic uveitis in juvenile idiopathic arthritis: a new model for EHR-based research

    PubMed Central

    2013-01-01

    Background Juvenile idiopathic arthritis is the most common rheumatic disease in children. Chronic uveitis is a common and serious comorbid condition of juvenile idiopathic arthritis, with insidious presentation and potential to cause blindness. Knowledge of clinical associations will improve risk stratification. Based on clinical observation, we hypothesized that allergic conditions are associated with chronic uveitis in juvenile idiopathic arthritis patients. Methods This study is a retrospective cohort study using Stanford’s clinical data warehouse containing data from Lucile Packard Children’s Hospital from 2000–2011 to analyze patient characteristics associated with chronic uveitis in a large juvenile idiopathic arthritis cohort. Clinical notes in patients under 16 years of age were processed via a validated text analytics pipeline. Bivariate-associated variables were used in a multivariate logistic regression adjusted for age, gender, and race. Previously reported associations were evaluated to validate our methods. The main outcome measure was presence of terms indicating allergy or allergy medications use overrepresented in juvenile idiopathic arthritis patients with chronic uveitis. Residual text features were then used in unsupervised hierarchical clustering to compare clinical text similarity between patients with and without uveitis. Results Previously reported associations with uveitis in juvenile idiopathic arthritis patients (earlier age at arthritis diagnosis, oligoarticular-onset disease, antinuclear antibody status, history of psoriasis) were reproduced in our study. Use of allergy medications and terms describing allergic conditions were independently associated with chronic uveitis. The association with allergy drugs when adjusted for known associations remained significant (OR 2.54, 95% CI 1.22–5.4). Conclusions This study shows the potential of using a validated text analytics pipeline on clinical data warehouses to examine practice

  16. Growth retardation and delayed puberty in children and adolescents with juvenile idiopathic arthritis.

    PubMed

    Umławska, Wioleta; Prusek-Dudkiewicz, Anna

    2010-03-01

    Juvenile idiopathic arthritis (JIA) is the most common joint disorder in developing children. Juvenile idiopathic arthritis is difficult to diagnose and treat. In some patients, signs and symptoms can be frustratingly inconsistent, contradictory or idiosyncratic. Short stature in patients with JIA is usually due to reduced growth in the lower extremities, and only rarely due to reduced growth in the spinal column. In some studies, children with JIA were found to have infantile body proportions. Puberty is delayed in children with JIA. In children with chronic arthritic disorders, there is a strong correlation between the activity of the disease and the age of puberty. The main goals in reducing growth retardation in children with JIA are promoting timely remission and reducing the duration and dosage of corticosteroid treatment. It is important to regularly monitor physical development. Further improvements to the treatment protocol depend on continued interdisciplinary research involving paediatricians, rheumatologists and clinical anthropologists.

  17. 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 ...

  18. 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 ...

  19. Osteoporosis

    SciTech Connect

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

    1988-01-01

    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.

  20. 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

  1. 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.

  2. Complicated uveitis in late onset juvenile idiopathic psoriatic arthritis.

    PubMed

    Bravo Ljubetic, L; Peralta Calvo, J; Larrañaga Fragoso, P

    2016-04-01

    A 6 year-old girl with juvenile psoriatic arthritis (JPsA) and bilateral complicated anterior uveitis developed several ocular complications that required 5 surgical procedures. Despite the aggressive course of ocular inflammation, her visual acuity remained good. Arthritis (main criterion for the diagnosis of JPsA) appeared years after ocular involvement. She showed a good anti-tumour necrosis factor initial response. The definitive diagnosis of JPsA was established years after the onset of symptoms. In addition, the patient maintained a good visual acuity, despite its complicated disease course. Finally, she showed a good clinical response to adalimumab. Copyright © 2015 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  3. An intra-articular ganglion cyst in a patient with juvenile idiopathic arthritis.

    PubMed

    Deng, Donna Y; Yee, Keolamau; Burkhalter, William; Okimoto, Kelley Chinen; Kon, Kevin; Kurahara, David K

    2014-01-01

    We report an intra-articular ganglion cyst (IAGC) presenting as knee pain and a mass in a patient with longstanding Juvenile Idiopathic Arthritis (JIA). We could not find a similar case of an IAGC occurring in the knee of JIA patients in the literature. IAGC may need to be included as a possibility in patients with inflammatory arthritis with new-onset knee pain, especially in those with a palpable mass. MRI was useful in distinguishing IAGC from more worrisome causes of a knee mass. Orthopedic input was helpful in diagnosis and treatment. In addition, methotrexate therapy was effective in bringing about a long-lasting remission.

  4. X-linked agammaglobulinemia combined with juvenile idiopathic arthritis and invasive Klebsiella pneumoniae polyarticular septic arthritis.

    PubMed

    Zhu, Zaihua; Kang, Yuli; Lin, Zhenlang; Huang, Yanjing; Lv, Huoyang; Li, Yasong

    2015-02-01

    X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease caused by mutations in the Bruton's tyrosine kinase (BTK) gene. XLA can also present in combination with juvenile idiopathic arthritis (JIA), the major chronic rheumatologic disease in children. We report herein the first known case of a juvenile patient diagnosed with XLA combined with JIA that later developed into invasive Klebsiella pneumoniae polyarticular septic polyarthritis. An additional comprehensive review of XLA combined with JIA and invasive K. pneumoniae septic arthritis is also presented. XLA was identified by the detection of BTK mutations while the diagnosis of JIA was established by clinical and laboratory assessments. Septic arthritis caused by invasive K. pneumoniae was confirmed by culturing of the synovia and gene detection of the isolates. Invasive K. pneumoniae infections can not only result in liver abscesses but also septic arthritis, although this is rare. XLA combined with JIA may contribute to invasive K. pneumoniae infection.

  5. Immunogenicity, safety and tolerability of a bivalent human papillomavirus vaccine in adolescents with juvenile idiopathic arthritis.

    PubMed

    Esposito, Susanna; Corona, Fabrizia; Barzon, Luisa; Cuoco, Federica; Squarzon, Laura; Marcati, Giorgia; Torcoletti, Marta; Gambino, Monia; Palù, Giorgio; Principi, Nicola

    2014-11-01

    To evaluate the immunogenicity, safety and tolerability of the bivalent HPV vaccine in female patients with juvenile idiopathic arthritis (JIA). Twenty-one patients with JIA aged 12-25 years and 21 healthy controls were enrolled and received three doses of the bivalent HPV vaccine. All of the subjects were seronegative at baseline and seroconverted after the scheduled doses. The JIA patients showed significantly lower HPV16 neutralising antibody titres than controls 1 month after the administration of the third dose (p < 0.05), whereas no significant difference was observed in HPV18 neutralising antibody titres. Local and systemic reactions were similarly frequent in the patients and controls, and there were no significant changes in 27-joint juvenile arthritis disease activity score or laboratory tests. The bivalent HPV vaccine is safe in patients with stable JIA and regardless of the use of medications the vaccine assures an adequate degree of protection for a certain time.

  6. Osteoporosis.

    PubMed

    Nieves, Jeri W; Mosner, Michelle; Silverstein, Shari

    2012-01-01

    Osteoporosis is a skeletal disorder characterized by compromised bone strength that predisposes a person to increased risk of fracture. Fractures have severe consequences, so fracture prevention is imperative. Risk factor assessment and bone density testing are important tools in the diagnosis of osteoporosis. Actions to promote strong bones include adequate intakes of calcium and vitamin D; being physically active; not smoking; and avoiding excessive alcohol use. There are several FDA-approved medications for the treatment of osteoporosis. The recent attention to osteonecrosis of the jaw (ONJ) and the association between dental and skeletal health speak to the importance of osteoporosis awareness for dental professionals.

  7. 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 BMDL1-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.

  8. 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

  9. Trabecular bone microstructure and local gene expression in iliac crest biopsies of men with idiopathic osteoporosis.

    PubMed

    Patsch, Janina M; Kohler, Thomas; Berzlanovich, Andrea; Muschitz, Christian; Bieglmayr, Christian; Roschger, Paul; Resch, Heinrich; Pietschmann, Peter

    2011-07-01

    Male idiopathic osteoporosis (MIO) is a metabolic bone disease that is characterized by low bone mass, microstructural alterations, and increased fracture risk in otherwise healthy men. Although the detailed pathophysiology of MIO has yet to be clarified, evidence increasingly suggests an osteoblastic defect as the underlying cause. In this study we tested the hypothesis that the expression profile of certain osteoblastic or osteoblast-related genes (ie, WNT10B, RUNX2, Osterix, Osteocalcin, SOST, RANKL, and OPG) is different in iliac crest biopsies of MIO patients when compared with healthy controls. Furthermore, we investigated the relation of local gene expression characteristics with histomorphometric, microstructural, and clinical features. Following written informed consent and diligent clinical patient characterization, iliac crest biopsies were performed in nine men. While RNA extraction, reverse-transcription, and real-time polymerase chain reactions (PCRs) were performed on one biopsy, a second biopsy of each patient was submitted for histomorphometry and micro-computed tomography (µCT). Age-matched bone samples from forensic autopsies served as controls. MIO patients displayed significantly reduced WNT10B, RUNX2, RANKL, and SOST expression. Performing µCT for the first time in MIO biopsies, we found significant decreases in trabecular number and connectivity density. Trabecular separation was increased significantly, but trabecular thickness was similar in both groups. Histomorphometry revealed decreased BV/TV and osteoid volume and fewer osteoclasts in MIO. By providing evidence for reduced local WNT10B, RUNX2, and RANKL gene expression and histomorphometric low turnover, our data support the osteoblast dysfunction model discussed for MIO. Further, MIO seems to lead to a different microstructural pathology than age-related bone loss.

  10. Osteoporosis

    MedlinePlus

    ... is at its highest level. After bone mass peaks, all adults start to lose some bone mass.Osteoporosis occurs if you lose too much bone or don't make enough bone to begin with.What are the risk factors for osteoporosis?The following things put you at ...

  11. [Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].

    PubMed

    Tavares, Anna Carolina Faria Moreira Gomes; Ferreira, Gilda Aparecida; Guimarães, Luciano Junqueira; Guimarães, Raquel Rosa; Santos, Flávia Patrícia Sena Teixeira

    2015-01-01

    Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.

  12. 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.

  13. 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.

  14. Intravoxel incoherent motion magnetic resonance imaging of the knee joint in children with juvenile idiopathic arthritis.

    PubMed

    Hilbert, Fabian; Holl-Wieden, Annette; Sauer, Alexander; Köstler, Herbert; Neubauer, Henning

    2017-05-01

    MRI of synovitis relies on use of a gadolinium-based contrast agent. Diffusion-weighted MRI (DWI) visualises thickened synovium but is of limited use in the presence of joint effusion. To investigate the feasibility and diagnostic accuracy of diffusion-weighted MRI with intravoxel incoherent motion (IVIM) for diagnosing synovitis in the knee joint of children with juvenile idiopathic arthritis. Twelve consecutive children with confirmed or suspected juvenile idiopathic arthritis (10 girls, median age 11 years) underwent MRI with contrast-enhanced T1-weighted imaging and DWI at 1.5 T. Read-out segmented multi-shot DWI was acquired at b values of 0 s/mm(2), 200 s/mm(2), 400 s/mm(2) and 800 s/mm(2). We calculated the IVIM parameters perfusion fraction (f) and tissue diffusion coefficient (D). Diffusion-weighted images at b=800 s/mm(2), f parameter maps and post-contrast T1-weighted images were retrospectively assessed by two independent readers for synovitis using the Juvenile Arthritis MRI Scoring system. Seven (58%) children showed synovial hypertrophy on contrast-enhanced imaging. Diagnostic ratings for synovitis on DWI and on f maps were fully consistent with contrast-enhanced imaging, the diagnostic reference. Two children had equivocal low-confidence assessments on DWI. Median f was 6.7±2.0% for synovitis, 2.1±1.2% for effusion, 5.0±1.0% for muscle and 10.6±5.7% for popliteal lymph nodes. Diagnostic confidence was higher based on f maps in three (25%) children and lower in one child (8%), as compared to DWI. DWI with IVIM reliably visualises synovitis of the knee joint. Perfusion fraction maps differentiate thickened synovium from joint effusion and hence increase diagnostic confidence.

  15. [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

    2017-01-01

    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.

  16. Osteoporosis.

    PubMed

    Nanes, Mark S; Kallen, Caleb B

    2014-01-01

    Osteoporotic fractures are common and result in extensive morbidity and mortality. It is possible to decrease the risk of fracture in postmenopausal, male, and glucocorticoid-induced osteoporosis with appropriate screening and treatment. The assessment of fracture risk, for which bone densitometry is only 1 component, should be the main focus of patient evaluation. Epidemiologically derived risk-assessment tools such as World Health Organization Fracture Risk Assessment Tool (FRAX) provide physicians with a way to determine the 10-year risk of osteoporotic fracture and effectively choose candidates for therapy. A number of potent skeletal antiresorptive and anabolic drugs have become available to treat osteoporosis and prevent up to 70% of fractures. Here, we provide a detailed update on clinical osteoporosis, the contribution of bone densitometry, and the approach to patients using risk assessment in the consideration of treatments. Progress in osteoporosis is an example of successful bench-to-bedside research benefitting populations worldwide.

  17. Osteoporosis

    MedlinePlus

    ... Prevention and Treatment The good news is that osteoporosis can often be prevented and treated. Healthy lifestyle choices such as proper diet, exercise, and treatment medications can help prevent further bone ...

  18. 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

  19. Validity of a three-variable Juvenile Arthritis Disease Activity Score in children with new-onset juvenile idiopathic arthritis

    PubMed Central

    Mcerlane, Flora; Beresford, Michael W; Baildam, Eileen M; Chieng, S E Alice; Davidson, Joyce E; Foster, Helen E; Gardner-Medwin, Janet; Lunt, Mark; Wedderburn, Lucy R; Thomson, Wendy; Hyrich, Kimme L

    2013-01-01

    Objectives To investigate the validity and feasibility of the Juvenile Arthritis Disease Activity Score (JADAS) in the routine clinical setting for all juvenile idiopathic arthritis (JIA) disease categories and explore whether exclusion of the erythrocyte sedimentation rate (ESR) from JADAS (the ‘JADAS3’) influences correlation with single markers of disease activity. Methods JADAS-71, JADAS-27 and JADAS-10 were determined at baseline for an inception cohort of children with JIA in the Childhood Arthritis Prospective Study. JADAS3-71, JADAS3-27 and JADAS3-10 were determined using an identical formula but with exclusion of ESR. Correlation of JADAS with JADAS3 and single measures of disease activity/severity were determined by category. Results Of 956 eligible children, sufficient data were available to calculate JADAS-71, JADAS-27 and JADAS-10 at baseline in 352 (37%) and JADAS3 in 551 (58%). The median (IQR) JADAS-71, JADAS-27 and JADAS-10 for all 352 children was 11 (5.9–18), 10.4 (5.7–17) and 11 (5.9–17.3), respectively. Median JADAS and JADAS3 varied significantly with the category (Kruskal–Wallis p=0.0001), with the highest values in children with polyarticular disease patterns. Correlation of JADAS and JADAS3 across all categories was excellent. Correlation of JADAS71 with single markers of disease activity/severity was good to moderate, with some variation across the categories. With the exception of ESR, correlation of JADAS3-71 was similar to correlation of JADAS-71 with the same indices. Conclusions This study is the first to apply JADAS to all categories of JIA in a routine clinical setting in the UK, adding further information about the feasibility and construct validity of JADAS. For the majority of categories, clinical applicability would be improved by exclusion of the ESR. PMID:23256951

  20. Idiopathic Inflammatory Myopathies: an Update on Classification and Treatment with Special Focus on Juvenile Forms.

    PubMed

    Pagnini, Ilaria; Vitale, Antonio; Selmi, Carlo; Cimaz, Rolando; Cantarini, Luca

    2017-02-01

    Juvenile inflammatory myopathies represent a heterogeneous group of rare and potentially fatal disorders of unknown aetiology, characterised by inflammation and proximal and symmetric muscle weakness. Beyond many similarities, specific clinical, laboratoristic and histopathologic features underlie different subsets with distinguishing demographic, prognostic and therapeutic peculiarities. Over time, several forms of inflammatory idiopathic myopathies have been described, including macrophagic myofascitis, immune-mediated necrozing myopathy and the spectrum of amyopathic dermatomyositis that include hypomyopathic dermatomyositis, inclusion body myositis and cancer-associated myositis occurring almost exclusively in adults. However, juvenile dermatomyositis is the most frequent in childhood, whereas polymyositis is relatively more frequent in adults. The aetiology is nowadays widely unclear; however, current theories contemplate a combination of environmental triggers, immune dysfunction and specific tissue responses involving muscle, skin and small vessels endothelium in genetically susceptible individuals. Myositis-specific autoantibodies, found almost exclusively in patients with myositis and myositis-associated autoantibodies, detectable both among patients with myositis and in subjects suffering from other autoimmune diseases, have an important clinical role because of their relation to specific clinical features, response to therapy and prognosis. The gold standard treatment for juvenile dermatomyositis is represented by corticosteroids, along with adjunctive steroid-sparing immunosuppressive therapies, which are used to counteract disease activity, prevent mortality, and reduce long-term disability. Further treatment approach such as biologic agents and autologous stem cell transplantation are emerging during the last years, in particular in patients difficult to treat and with poor prognosis. Therefore, a highly medical specialised approach is required for

  1. Imaging in juvenile idiopathic arthritis (JIA): an update with particular emphasis on MRI.

    PubMed

    Damasio, Maria Beatrice; de Horatio, L Tanturri; Boavida, P; Lambot-Juhan, K; Rosendahl, K; Tomà, P; Muller, Ls Ording

    2013-11-01

    Juvenile idiopathic arthritis (JIA) is a heterogeneous condition encompassing all forms of chronic arthritis of unknown origin and with onset before 16 years of age. During the last decade new, potent therapeutic agents have become available, underscoring the need for accurate monitoring of therapeutic response on both disease activity and structural damage to the joint. However, so far, treatment efficacy is based on clinical ground only, although clinical parameters are poor markers for disease activity and progression of structural damage. Not so for rheumatoid arthritis patients where the inclusion of radiographic assessment has been required by FDA to test the disease-modifying potential of new anti-rheumatic drugs. In imaging of children with JIA there has been a shift from traditional radiography towards newer techniques such as ultrasound and MRI, however without proper evaluation of their accuracy and validity. We here summarize present knowledge and discuss future challenges in imaging children with JIA.

  2. 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.

  3. [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.

  4. Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome - effective treatment with tocilizumab.

    PubMed

    Kwiatkowska, Małgorzata; Jednacz, Ewa; Rutkowska-Sak, Lidia

    2015-01-01

    A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5(th) year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  5. Prevention and Management of Cataracts in Children with Juvenile Idiopathic Arthritis–Associated Uveitis

    PubMed Central

    Yeh, Steven

    2013-01-01

    Juvenile idiopathic arthritis (JIA)-associated uveitis can be associated with vision-compromising complications such as cataracts, glaucoma, synechiae, and band keratopathy. Of these, cataracts are one of the most common sequelae of JIA-associated uveitis and can result in significant visual disability. Risk factors for cataracts include posterior synechiae and longstanding ocular inflammation. Prevention of cataract development is crucial through appropriate control of uveitis. However, not all preventive measures are successful, and further management consisting of medical and surgical techniques is often necessary. Various factors should be taken into consideration when deciding on cataract management, including timing of surgery and placement of an intraocular lens. Continued partnership between pediatric rheumatologists and pediatric ophthalmologists can help ensure favorable visual outcomes. PMID:22201032

  6. [Guidelines on biologic drugs for the treatment of children with juvenile idiopathic arthritis (JIA)].

    PubMed

    Bukovac, Lana Tambić; Vidović, Mandica; Lamot, Lovro; Perica, Marija; Harjacek, Miroslav

    2013-01-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children, and one of the major causes of short-term or long-term disability, and impairment of quality of life in childhood. Without early and adequate treatment the disease will progress and result with irreparable joint damage. The choice of therapy depends on the JIA subtype, disease activity index, prognostic factors, and prooven efficacy and probable side-effects of the drugs. The goal of modern JIA therapy is the achievement of complete disease remission, and not only the improvement of symptoms and temporarily inflammation control. The implementation of biologics significantly altered therapeutic approach to children with resistant JIA. We present Croatian guidelines on biologic drugs for the treatment of patients with JIA.

  7. Osteoporosis.

    PubMed

    Ensrud, Kristine E; Crandall, Carolyn J

    2017-08-01

    Osteoporosis is a common systemic skeletal disorder resulting in bone fragility and increased fracture risk. However, management of osteoporosis and fracture prevention strategies are often not addressed by primary care clinicians, even in older patients with recent fractures. Evidence-based screening strategies will improve identification of patients who are most likely to benefit from drug treatment to prevent fracture. In addition, careful consideration of when pharmacotherapy should be started and choice of medication and duration of treatment will maximize the benefits of fracture prevention while minimizing potential harms of long-term drug exposure.

  8. 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.

  9. [Osteoporosis].

    PubMed

    Pilipović, N

    2001-01-01

    Osteoporosis is a systemic skeletal disease characterized by low bone density and microarchitectural deterioration of bone tissue, leading to enhanced bone fracture risk. Due to its increasing prevalence (affects more than 10 percent of population) and consequences (bone fractures), osteoporosis is a growing medical, social and economic problem. Of particular importance is hip fracture, with increasing incidence and lethal outcome in 20 percent, and less than 33 percent of patients with complete recovery. For better prevention of such undesired consequences, accurate diagnosis in the early phase of bone loss is necessary. Bone loss is a silent process, without signs and symptoms of the disease, and so active screening of persons at risk is needed, particulary among the postmenopausal women with some of the recognized risk factors for the development of osteoporotic bone fracture. In such persons, for early detection of bone mass loss, diagnostic measurement of bone density should be done, preferably dual-energy x-ray absorptiometry (DEXA). Prevention of osteoporosis is the best approach to the problem, and it should be started in early childhood with general measures (diet with sufficient calcium intake, life style with physical exercise) in a involved national program. Treatment of osteoporosis lasts for many years and includes general measures and medication with osteodensitometric assessment over a two-year period.

  10. Juvenile Arthritis

    MedlinePlus

    ... A Patient / Caregiver Diseases & Conditions Juvenile Arthritis Juvenile Arthritis Fast Facts Arthritis in children is treatable. It ... as fevers or rash. What is juvenile idiopathic arthritis? Several types of arthritis, all involving chronic (long- ...

  11. 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

  12. Osteoporosis.

    PubMed

    Lane, J M; Russell, L; Khan, S N

    2000-03-01

    Osteoporosis is a disorder of decreased bone mass, microarchitectural deterioration, and fragility fractures. Osteoporosis is widespread and can affect people of all ethnic backgrounds and many older women and men. An essential element in preventing osteoporosis is the achievement of normal peak bone mass. Adequate nutrition, appropriate calcium and vitamin D intake, regular menstrual cycles and a well balanced exercise program of exercise are essential elements in achieving peak bone mass. At menopause women undergo accelerated bone loss. Thereafter, women and men gradually lose bone mass. A loss of one standard deviation give rise to an enhanced twofold risk of spine fractures or a 2.5 risk of hip fracture. Bone mass is determined by dual energy x-ray absorptiometry, quantitative computed tomography scan, and a peripheral ultrasound. Dual energy x-ray absorptiometry has outstanding precision (within 1% to 2%), and has the ability to show the efficacy of drug intervention. Peripheral measurements may identify osteoporosis but only have a 70% correlation with hip and spine bone mass. Dual energy x-ray absorptiometry determines bone mass in a patient but the bone collagen breakdown products (N-telopeptide crosslinks) establish the current rate of bone loss. Major risk factors leading to fragility fracture include low body weight, history of fracture, family history of osteoporosis, and smoking. All individuals should ingest adequate calcium and vitamin D, exercise, and prevent falls. Women with low bone mass, high urinary bone collagen breakdown products, and/or major risk factors should consider hormone replacement therapy or a selective estrogen receptor modulator (Evista), calcitonin and bisphosphonates (alendronate). These agents successfully increase bone mass and limit fracture risk. Men at risk for fragility fractures respond similarly as women to alendronate and calcitonin. Although vertebral compression fractures can occur spontaneously, hip fractures are

  13. Toward a treat-to-target approach in the management of juvenile idiopathic arthritis.

    PubMed

    Consolaro, Alessandro; Negro, Giorgia; Lanni, Stefano; Solari, Nicoletta; Martini, Alberto; Ravelli, Angelo

    2012-01-01

    Recent advances in the management of juvenile idiopathic arthritis (JIA) render disease remission an attainable goal in many, if not most, patients. This has led to suggestions that future treatment guidelines include an overriding goal to achieve clinical remission or, at least, minimal disease activity. Furthermore, implementation of treatment strategies aimed at achieving and maintaining tight disease control in standard paediatric rheumatology practice has been proposed. A compelling argument is available at this time to suggest that the incorporation of treat-to-target approach in the management of children with JIA may improve disease outcome. Recently, descriptions of disease states that represent suitable therapeutic targets, such as inactive disease, minimal disease activity, or parent- or child-acceptable symptom states, have been developed. In addition, criteria for these states based on the Juvenile Arthritis Disease Activity Score (JADAS) have been identified. Future studies will clarify whether the addition of an imaging assessment to the management of children with JIA will improve the prediction of clinical outcomes.

  14. Serum calreticulin as a novel biomarker of juvenile idiopathic arthritis disease activity

    PubMed Central

    Hashaad, Nashwa Ismail; Fawzy, Rasha Mohamed; Elazem, Abeer Ahmed Abo; Youssef, Mohamed Ibrahim

    2017-01-01

    Objective This study aimed to investigate the relations between calreticulin (CRT) serum level and both disease activity and severity parameters in juvenile idiopathic arthritis (JIA). Material and Methods In this study, 60 children with JIA and 50 age-and-sex-matched healthy subjects were enrolled. The assessment of the disease activity was done using juvenile arthritis disease activity score 27 (JADAS-27). The assessment of disease severity was done via gray-scale ultrasonography (US) and power Doppler US (PDUS). Enzyme-linked immunosorbent assay (ELISA) was used to assay the serum level of human CRT. Results The mean serum CRT levels in JIA patients was 8.6±1.2 ng/mL and showed a highly significant increase (p=0.001) as compared to the mean serum levels in the controls (5.02±0.77 ng/mL). There were statistically significant positive correlations between the serum CRT levels and disease duration, tender joint count, swollen joint count, visual analog scale, erythrocyte sedimentation rate, JADAS-27, C-reactive protein, rheumatoid factor titer, and ultrasonographic grading for synovitis and neovascularization. Conclusion Elevated serum CRT levels in JIA patients and its correlations with JIA disease activity and severity parameters signified that CRT might be used as a novel biomarker for disease activity and severity in JIA. PMID:28293448

  15. [Osteoporosis].

    PubMed

    Ziegler, R

    1994-09-20

    Liver cirrhosis may be accompanied by osteoporosis and, rarely, osteomalacia. Normal liver function is required for normal digestion and absorption of calcium-containing nutrients. The liver plays an important role for the metabolisation of vitamin D: the 25-hydroxylation takes place in the liver. However, the respective enzymatic capacity is not limited by liver diseases except for almost complete liver insufficiency. Therefore, true hypovitaminosis D only rarely plays a role in hepatic osteopenia, but direct toxic effects on bone forming cells (osteoblasts) are discussed: e.g. by bile salts. Coexisting hypogonadism leads to further bone loss. Patients with primary biliary cirrhosis in part present with osteoporosis and fractures. Bone histology reveals normal resorption, but decreased formation. Calcitropic hormones are generally normal. Chronic alcoholism induces the same histologic picture in bone, i.e. normal resorption and diminished formation. These changes are reversible after abstinence and as long as of cirrhosis has not yet developed. Patients undergoing liver transplantation due to end stage liver insufficiency including cirrhosis present with diminished bone mass before receiving a new liver, and they show further bone loss after the transplantation due to immunosuppressive treatment including glucocorticoids. There is no specific treatment of bone loss or osteoporosis due to liver cirrhosis. Preventive efforts should be devoted to the avoidance of suboptimal calcium and vitamin D supply, immobilization, and hypogonadism. Fluorides may increase bone mass after liver transplantation--perhaps they are also useful in liver cirrhosis. Antiresorption agents like calcitonins or bisphosphonates may be cautiously tried.

  16. [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.

  17. 12A. The Gut-Joint Connection: Treating Juvenile Idiopathic Arthritis and Intestinal Hyperpermeability With Acupuncture, Natural Products, and Diet

    PubMed Central

    Hohmann, Nicole; Walker, Charlotte

    2013-01-01

    Focus Areas: Integrative Approaches to Care, Pediatrics, Alleviating Pain Juvenile idiopathic arthritis (JIA) patients and their parents have a high propensity to use complementary therapies to relieve pain. Medications and exercise therapy are a cornerstone of conventional treatment with a substantial rate of inadequate pain relief and the potential of side effects from pharmaceuticals. This session will highlight the evidence behind treating intestinal permeability to reduce pain from JIA.

  18. Role of KCNQ2 and KCNQ3 genes in juvenile idiopathic epilepsy in Arabian foals.

    PubMed

    Lichter-Peled, Anat; Polani, Sagi; Stanyon, Roscoe; Rocchi, Mariano; Kahila Bar-Gal, Gila

    2013-04-01

    Juvenile idiopathic epilepsy (JIE) in Arabian foals resembles benign-familial neonatal convulsion (BFNC) syndrome, a rare idiopathic epilepsy of new-born humans. BFNC syndrome exhibits genetic heterogeneity, as has been hypothesised to occur in Arabian foals, and is known to be caused by mutations in the voltage-gated potassium channel subunit KCNQ2 and KCNQ3 genes. The close phenotypic characteristics of both Arabian foals and children suggest these epileptic syndromes are caused by the same genetic disorder. In horses, the KCNQ2 and KCNQ3 genes are located on the terminal region of chromosomes 22 and 9, respectively, essentially homologous to their location on chromosomes 20q13.3 and 8q24 in humans. Gene trees for the KCNQ2 and KCNQ3 genes between horses and other mammals, particularly humans and mice, were constructed and compared to widely accepted mammalian phylogenetic trees. The KCNQ2 gene tree exhibited close clustering between horses and humans, relative to horses and mice, in contrast to the evolutionary trees of other mammals. Distance values between the horse and human groups were lower as opposed to those found between the horse and mouse groups. The similarity between the horse and the human, especially for the KCNQ2 gene, where the majority of mutations causing BFNC have been found, supports the hypothesis of similar heritable and genetic patterns of the disease in both species and suggests that contrary to the classic mouse-model concept, humans may be a more suitable model for the study of JIE in Arabian foals.

  19. Sustained-release dexamethasone intravitreal implant in juvenile idiopathic arthritis-related uveitis.

    PubMed

    Pichi, Francesco; Nucci, Paolo; Baynes, Kimberly; Lowder, Careen Y; Srivastava, Sunil K

    2017-02-01

    The purpose of this study is to review the results of treatment of juvenile idiopathic arthritis-related uveitis with the use of intravitreal dexamethasone implant. Sixteen eyes with Juvenile idiopathic arthritis (JIA)-associated uveitis received intravitreal dexamethasone implant to treat recalcitrant anterior segment inflammation (43.7 %), chronic macular edema (6.2 %), or a combination of both (50 %). One month after injection, mean visual acuity had improvement to 39.6 ± 11 ETDRS letters (p < 0.001). Mean AC cells measure at 1 month was 0.79 and 0.75 at 3 months. One month after injection, there was a significant reduction of central retinal thickness (CRT) to 342.4 ± 79.3 µm (p < 0.01). One month after the second implant, 11 eyes (91.6 %) achieved improved activity of the anterior uveitis, and mean best-corrected visual acuity improved to 44.6 ± 8.1 ETDRS letters (p < 0.01). At 1 month after the second injection, 4/5 eyes had resolution of macular edema with CRT of 250.4 ± 13.7 µm (p < 0.01). Of the 16 eyes, 12 eyes received a second injection at mean of 7.5 ± 3.1 months after the first treatment, and 5 eyes received a third Ozurdex injection on average 7 ± 4.6 months after the second injection. Of the 16 eyes, five eyes were pseudophakic prior to injection. Of the remaining 11 eyes, 8 (73 %) developed worsening posterior subcapsular cataract at a mean of 7.3 ± 1.2 months after the first injection. After the first injection, only one eye required topical antiglaucoma therapy with maximum pressure of 25 mmHg. In patients with recalcitrant JIA-associated active uveitis, injection of sustained-release dexamethasone can achieve control of anterior inflammation and resolution of macular edema.

  20. Correlation analyses of clinical and molecular findings identify candidate biological pathways in systemic juvenile idiopathic arthritis.

    PubMed

    Ling, Xuefeng B; Macaubas, Claudia; Alexander, Heather C; Wen, Qiaojun; Chen, Edward; Peng, Sihua; Sun, Yue; Deshpande, Chetan; Pan, Kuang-Hung; Lin, Richard; Lih, Chih-Jian; Chang, Sheng-Yung P; Lee, Tzielan; Sandborg, Christy; Begovich, Ann B; Cohen, Stanley N; Mellins, Elizabeth D

    2012-10-23

    Clinicians have long appreciated the distinct phenotype of systemic juvenile idiopathic arthritis (SJIA) compared to polyarticular juvenile idiopathic arthritis (POLY). We hypothesized that gene expression profiles of peripheral blood mononuclear cells (PBMC) from children with each disease would reveal distinct biological pathways when analyzed for significant associations with elevations in two markers of JIA activity, erythrocyte sedimentation rate (ESR) and number of affected joints (joint count, JC). PBMC RNA from SJIA and POLY patients was profiled by kinetic PCR to analyze expression of 181 genes, selected for relevance to immune response pathways. Pearson correlation and Student's t-test analyses were performed to identify transcripts significantly associated with clinical parameters (ESR and JC) in SJIA or POLY samples. These transcripts were used to find related biological pathways. Combining Pearson and t-test analyses, we found 91 ESR-related and 92 JC-related genes in SJIA. For POLY, 20 ESR-related and 0 JC-related genes were found. Using Ingenuity Systems Pathways Analysis, we identified SJIA ESR-related and JC-related pathways. The two sets of pathways are strongly correlated. In contrast, there is a weaker correlation between SJIA and POLY ESR-related pathways. Notably, distinct biological processes were found to correlate with JC in samples from the earlier systemic plus arthritic phase (SAF) of SJIA compared to samples from the later arthritis-predominant phase (AF). Within the SJIA SAF group, IL-10 expression was related to JC, whereas lack of IL-4 appeared to characterize the chronic arthritis (AF) subgroup. The strong correlation between pathways implicated in elevations of both ESR and JC in SJIA argues that the systemic and arthritic components of the disease are related mechanistically. Inflammatory pathways in SJIA are distinct from those in POLY course JIA, consistent with differences in clinically appreciated target organs. The limited

  1. National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis.

    PubMed

    Constantin, T; Ponyi, A; Orbán, I; Molnár, K; Dérfalvi, B; Dicso, F; Kálovics, T; Müller, J; Garami, M; Sallai, A; Balogh, Z; Szalai, Z; Fekete, G; Dankó, K

    2006-05-01

    Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease

  2. Osteoporosis.

    PubMed

    Sambrook, Philip; Cooper, Cyrus

    2006-06-17

    Osteoporosis is a serious public health issue. The past 10 years have seen great advances in our understanding of its epidemiology, pathophysiology, and treatment, and further advances are rapidly being made. Clinical assessment will probably evolve from decisions mainly being made on the basis of bone densitometry, to use of algorithms of absolute fracture risk. Biochemical markers of bone turnover are also likely to become more widely used. Bisphosphonates will probably remain the mainstay of therapy, but improved understanding of the optimum amount of remodelling suppression and duration of therapy will be important. At the same time, other diagnostic and therapeutic approaches, including biological agents, are likely to become more widespread.

  3. Immunological characteristics and T-cell receptor clonal diversity in children with systemic juvenile idiopathic arthritis undergoing T-cell-depleted autologous stem cell transplantation.

    PubMed

    Wu, Qiong; Pesenacker, Anne M; Stansfield, Alka; King, Douglas; Barge, Dawn; Foster, Helen E; Abinun, Mario; Wedderburn, Lucy R

    2014-06-01

    Children with systemic Juvenile Idiopathic Arthritis (sJIA), the most severe subtype of JIA, are at risk from destructive polyarthritis and growth failure, and corticosteroids as part of conventional treatment can result in osteoporosis and growth delay. In children where there is failure or toxicity from drug therapies, disease has been successfully controlled by T-cell-depleted autologous stem cell transplantation (ASCT). At present, the immunological basis underlying remission after ASCT is unknown. Immune reconstitution of T cells, B cells, natural killer cells, natural killer T cells and monocytes, in parallel with T-cell receptor (TCR) diversity by analysis of the β variable region (TCRVb) complementarity determining region-3 (CDR3) using spectratyping and sequencing, were studied in five children with sJIA before and after ASCT. At time of follow up (mean 11.5 years), four patients remain in complete remission, while one child relapsed within 1 month of transplant. The CD8(+) TCRVb repertoire was highly oligoclonal early in immune reconstitution and re-emergence of pre-transplant TCRVb CDR3 dominant peaks was observed after transplant in certain TCRVb families. Further, re-emergence of pre-ASCT clonal sequences in addition to new sequences was identified after transplant. These results suggest that a chimeric TCR repertoire, comprising T-cell clones developed before and after transplant, can be associated with clinical remission from severe arthritis. © 2014 The Authors. Immunology published by John Wiley & Sons Ltd.

  4. Subclinical Cardiovascular System Changes in Obese Patients with Juvenile Idiopathic Arthritis

    PubMed Central

    Głowińska-Olszewska, Barbara; Bossowski, Artur; Dobreńko, Elżbieta; Hryniewicz, Andrzej; Konstantynowicz, Jerzy; Milewski, Robert; Łuczyński, Włodzimierz; Piotrowska-Jastrzębska, Janina; Kowal-Bielecka, Otylia

    2013-01-01

    Objective. We aimed to determine the prevalence of excess body mass in juvenile idiopathic arthritis (JIA) children and to investigate the influence of obesity into the early, subclinical changes in cardiovascular system in these patients. Methods. Fifty-eight JIA patients, aged median 13 years, were compared to 36 healthy controls. Traditional cardiovascular risk factors and inflammatory markers (hsCRP, IL-6, TNFα, adiponectin) were studied together with IMT (intima-media thickness), FMD (flow mediated dilation), and LVMi (left ventricle mass index) as surrogate markers of subclinical atherosclerosis. Results. Thirteen JIA children (22%) were obese and had increased systolic blood pressure, cholesterol, triglycerides, insulin, HOMA, hsCRP, and IL-6 compared to nonobese JIA and controls. FMD was decreased compared to nonobese JIA and controls, whereas IMT and LVMi were increased. In multivariate regression analysis, TNFα, SDS-BMI, and systolic blood pressure were independent predictors of early CV changes in JIA. Conclusions. Coincident obesity is common in JIA children and is associated with insulin resistance, dyslipidemia, and increased levels of inflammatory markers leading to early changes in cardiovascular system. Thus, medical care of children with JIA should include strategies preventing cardiovascular disease by maintenance of adequate body weight. PMID:23554546

  5. 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/

  6. 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

  7. 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.

  8. 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.

  9. Ureaplasma septic arthritis in an immunosuppressed patient with juvenile idiopathic arthritis.

    PubMed

    George, Michael David; Cardenas, Ana Maria; Birnbaum, Belinda K; Gluckman, Stephen J

    2015-06-01

    Mycoplasmas, including Ureaplasma and Mycoplasma species, are uncommon but important causes of septic arthritis, especially affecting immunosuppressed patients. Many of the reported cases have been associated with congenital immunodeficiency disorders, especially hypogammaglobulinemia. Mycoplasmas are difficult to grow in the laboratory, and these infections may be underdiagnosed using culture techniques. We report a case of a 21-year-old woman with juvenile idiopathic arthritis and hip arthroplasties treated with rituximab and adalimumab who developed urogenital infections and soft tissue abscesses followed by knee arthritis with negative routine cultures. Ureaplasma species was identified from synovial fluid on 2 separate occasions using a broad-range 16S ribosomal RNA gene polymerase chain reaction. Azithromycin led to rapid improvement in symptoms, but after completion of therapy, involvement of the hip prosthesis became apparent, and again, 16S rRNA gene polymerase chain reaction was positive for Ureaplasma species. The literature is reviewed with a discussion of risk factors for Mycoplasma septic arthritis, clinical presentation, methods of diagnosis, and treatment.

  10. Plantar- and dorsiflexor strength in prepubertal girls with juvenile idiopathic arthritis.

    PubMed

    Broström, Eva; Nordlund, Maria M; Cresswell, Andrew G

    2004-08-01

    To compare lower-leg strength of young girls with polyarticular juvenile idiopathic arthritis (JIA) with that of healthy, age-matched controls. Isometric and isokinetic strength tests of the plantar- and dorsiflexors. All strength measures were made at an ankle angle of 90 degrees. Isokinetic plantar- and dorsiflexor measures were made at 15 degrees/s during shortening (concentric) and lengthening (eccentric) actions. Strength testing laboratory. Ten prepubertal girls diagnosed with JIA and 10 healthy girls. Not applicable. Isometric and isokinetic plantar- and dorsiflexor strength. Isometric plantar- and dorsiflexion torques were significantly lower (48% and 38% respectively; P<.05) for the children with JIA than for the controls. The JIA group also produced lower shortening plantarflexion torques (52%, P<.05). Lengthening plantarflexor torques did not differ significantly between the 2 groups (P<.05). Controls were stronger than the JIA group for both shortening and lengthening maximal dorsiflexor actions (P<.05). All children were 4 to 5 times stronger in plantarflexion than in dorsiflexion. Girls with JIA had significantly less plantar- and dorsiflexor strength than age-matched, healthy peers. The reduced strength of children with JIA is likely to affect function in daily activities and probably contributes to reduced levels of physical activity.

  11. A Novel Method for Pathway Identification Based on Attractor and Crosstalk in Polyarticular Juvenile Idiopathic Arthritis

    PubMed Central

    Wang, Yuanji; Lin, Shunhua; Li, Changhui; Li, Yizhao; Chen, Lei; Wang, Yingzhen

    2016-01-01

    Background Juvenile idiopathic arthritis (JIA) is one of the most common inflammatory disorders of unknown etiology. We introduced a novel method to identify dysregulated pathways associated with polyarticular JIA (pJIA). Material/Methods Gene expression profiling of 61 children with pJIA and 59 healthy controls were collected from E-GEOD-13849; 300 pathways were obtained from Kyoto Encyclopedia of Genes and Genomes (KEGG) database and 787,896 protein-protein interaction sets were gathered from the Retrieval of Interacting Genes. Attractor and crosstalk were designed to complement each other to increase the integrity of pathways assessment. Then, impact factor was used to assess the interactions inter-pathways, and RP-value was used to evaluate the comprehensive influential ability of attractors. Results There were seven attractors with p<0.01 and 14 pathways with RP<0.01. Finally, two significantly dysfunctional pathways were found, which were related to pJIA progression: p53 signaling pathway (KEGG ID: 04115) and non-alcoholic fatty liver disease (NAFLD) (KEGG ID: 04932). Conclusions A novel approach that identified the dysregulated pathways in pJIA was constructed based on attractor and crosstalk. The new process is expected to be efficient in the upcoming era of medicine. PMID:27804927

  12. Differential cytokine profiles in juvenile idiopathic arthritis subtypes revealed by cluster analysis.

    PubMed

    van den Ham, Henk-Jan; de Jager, Wilco; Bijlsma, Johannes W J; Prakken, Berent J; de Boer, Rob J

    2009-08-01

    With the introduction of high-throughput biomarker measurements, traditional analysis of these markers is increasingly difficult. Using samples from a diverse group of patients, we tested the applicability of cluster analysis to these data. Using this method, we aim to visualize some of the patterns specific to certain disease groups. In particular, we focus on juvenile idiopathic arthritis (JIA), a multifactorial autoimmune disorder that ultimately leads to chronic inflammation of the joints. Cytokine measurements were performed using multiplex immunoassays. Using heuristic clustering methods, we set out to compare the pattern of 30 cytokines in plasma and SF of JIA, RA, OA, or diabetes type II patients and healthy controls. Analysis shows that oligo- and polyarticular JIA have similar biomarker profiles, both in plasma and SF. Systemic onset JIA (SoJIA) has a profile distinct from other JIA subtypes, suggesting that they involve different inflammatory processes. SoJIA samples do, however, cluster together with RA in SF, suggesting that these two conditions have similar cytokine profiles. Furthermore, we identify several clusters of ILs and chemokines that are co-expressed, suggesting that they are co-regulated. We show that previously undetected clusters of cytokines and patients can be identified by applying cluster analysis to multiplex data. Cytokine clusters identified in plasma and SF samples were quite different, which underscore the differential cytokine signalling in these two compartments, and suggest that plasma samples may not be suitable for estimating joint biomarker profiles and inflammation.

  13. Remission rate is not dependent on the presence of antinuclear antibodies in juvenile idiopathic arthritis.

    PubMed

    Glerup, M; Herlin, T; Twilt, M

    2017-03-01

    Recently, it has been hypothesized that the subcategories of the ILAR classification of juvenile idiopathic arthritis (JIA) are not homogeneous, and that the presence of antinuclear antibodies (ANA) should lead to a separate entity. Therefore, the aim of this study was to evaluate ANA positivity as a predictor of achieving remission. A retrospective single-center cohort study including all JIA patients diagnosed between January 2000 and May 2014. A minimum follow-up of 1 year was required plus the ANA status. ANA positivity was defined as at least two positive results with a titer ≥1:160. Demographic and clinical features were collected. Remission at last follow-up was defined by the Wallace criteria. A total of 625 patients met the inclusion criteria and 230 (37%) were found ANA positive. Analysis showed no difference in remission rate between ANA-positive and ANA-negative patients. Additionally, joint count at diagnosis and at last follow-up were comparable in both groups. ANA positivity was correlated to a female predominance and young age at diagnosis (p < 0.001). Remission rates are not different in ANA-positive patients than in ANA-negative patients. This does not support the hypothesis to possibly divide JIA patients based on their ANA status.

  14. Atypical Cutaneous Blastomycosis in a Child With Juvenile Idiopathic Arthritis on Infliximab.

    PubMed

    Smith, Robert J; Boos, Markus D; Burnham, Jon M; McKay, Eileen M; Kim, Jason; Jen, Melinda

    2015-11-01

    Blastomyces dermatitidis is a dimorphic fungus endemic to much of North America, particularly the soils of the midwestern and southeastern United States. Human infection typically occurs through inhalation of airborne conidia, which can be followed occasionally by dissemination to the skin, bone, genitourinary system, and central nervous system. A hallmark of the pathogen is that it can cause disease in both immunocompetent and immunosuppressed populations. Blastomycosis is rare in pediatric patients, with cutaneous manifestations occurring even less frequently. Here, we report the case of a 9-year-old boy on iatrogenic immunosuppression with infliximab and methotrexate for juvenile idiopathic arthritis who presented with a nonhealing, indurated plaque of his right ear with significant superficial yellow crusting in the absence of constitutional symptoms. After failing a prolonged course of topical and oral antibiotic therapy, biopsy and tissue culture revealed Blastomyces dermatitidis infection. The area cleared after treatment with oral fluconazole and withdrawal of infliximab. To our knowledge, this is the first report of a pediatric patient developing an infection with B dermatitidis after initiation of therapy with a tumor necrosis factor-α inhibitor. This case also highlights an unusual morphology of cutaneous blastomycosis in an iatrogenically immunosuppressed child. Copyright © 2015 by the American Academy of Pediatrics.

  15. Subclinical cardiovascular system changes in obese patients with juvenile idiopathic arthritis.

    PubMed

    Głowińska-Olszewska, Barbara; Bossowski, Artur; Dobreńko, Elżbieta; Hryniewicz, Andrzej; Konstantynowicz, Jerzy; Milewski, Robert; Łuczyński, Włodzimierz; Piotrowska-Jastrzębska, Janina; Kowal-Bielecka, Otylia

    2013-01-01

    We aimed to determine the prevalence of excess body mass in juvenile idiopathic arthritis (JIA) children and to investigate the influence of obesity into the early, subclinical changes in cardiovascular system in these patients. Fifty-eight JIA patients, aged median 13 years, were compared to 36 healthy controls. Traditional cardiovascular risk factors and inflammatory markers (hsCRP, IL-6, TNF α, adiponectin) were studied together with IMT (intima-media thickness), FMD (flow mediated dilation), and LVMi (left ventricle mass index) as surrogate markers of subclinical atherosclerosis. Thirteen JIA children (22%) were obese and had increased systolic blood pressure, cholesterol, triglycerides, insulin, HOMA, hsCRP, and IL-6 compared to nonobese JIA and controls. FMD was decreased compared to nonobese JIA and controls, whereas IMT and LVMi were increased. In multivariate regression analysis, TNF α, SDS-BMI, and systolic blood pressure were independent predictors of early CV changes in JIA. Coincident obesity is common in JIA children and is associated with insulin resistance, dyslipidemia, and increased levels of inflammatory markers leading to early changes in cardiovascular system. Thus, medical care of children with JIA should include strategies preventing cardiovascular disease by maintenance of adequate body weight.

  16. 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.

  17. Interleukin-18 for predicting the development of macrophage activation syndrome in systemic juvenile idiopathic arthritis.

    PubMed

    Shimizu, Masaki; Nakagishi, Yasuo; Inoue, Natsumi; Mizuta, Mao; Ko, Giyo; Saikawa, Yutaka; Kubota, Tomohiro; Yamasaki, Yuichi; Takei, Syuji; Yachie, Akihiro

    2015-10-01

    To assess the role of IL-6/IL-18 in the pathogenesis of systemic juvenile idiopathic arthritis (s-JIA) and to investigate the clinical significance of serum IL-18 levels for predicting macrophage activation syndrome (MAS) development, we measured the serum IL-6/IL-18 levels in 76s-JIA patients, including 15 with MAS, and compared them with the clinical features. We identified 2 distinct subsets on the basis of serum IL-6/IL-18 levels. The IL-18-dominant subset had more patients who developed MAS. Serum IL-18 levels during active phase in patients with MAS were significantly higher than those without MAS. The cutoff value of serum IL-18 levels for predicting MAS development was 47750 pg/ml. The patients with IL-18 dominant subset at their disease onset were significantly more likely to develop MAS after TCZ therapy started. IL-18 might have a key role in the pathogenesis of MAS. Serum IL-18 levels >47750 pg/ml might be useful to predict MAS development. Copyright © 2015 Elsevier Inc. All rights reserved.

  18. Gout in a 15-year-old boy with juvenile idiopathic arthritis: a case study

    PubMed Central

    2014-01-01

    Joint pain is a common complaint in pediatrics and is most often attributed to overuse or injury. In the face of persistent, severe, or recurrent symptoms, the differential typically expands to include bony or structural causes versus rheumatologic conditions. Rarely, a child has two distinct causes for joint pain. In this case, an obese 15-year-old male was diagnosed with gout, a disease common in adults but virtually ignored in the field of pediatrics. The presence of juvenile idiopathic arthritis (JIA) complicated and delayed the consideration of this second diagnosis. Indeed, the absence of gout from this patient’s differential diagnosis resulted in a greater than two-year delay in receiving treatment. The patients’ BMI was 47.4, and he was also mis-diagnosed with osteochondritis dissecans and underwent medical treatment for JIA, assorted imaging studies, and multiple surgical procedures before the key history of increased pain with red meat ingestion, noticed by the patient, and a subsequent elevated uric acid confirmed his ultimate diagnosis. With the increased prevalence of obesity in the adolescent population, the diagnosis of gout should be an important consideration in the differential diagnosis for an arthritic joint in an overweight patient, regardless of age. PMID:24393408

  19. 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

  20. 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

  1. Using the attract method to identify core pathways in juvenile idiopathic arthritis.

    PubMed

    Li, J S; Gao, X L; Liu, Y R; Dong, Y

    2016-08-12

    The aim of this study was to identify core pathways associated with juvenile idiopathic arthritis (JIA) using the attract method. Kyoto Encyclopedia of Genes and Genomes pathways were determined using the GSEA-ANOVA method, based on the gene expression data of JIA. Syn-expression groups within core attractor pathways were identified by hierarchical clustering. Correlated sets of genes exhibiting highly similar profiles to the syn-expression groups were identified and each correlated set was subjected to a gene ontology functional enrichment analysis to discover potentially shared biological themes. Based on a false-discovery rate < 0.05, we identified 11 significant pathways were identified as potential attractors. Flag genes or uninformative genes were removed and 5 discriminative pathways: the proteasome, ribosome, protein export, spliceosome, and Parkinson's disease pathways were identified. A final set of syn-expression groups with a consistent trend of relative expression of pathway-related genes was obtained; that is, the proteasome, ribosome, protein export, spliceosome, and Parkinson's disease pathways were composed of 2, 2, 1, 2, and 3 clusters, respectively. Genes in each correlated set shared common roles, and changes at the pathway level were more likely to be real. In light of these, the attract method was able to on expand important context to find distinguishing expression patterns within pathways. This paper predicted that the functional themes involved in protein synthesis (such as proteasome, ribosome, spliceosome) were closely related to the progression of JIA, which might contribute to the detection of therapy target for JIA.

  2. Gene Expression Deconvolution for Uncovering Molecular Signatures in Response to Therapy in Juvenile Idiopathic Arthritis

    PubMed Central

    Rosenberg, Alan M.; Yeung, Rae S. M.; Morris, Quaid

    2016-01-01

    Gene expression-based signatures help identify pathways relevant to diseases and treatments, but are challenging to construct when there is a diversity of disease mechanisms and treatments in patients with complex diseases. To overcome this challenge, we present a new application of an in silico gene expression deconvolution method, ISOpure-S1, and apply it to identify a common gene expression signature corresponding to response to treatment in 33 juvenile idiopathic arthritis (JIA) patients. Using pre- and post-treatment gene expression profiles only, we found a gene expression signature that significantly correlated with a reduction in the number of joints with active arthritis, a measure of clinical outcome (Spearman rho = 0.44, p = 0.040, Bonferroni correction). This signature may be associated with a decrease in T-cells, monocytes, neutrophils and platelets. The products of most differentially expressed genes include known biomarkers for JIA such as major histocompatibility complexes and interleukins, as well as novel biomarkers including α-defensins. This method is readily applicable to expression datasets of other complex diseases to uncover shared mechanistic patterns in heterogeneous samples. PMID:27244050

  3. Clinical predictors of temporomandibular joint arthritis in juvenile idiopathic arthritis: A systematic literature review.

    PubMed

    Kristensen, Kasper Dahl; Stoustrup, Peter; Küseler, Annelise; Pedersen, Thomas Klit; Twilt, Marika; Herlin, Troels

    2016-06-01

    To assess the level of evidence for subjective and objective parameters in clinical orofacial examination and determine if predictors for temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) patients exist in the current literature. A comprehensive systematic electronic search strategy was performed in all major medical databases in June 2015. Studies were selected independently by two reviewers in accordance with a prespecified protocol and a risk of bias assessment for all included studies. Subjective examination outcome measures assessed were pain, decreased TMJ function, and TMJ sounds. The objective outcome measures assessed were maximal incisor opening, mandibular asymmetric opening, condylar translation, protrusion, myofascial pain on palpation, facial asymmetry, and micro- or retrognathism. The electronic database search identified 345 unique citations. After application of our strict, predefined inclusion and exclusion criteria, 21 articles were included and data extracted. The study heterogeneity did not allow for meta-analyses. No singular outcome measure can be suggested as a predictor of TMJ involvement in JIA, as sensitivity and/or specificity is too low compared to contrast-enhanced magnetic resonance imaging. The current low level of evidence and study heterogeneity do not allow us to conclude on singular clinical outcome measures. To increase study comparability, we call for a standardized terminology and evidence-based guidelines for clinical orofacial examination parameters in JIA patients. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Perceived Oral Health and Care of Children with Juvenile Idiopathic Arthritis: A Qualitative Study.

    PubMed

    Leksell, Eva; Hallberg, Ulrika; Magnusson, Bo; Ernberg, Malin; Hedenberg-Magnusson, Britt

    2015-01-01

    To increase knowledge about how children diagnosed with juvenile idiopathic arthritis (JIA) perceive their oral health and dental care. Fifteen interviews with children diagnosed with JIA, aged 6 to 16 years, were analyzed according to classical grounded theory. The children's main concern about their oral health was identified as creating a positive identity after being diagnosed with JIA and learning to live with oral health problems. While attempting to cope with this concern, the children often endured in silence, the core category in the analysis. A variety of aspects were found of this core coping strategy, which were categorized as differentiating from the disease, working on personal caretaking and positive attitude, fighting fears and sadness, control of professional aid, and building supportive relationships. The results emphasize the importance for caregivers to show empathy and interest in the child as a person, to ask precise questions when taking case histories so the child does not remain silent, to provide psychosocial support and suggest positive coping strategies, to describe and administer treatments, and to give hope for the future. Awareness of the social interaction between a child diagnosed with JIA and health professionals as well as awareness of how to approach a child with longstanding illness are crucial for disclosing and treating the child's orofacial symptoms.

  5. Use of adalimumab in patients with juvenile idiopathic arthritis refractory to etanercept and/or infliximab.

    PubMed

    Katsicas, María M; Russo, Ricardo A G

    2009-08-01

    To analyse the effectiveness and safety of adalimumab in a group of patients with juvenile idiopathic arthritis (JIA) who had failed treatment with etanercept and/or infliximab in a single paediatric rheumatology clinic. Patients with JIA with active polyarthritis refractory to metotrexate (MTX) (> or =20 mg/m2/week) for at least 3 months and to etanercept (up to 1 mg/kg twice weekly) and/or infliximab (up to 10 mg/kg every 4 weeks) for at least 6 months were included. All patients received adalimumab 24 mg/m2/week concomitantly with MTX 7.5-10 mg/week. Evaluation of efficacy included improvement as defined by the ACR paediatric 30 criteria, 50% and 70% improvement and remission. Six patients were included. Three patients met improvement criteria; 50% and 70% improvement occurred in two children. Improvement was sustained for 12, 24 and 36 months, respectively. Remission occurred in one patient. Adalimumab was discontinued due to lack of efficacy in three patients. No side effects were observed. Adalimumab appears to be effective and safe in patients with JIA refractory to other anti-TNF agents. Further controlled studies are needed in order to assess efficacy of adalimumab in children with refractory JIA.

  6. Simultaneous juvenile idiopathic arthritis and diabetes mellitus type 1 -- a Finnish nationwide study.

    PubMed

    Pohjankoski, Heini; Kautiainen, Hannu; Korppi, Matti; Savolainen, Anneli

    2012-02-01

    To describe the occurrence and main clinical and laboratory findings of patients having both juvenile idiopathic arthritis (JIA) and diabetes mellitus type 1 (DM-1) in a period of 30 years. Eighty-two patients having simultaneous JIA and DM-1 were identified in the reimbursement registers of the Finnish National Institute of Insurance during the period 1976-2005. Data on their clinical histories were collected from patient files. Occurrence of simultaneous JIA and DM-1 increased 4.5-fold between the first (1976-85) and the last (1996-2005) decade. Prevalence of uveitis was 7%, of rheumatoid factor seropositivity 15%; 22% of patients had a third autoimmune disease [autoimmune disease (AID)], and 16% had serious psychiatric problems. The occurrence of patients with the 2 diseases, JIA and DM-1, increased over 3 decades. The prevalence of uveitis was low, the number of seropositive patients was high, and further cases of AID were frequent. Patients had multiple additional problems necessitating multiprofessional care.

  7. Apoptosis of peripheral blood lymphocytes in patients with juvenile idiopathic arthritis

    PubMed Central

    Smolewska, E; Brozik, H; Smolewski, P; Biernacka-Zielins..., M; Darzynkiewicz, Z; Stanczyk, J

    2003-01-01

    Objective: To evaluate different aspects of apoptosis in children with juvenile idiopathic arthritis (JIA). Methods: The frequency of TUNEL positive peripheral blood (PB) lymphocytes (apoptotic index (AI)), as well as serum CD95 (APO1/Fas) antigen expression and serum levels of sFas and interleukin 15 (IL15), were examined in 44 cases of JIA. Results were correlated with type of onset, activity of JIA, and acute phase indicators. Results: The AI of lymphocytes was significantly higher in patients with JIA than in controls (p=0.020). The mean AI of lymphocytes was increased in JIA with systemic type of onset and high activity (p=0.001). Moreover, IL15 levels in systemic disease were higher than in controls (p=0.012). An increased AI correlated with raised IL15 (p=0.046), erythrocyte sedimentation rate (p=0.005) and C reactive protein (CRP; p=0.017). Additionally, correlation was found between IL15 and CRP levels (p=0.039). CD95 and sFas levels were unchanged compared with controls. Conclusion: PB lymphocytes of children with JIA have an increased tendency to undergo apoptosis. The degree of apoptosis depends on the type of onset and activity of JIA and correlates with serum levels of IL15. Further studies are needed to explain whether this is an epiphenomenon of the disease activity or is related to the pathogenesis of JIA. PMID:12860732

  8. Gout in a 15-year-old boy with juvenile idiopathic arthritis: a case study.

    PubMed

    Morris, Hallie; Grant, Kristen; Khanna, Geetika; White, Andrew J

    2014-01-06

    Joint pain is a common complaint in pediatrics and is most often attributed to overuse or injury. In the face of persistent, severe, or recurrent symptoms, the differential typically expands to include bony or structural causes versus rheumatologic conditions. Rarely, a child has two distinct causes for joint pain. In this case, an obese 15-year-old male was diagnosed with gout, a disease common in adults but virtually ignored in the field of pediatrics. The presence of juvenile idiopathic arthritis (JIA) complicated and delayed the consideration of this second diagnosis. Indeed, the absence of gout from this patient's differential diagnosis resulted in a greater than two-year delay in receiving treatment. The patients' BMI was 47.4, and he was also mis-diagnosed with osteochondritis dissecans and underwent medical treatment for JIA, assorted imaging studies, and multiple surgical procedures before the key history of increased pain with red meat ingestion, noticed by the patient, and a subsequent elevated uric acid confirmed his ultimate diagnosis. With the increased prevalence of obesity in the adolescent population, the diagnosis of gout should be an important consideration in the differential diagnosis for an arthritic joint in an overweight patient, regardless of age.

  9. Children's experiences of living with juvenile idiopathic arthritis: a thematic synthesis of qualitative studies.

    PubMed

    Tong, Allison; Jones, Julie; Craig, Jonathan C; Singh-Grewal, Davinder

    2012-09-01

    To describe the experiences and perspectives of children and adolescents living with juvenile idiopathic arthritis (JIA). We conducted a systematic review of qualitative studies that explored the experiences of children living with JIA. We searched electronic databases (to week 2 of July 2011) and reference lists of relevant articles. Twenty-seven studies that reported the experiences of more than 542 participants were included. Six major themes were identified: aversion to being different (unrelenting and unpredictable pain, disablement, internal disfigurement, differential treatment, and forced dependency on others); striving for normality (preserving social identity, resourcefulness, sense of community, focus on remission, and mastery over body and pain); stigma and misunderstanding (trivialization of disease, invisible pain, and discrimination); suspension in uncertainty (control versus powerlessness, hope versus disappointment); managing treatment (benefits of taking medicines, respect and involvement in health care, and motivation for physical therapy); and desire for knowledge (medical treatment and advances, lifestyle management). JIA disrupts a child's sense of normality and impairs his or her capacity for social participation. Children with JIA have a sense of being misunderstood and stigmatized, and they feel perpetually caught between having hope and control over their bodies and overwhelming pain and despair. To increase their confidence, the ability to manage pain, and their resourcefulness for self-management, children need ongoing information about treatments and lifestyle management, strong social support, community advocacy, and active involvement in their own health decision making. Copyright © 2012 by the American College of Rheumatology.

  10. Anti-cyclic citrullinated peptide antibodies in children with Juvenile Idiopathic Arthritis.

    PubMed

    Hamooda, Mohamed; Fouad, Hala; Galal, Nermeen; Sewelam, Nadia; Megahed, Dina

    2016-09-01

    The purpose of present study was to access the prevalence of anti-cyclic citrullinated peptide (anti-CCP) antibodies in children with Juvenile Idiopathic Arthritis (JIA), and to investigate the clinical significance and diagnostic value of the anti-CCP antibodies in correlation with age, sex & activity. This case-control study was performed on 50 patients with JIA in addition to 40 sex and age-matched children as a control group. The participants were recruited from rheumatology Outpatient Clinic of Cairo University Specialized Pediatric Hospital. Patients were subjected to full history taking, clinical examination, routine laboratory investigations and x-rays on involved joints. Both patients and controls underwent assay of anti-CCP antibodies by AxSYM Anti-CCP IgG Microparticle Enzyme Immunoassay (MEIA) which is a semi-quantitative determination of the IgG class of autoantibodies specific to cyclic citrullinated peptide (CCP) in patients' serum or plasma. Data were analyzed using Mann-Whitney U test, ANOVA, and independent-samples t-test by SPSS version 15. Anti-CCP positivity was identified amongst patients with JIA, particularly those JIA patients experiencing RF positive polyarticular disease onset. Above all, it is important that anti-CCP positivity and bone erosions, degree of joint damage, and ESR levels were significantly correlated. Anti-CCP could be utilized as a valuable marker in the polyarticular form of JIA to direct early, and could be aggressive therapeutic intervention.

  11. Optimizing the Wingate Anaerobic Cycling Test for youth with juvenile idiopathic arthritis.

    PubMed

    Obeid, Joyce; Larché, Maggie J; Timmons, Brian W

    2011-08-01

    The Wingate Anaerobic Test (WAnT) can assess muscle function in youth with juvenile idiopathic arthritis (JIA). Our objective was to compare peak power (PP) and mean power (MP) when the WAnT is performed with a standard vs. an optimized braking force. Eight patients with JIA between the ages of 8 and 18 participated in two sessions. Optimal braking force was determined with a series of 15-s force-velocity tests performed against braking forces ranging from 3.5 to 8.5% of body weight. Participants then performed two randomized WAnTs against the standard (4.5%) and optimal braking forces. PP tended to be greater in the optimized vs. standard WAnT (12.5 ± 2.6 vs. 10.8 ± 1.0 W/kg, respectively; p = .07). No differences were observed for MP (standard: 6.2 ± 0.9 vs. optimized: 6.2 ± 1.1 W/kg; p = .9). Optimization of the WAnT tended to increase PP by 10-28% in youth with JIA.

  12. Evaluation of children with juvenile idiopathic arthritis in southeastern Turkey: a single center experience

    PubMed Central

    Şen, V; Ece, A; Uluca, Ü; Güneş, A; Yel, S; Tan, İ; Karabel, D; Yıldırım, B; Haspolat, K

    2015-01-01

    Background: The aim of this study was to investigate the disease characteristics of children with juvenile idiopathic arthritis (JIA) in southeast Turkey. Methods: The International League of Associations for Rheumatology (ILAR) criteria were used to diagnose JIA. Hospital records of the Pediatric Rheumatology Unit, of the Dicle University Hospital, were reviewed retrospectively and demographic, clinical and laboratory data were recorded. Results: Totally 213 children (103 boys, 110 girls), with an age range of 1.6-18 years were enrolled. The mean age of the disease onset was 8.1 years. Polyarticular type was the most common (42.3%) presentation. The frequencies of other JIA subtypes were as follows: oligoarticular 37.1%, systemic 8.9%, enthesitis-related arthritis (ERA) 10.8% and psoriatic arthritis 0.9%. The knees (74.2%) and ankles (54.0%) were the most commonly affected joints. Uveitis was found in 4.2% of patients. Anti-nuclear antibodies were positive in 11.7% and HLA-B27 in 2.8% of patients. Active disease was seen in 57 (26.7%) patients at the last visit. Conclusion: In the present study, polyarticular JIA was the predominant subtype and there were fewer patients with positive ANA or uveitis compared to previous studies. Hippokratia 2015, 19 (1): 63-68. PMID:26435650

  13. US findings of metacarpophalangeal joints in children with idiopathic juvenile arthritis.

    PubMed

    Karmazyn, Boaz; Bowyer, Suzanne L; Schmidt, Kara Murphy; Ballinger, Susan H; Buckwalter, Kenneth; Beam, Thuy T; Ying, Jun

    2007-05-01

    Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children, with frequent involvement of the metacarpophalangeal joints (MCPJ). To compare US findings with those of radiography and clinical examination. All MCPJs in 20 children with JIA (17 females, median age 9.7 years, range 3.6 to 16.8 years) were evaluated clinically and imaged with gray-scale and color Doppler US, and 90 MCPJs were also imaged radiographically. Each MCPJ was graded on physical examination from 0 (normal) to 4 (severe) by the patient's rheumatologist. US demonstrated abnormalities in 64 of 200 MCPJs (32.0%), including pannus vascularity and/or tenosynovitis in 55 joints (27.5%) (pannus vascularity in 43, tenosynovitis in 40) and bone destruction in 25 joints (12.5%). Overall, US abnormalities and physical examination scores were significantly associated (P < 0.001). However, interobserver agreement between US and clinical evaluation was poor (kappa 0.1) and between US and radiography was only fair (kappa 0.4). US of the MCPJ in children with JIA can demonstrate cartilage thinning, bone erosions, and pannus vascularity. Abnormal US findings are significantly correlated with severity of disease as evaluated clinically.

  14. 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.

  15. Level of agreement between children, parents, and physicians in rating pain intensity in juvenile idiopathic arthritis.

    PubMed

    Garcia-Munitis, Pablo; Bandeira, Marcia; Pistorio, Angela; Magni-Manzoni, Silvia; Ruperto, Nicolino; Schivo, Ambra; Martini, Alberto; Ravelli, Angelo

    2006-04-15

    To investigate the level of agreement between patients, mothers, fathers, and physicians in rating pain intensity in juvenile idiopathic arthritis (JIA), and to identify factors explaining discrepancies between raters. Ninety-four children with JIA and their mothers and fathers were asked to rate independently the intensity of present pain and pain in the previous week on a visual analog scale. The physicians rated pain intensity after physical examination. Agreement between raters was determined using intraclass correlation coefficient and Bland and Altman method. Correlations of explanatory variables with discordance in rating pain intensity were determined by univariate and multivariate analyses. Explanatory variables included sex, age, JIA category, disease duration, results of study ratings, joint inflammation measures, and erythrocyte sedimentation rate. Agreement in rating present pain was moderate between children and mothers, but was poor between children and fathers and children and physicians. The agreement in rating pain in the previous week was moderate between children and mothers and children and fathers. Mother-father agreement was good. Parents and physicians agreed at a moderate level. In multiple regression analyses, only intensity of present pain was significantly associated with discordance within child-mother, child-father, and child-physician dyads. Children's ratings of pain were only in moderate agreement with those of their parents and were in poor agreement with those of the physicians, whereas the father and mothers agreed at a good level. The intensity of pain was the strongest determinant of discordance between children and other raters.

  16. Jointly managing arthritis: information needs of children with juvenile idiopathic arthritis (JIA) and their parents.

    PubMed

    Stinson, Jennifer N; Feldman, Brian M; Duffy, Ciaran M; Huber, Adam M; Tucker, Lori B; McGrath, Patrick J; Tse, Shirley Ml; Hetherington, Ross; Spiegel, Lynn R; Campillo, Sarah; Benseler, Susanne; Gill, Navreet; White, Meghan E; Baker, Natalie; Vijenthira, Abi

    2012-06-01

    The objective of this article is to explore information needs of children with juvenile idiopathic arthritis (JIA) and their parents in order to develop a web-based psychoeducational program aimed at improving their quality of life. A qualitative study design was used. A purposive sample of children (n = 41; 8-11 years) with JIA and parents (n = 48) participated in parent-child interviews (n = 29), and four child-focus and four parent-focus group interviews. Transcribed data were organized into categories that reflected emerging themes. Findings uncovered three major themes: "living with JIA", "jointly managing JIA", and "need for a web-based program of JIA information and social Support". Subthemes for "Living with JIA" were as follows: "impact on participation", "worry and distress", and "receiving social support". Subthemes under "Jointly Managing JIA" included "obtaining JIA information", "communication and advocacy", and "strategies to manage JIA". Participants endorsed a web-based program as a way to access JIA information and social support. In order to jointly manage JIA, participants expressed the need for disease-specific information, management strategies, and social support and felt that the Internet was acceptable for delivering these disease-management strategies. Findings from this study will inform development and evaluation of an online program to help children and parents jointly manage JIA.

  17. 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.

  18. 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.

  19. Inflammatory Bowel Disease in Juvenile Idiopathic Arthritis Patients Treated with Biologics.

    PubMed

    Barthel, Deborah; Ganser, Gerd; Kuester, Rolf-Michael; Onken, Nils; Minden, Kirsten; Girschick, Hermann Josef; Hospach, Anton; Horneff, Gerd

    2015-11-01

    Evolving inflammatory bowel disease (IBD) is a matter of interest in patients with juvenile idiopathic arthritis (JIA) and might be associated with JIA therapy. Data from the German biologics registry (Biologika in der Kinderrheumatologie; BiKeR) from 2001 to 2013 were analyzed. There were 3071 patients with 8389 patient-years (PY) of observation followed. IBD was diagnosed in 11 patients, 8 with Crohn disease and 3 with ulcerative colitis. IBD incidence in patients with JIA was 1.31/1000 PY and higher than published IBD incidences in pediatric populations. Compared with the total BiKeR cohort, patients with IBD more commonly had enthesitis-related arthritis, extended oligoarthritis, psoriatic arthritis, and also rheumatoid factor (RF)-negative polyarthritis. No IBD occurred in patients with systemic JIA or RF-positive polyarthritis. In patients treated with methotrexate (MTX), the IBD incidence was significantly lower compared with patients not treated with MTX. Etanercept (ETN) monotherapy, but not the combination of ETN and MTX, was associated with an increased incidence of IBD. Incidence of IBD in patients with JIA is higher than in the population. MTX turned out to be protective, even in combination with ETN.

  20. Infection-Related Death among Persons with Refractory Juvenile Idiopathic Arthritis.

    PubMed

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

    2016-10-01

    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.

  1. A multidimensional blood stimulation assay reveals immune alterations underlying systemic juvenile idiopathic arthritis.

    PubMed

    Cepika, Alma-Martina; Banchereau, Romain; Segura, Elodie; Ohouo, Marina; Cantarel, Brandi; Goller, Kristina; Cantrell, Victoria; Ruchaud, Emily; Gatewood, Elizabeth; Nguyen, Phuong; Gu, Jinghua; Anguiano, Esperanza; Zurawski, Sandra; Baisch, Jeanine M; Punaro, Marilynn; Baldwin, Nicole; Obermoser, Gerlinde; Palucka, Karolina; Banchereau, Jacques; Amigorena, Sebastian; Pascual, Virginia

    2017-09-21

    The etiology of sporadic human chronic inflammatory diseases remains mostly unknown. To fill this gap, we developed a strategy that simultaneously integrates blood leukocyte responses to innate stimuli at the transcriptional, cellular, and secreted protein levels. When applied to systemic juvenile idiopathic arthritis (sJIA), an autoinflammatory disease of unknown etiology, this approach identified gene sets associated with specific cytokine environments and activated leukocyte subsets. During disease remission and off treatment, sJIA patients displayed dysregulated responses to TLR4, TLR8, and TLR7 stimulation. Isolated sJIA monocytes underexpressed the IL-1 inhibitor aryl hydrocarbon receptor (AHR) at baseline and accumulated higher levels of intracellular IL-1β after stimulation. Supporting the demonstration that AHR down-regulation skews monocytes toward macrophage differentiation, sJIA monocytes differentiated in vitro toward macrophages, away from the dendritic cell phenotype. This might contribute to the increased incidence of macrophage activation syndrome in these patients. Integrated analysis of high-dimensional data can thus unravel immune alterations predisposing to complex inflammatory diseases. © 2017 Cepika et al.

  2. Interleukin 10 and transforming growth factor beta 1 gene polymorphisms in juvenile idiopathic arthritis.

    PubMed

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

    2016-01-01

    The aim of this study is to identify the associations between interleukin 10 (IL-10) and transforming growth factor beta 1 (TGF-β1) gene polymorphisms and individual susceptibility to juvenile idiopathic arthritis (JIA) in a group of Iranian patients. Cytokine genes, including IL-10 and TGF-β1, are known to play important roles in the pathogenesis of JIA. Using polymerase chain reaction with sequence-specific primers method, the frequency of alleles, genotypes and haplotypes of IL-10 (positions -1082, -819, -592) and TGF-β1 (codon 10, codon 25) single-nucleotide polymorphisms (SNPs) were investigated in 55 patients with JIA as a case group and compared with 140 healthy unrelated controls. The G allele was significantly less frequent at TGF-β1 codon 25 in patients with JIA than in the controls (p < 0.01). The frequency of CT genotype at TGF-β1 codon 10 was found to be higher in healthy individuals in comparison with that in patients group (p = 0.04). We observed no differences in the frequency of alleles, genotypes and haplotypes of IL-10 gene between the groups of patients and controls. Considering the low frequency of existence of TGF-β1 G allele at codon 25 as well as TGF-β1 CT genotype at codon 10 in patients with JIA, it seems that these cytokine gene polymorphisms could play role as the protective factors against JIA.

  3. Novel IL10 gene family associations with systemic juvenile idiopathic arthritis

    PubMed Central

    Fife, Mark S; Gutierrez, Ana; Ogilvie, Emma M; Stock, Carmel JW; Samuel, Jane M; Thomson, Wendy; Mack, Lisa F; Lewis, Cathryn M; Woo, Patricia

    2006-01-01

    Juvenile idiopathic arthritis (JIA) is the most common cause of chronic childhood disability and encompasses a number of disease subgroups. In this study we have focused on systemic JIA (sJIA), which accounts for approximately 11% of UK JIA cases. This study reports the investigation of three members of the IL10 gene family as candidate susceptibility loci in children with sJIA. DNA from 473 unaffected controls and 172 patients with sJIA was genotyped for a single nucleotide polymorphism (SNP) in IL19 and IL20 and two SNPs in IL10. We examined evidence for association of the four SNPs by single marker and haplotype analysis. Significant differences in allele frequency were observed between cases and controls, for both IL10-1082 (p = 0.031) and IL20-468 (p = 0.028). Furthermore, examination of the haplotypes of IL10-1082 and IL20-468 revealed greater evidence for association (global p = 0.0006). This study demonstrates a significant increased prevalence of the low expressing IL10-1082 genotype in patients with sJIA. In addition, we show a separate association with an IL20 polymorphism, and the IL10-1082A/IL20-468T haplotype. The two marker 'A-T' haplotype confers an odds ratio of 2.24 for sJIA. This positive association suggests an important role for these cytokines in sJIA pathogenesis. PMID:16959027

  4. 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

  5. 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

  6. Treatment of patients with juvenile idiopathic arthritis (JIA) in a population-based cohort.

    PubMed

    Zamora-Legoff, Jorge A; Krause, Megan L; Crowson, Cynthia S; Muskardin, Theresa Wampler; Mason, Thomas; Matteson, Eric L

    2016-06-01

    A population-based cohort was utilized to evaluate medications and intra-articular injection utilization for patients with juvenile idiopathic arthritis (JIA) to inform clinical practice and further research. In a geographically defined population, all incident cases of JIA cases were identified between January 1, 1994 and December 31, 2013 based first on diagnosis code followed by medical chart confirmation. Medications and intra-articular glucocorticoid injections were abstracted. Predictors of the first disease-modifying antirheumatic drug (DMARD)/biologic and injections were reported as a hazard ratio (HR) with 95 % confidence intervals (CIs) adjusted for age and sex. Kaplan-Meier methods evaluated therapy at 6 months and 1 year. Injections were reported per 100 person-years (py) with 95 % CI using the Poisson methods. Seventy-one incident cases were identified. Forty-two (59 %) were female with mean age (standard deviation) at diagnosis of 8.2 (5.3) years. Twenty-six (37 %) utilized at least one DMARD or biologic, in which 77 % of these were prescribed in the first 6 months. Subtype of JIA was significantly associated with DMARDs/biologics (p < 0.001). Intra-articular injections were performed in 48 %. The rate of intra-articular injections was 20.7 per 100 py (95 % CI 16.5, 25.6). The rate of joint injections was higher in the first year after diagnosis (p < 0.001) and more common in recent years (p < 0.001). The majority of patients with JIA in a modern population-based cohort do not require DMARDs or biologics. In those who do, the majority receives these within the first 6 months. Intra-articular injections were utilized in almost half of patients with JIA and were increasingly used.

  7. Do patients with juvenile idiopathic arthritis in remission exhibit active synovitis on joint ultrasound?

    PubMed

    Bugni Miotto e Silva, Vanessa; de Freitas Tavares da Silva, Carolina; de Aguiar Vilela Mitraud, Sônia; Nely Vilar Furtado, Rita; Esteves Hilário, Maria Odete; Natour, Jamil; Terreri, Maria Teresa

    2014-07-01

    The aim of the study was to assess the presence and characteristics of subclinical synovitis using power Doppler (PD) ultrasonography on patients with juvenile idiopathic arthritis (JIA) in clinical remission and compare the findings with those of healthy children. A cross-sectional study was carried out involving the clinical (physical exam, functional capacity and laboratory tests) and ultrasonography evaluation of 34 joints (synovial fluid/hypertrophy, PD signal and bone erosion). Subclinical synovitis was defined as the presence of synovial hypertrophy/joint effusion with or without any PD signal. Thirty-six patients (11.5 ± 3.74 years) and 36 controls (sex and age matched) were evaluated (2,448 joints). Twenty-seven patients were in remission on medication (mean duration: 1.8 ± 2.2 years). Subclinical synovitis was detected in 41.7% patients and 11.1% controls (p = 0.003). Erosion was detected in three patients (8.3%). Subclinical synovitis was found in 38/1,224 (3.1%) joints in the patients (most affected: radiocarpal wrist, anterior elbow and tibiotalar ankle) and 8/1,224 (0.6%) joints in the controls (most affected: radiocarpal wrist). Differences in subclinical synovitis between patients and controls were found in the elbows (p = 0.033) and ankles (p = 0.006). A greater frequency of subclinical synovitis was found in patients with the extended oligoarticular or polyarticular subtypes (p = 0.013), those at an older age at disease onset (p = 0.007) and using methotrexate (p = 0.049). Patients with JIA in remission exhibit subclinical synovitis more frequently than controls. Subclinical synovitis was more frequent in patients with the polyarticular involvement and those at an older age at disease onset.

  8. 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

    2015-01-01

    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.

  9. [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.

  10. Perceived adherence to prescribed treatment in juvenile idiopathic arthritis over a one-year period.

    PubMed

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

    2007-03-15

    To document perceived adherence to treatment (taking medications and performing exercises) in patients with juvenile idiopathic arthritis (JIA) over a 1-year period and to identify related factors. We surveyed parents of patients with JIA at the Montreal Children's Hospital and British Columbia's Children's Hospital in Vancouver. Parents were asked to respond to a series of questionnaires every 3 months over a 12-month period. Perceived adherence was evaluated on a 100-mm visual analog scale (VAS) in the Parent Adherence Report Questionnaire (PARQ). Parental coping, distress, child function, disease severity and duration, perceived helpfulness of treatment, problems encountered, and sociodemographic data were also assessed. The mean age of our sample of 175 children was 10.2 years; mean age at diagnosis was 6.1 years and mean disease duration was 4.1 years. Perceived adherence to medications was consistently high, with average adherence at baseline, 3, 6, 9, and 12 months being 86.1, 91.7, 90.4, 92.0, and 88.8, respectively, on the PARQ VAS. Perceived adherence to exercise was lower but remained steady, with corresponding means of 54.5, 64.1, 61.2, 63.0, and 54.3, respectively. Using generalized estimating equation analysis, factors associated with higher perceived adherence to medications included perceived helpfulness of medications and lower disease severity; those associated with higher perceived adherence to exercise were younger age of the child, child involvement in responsibility for treatment, and higher perceived helpfulness of the treatment. Belief in helpfulness of treatment is associated with higher parental perceived adherence to treatment.

  11. Incidence of Selected Opportunistic Infections Among Children with Juvenile Idiopathic Arthritis

    PubMed Central

    Beukelman, Timothy; Xie, Fenglong; Baddley, John W; Chen, Lang; Delzell, Elizabeth; Grijalva, Carlos G; Mannion, Melissa L; Patkar, Nivedita M; Saag, Kenneth G; Winthrop, Kevin L; Curtis, Jeffrey R

    2013-01-01

    Objective To compare incidence rates of selected opportunistic infections (OI) among children with and without juvenile idiopathic arthritis (JIA). Methods Using United States national Medicaid administrative claims data from 2000 through 2005, we identified a cohort of children with JIA based on physician diagnosis codes and dispensed medications. We defined a non-JIA comparator cohort of children diagnosed with attention deficit hyperactivity disorder (ADHD). We defined 15 types of OI using physician diagnosis or hospital discharge codes, and 7 of these types also required evidence of treatment with specific antimicrobials. We calculated infection incidence rates (IR). The rates in the ADHD comparator cohort were standardized to the age, sex, and race distribution of the JIA cohort. We calculated incidence rate ratios (IRR) to compare infection rates. Results The JIA cohort included 8,503 children with 13,990 person-years (p-y) of follow-up. The ADHD comparator cohort included 360,362 children with 477,050 p-y of follow-up. When all OI were considered together as a single outcome, there were 42 infections in the JIA cohort (IR 300 per 100,000 p-y; IRR 2.4 [1.7–3.3] versus ADHD). The most common OI among children with JIA were 3 Coccidioides (IR 21 per 100,000 p-y; IRR 101 [8.1–5319] versus ADHD); 5 Salmonella (IR 35 per 100,000 p-y; IRR 3.8 [1.2–9.5]); and 32 herpes zoster (IR 225 per 100,000 p-y; IRR 2.1 [1.4–3.0]). Conclusions OI are rare among children with JIA. Nevertheless, children with JIA had a higher rate of OI, including Coccidioides, Salmonella, and herpes zoster, than children with ADHD. PMID:23460423

  12. 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

    2017-07-18

    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

  13. [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.

  14. 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.

  15. Sleep problems and associated factors in children with juvenile idiopathic arthritis: a systematic review.

    PubMed

    Stinson, Jennifer N; Hayden, Jill A; Ahola Kohut, Sara; Soobiah, Charlene; Cartwright, Jenny; Weiss, Shelly K; Witmans, Manisha B

    2014-01-01

    Sleep problems are common among children with chronic illnesses such as Juvenile Idiopathic Arthritis (or JIA). However, little is known about the frequency and severity of sleep disturbance(s) and the factors that are associated with sleep problems in children with JIA. The mechanism(s) of the relationships characterizing the development or exacerbation of sleep problems in children with JIA are still unknown, however studies have reported an association. The purpose of this study was to synthesize existing research related to sleep problems in children with JIA. The Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement guided the conduct and reporting of this review. An experienced librarian conducted searches in MEDLINE, EMBASE, PsychINFO, CINAHL, and the Cochrane Central Register of Controlled Trials from inception to January 2012, to identify potentially relevant citations. Two members independently selected, rated methodological quality using the QUIPS tool, and extracted data from included studies. Ten studies were included and findings varied across studies; studies were mostly cross-sectional, or case-controlled designs, with only one cohort study available. Four studies found that children and adolescents diagnosed with JIA had significantly more sleep disturbances when compared to healthy controls. Pain was most often associated with sleep disturbances. The heterogeneous findings highlight the complex relationships between JIA and sleep, and low methodological quality of studies in the field. This review supports an association between poor sleep and increased symptoms related to JIA, specifically the experience of pain. However, results need to be interpreted cautiously given the inconsistent findings regarding factors associated with sleep problems in JIA, the limited evidence available, and its low quality. Furthermore it is not yet determined if the poor sleep patterns predate the symptoms reported with JIA. More

  16. Characteristics and relationship of periodontal disease with juvenile idiopathic and rheumatoid arthritis

    PubMed Central

    Vahabi, Surena; Rostamian, Abdolrahman; Baniebrahimi, Ghazaleh

    2015-01-01

    Background: Rheumatoid arthritis (RA) is the most prevalent chronic inflammatory disease of the joints. It is correlated with periodontal disease due to similar factors that exist in both diseases. The present study assessed the relationship of periodontal disease with RA and juvenile idiopathic arthritis (JIA). Materials and Methods: In this case-control study, 30 RA and 30 JIA patients along with similar number of matched controls were selected among patients referred to Imam Khomeini Hospital, Tehran, Iran. Periodontal parameters including pocket depth (PD), clinical attachment level (CAL), O’Leary and Bay plaque index (PI) and bleeding on probing (BOP) were determined in cases and controls. Erythrocyte sedimentation rate, number of painful and inflamed joints and severity of disease were evaluated in RA and JIA patients. Mann-Whitney U-test nonparametric, Spearman and Pearson's correlation coefficients, and Chi-square tests were used as statistical analysis (α = 0.05). Results: PD (4.17 vs. 3.6 mm; P < 0.0001), CAL (4.89 vs. 4.18 mm; P < 0.002), percentage of sites with PD >4 mm (58.83% vs. 44.33%; P < 0.002), percentage of sites with CAL >3 mm (74.13% vs. 64.4%; P < 0.001), percentage of sites with BOP (9.67% vs. 6.87%; P < 0.0001) and PI index (85.73% vs. 80.63%; P < 0.0001) were significantly higher in RA patients than controls. In this group, direct and significant correlations were found between serologic findings, disease severity and number of painful and inflamed joints with periodontal factors. In JIA patients, no significant relationships were found between JIA findings and periodontal parameters. Conclusion: Considering the limitations of this study, there was a relationship between RA and periodontal disease. Severity of periodontal disease increases in patients with RA, while no increased risk of periodontal disease or its severity was observed among JIA patients. PMID:26759590

  17. Complementary and alternative medicine use in adolescents with inflammatory bowel disease and juvenile idiopathic arthritis

    PubMed Central

    2014-01-01

    Background The use of complementary alternative medicine (CAM) is potentially prevalent among paediatric patients with chronic diseases but with variable rates among different age groups, diseases and countries. There are no recent reports on CAM use among paediatric patients with inflammatory bowel disease (IBD) and juvenile idiopathic arthritis (JIA) in Europe. We hypothesized that CAM use associates with a more severe disease in paediatric IBD and JIA. Methods A cross-sectional questionnaire study among adolescent outpatients with IBD and JIA addressing the frequency and type of CAM use during the past year. The patients were recruited at the Children’s Hospital, University of Helsinki, Finland. Results Of the 147 respondents, 97 had IBD (Crohn’s disease: n = 46; median age 15.5, disease duration 3.4 years) and 50 had JIA (median age 13.8, disease duration 6.9 years). During the past 12 months, 48% regularly used CAM while 81% reported occasional CAM use. Compared to patients with JIA, the use of CAM in IBD patients tended to be more frequent. The most commonly used CAM included probiotics, multivitamins, and mineral and trace element supplements. Self-imposed dietary restrictions were common, involving 27.6% of the non-CAM users but 64.8% of all CAM users. Disease activity was associated with CAM use in JIA but not in IBD. Conclusions CAM use is frequent among adolescents with IBD and JIA and associates with self-imposed dietary restrictions. Reassuringly, adherence to disease modifying drugs is good in young CAM users. In JIA, patients with active disease used more frequently CAM than patients with inactive disease. As CAM use is frequent, physicians should familiarise themselves with the basic concepts of CAM. The potential pharmacological interaction or the toxicity of certain CAM products warrants awareness and hence physicians should actively ask their patients about CAM use. PMID:24708564

  18. Periodontal and hematological characteristics associated with aggressive periodontitis, juvenile idiopathic arthritis, and rheumatoid arthritis.

    PubMed

    Havemose-Poulsen, Anne; Westergaard, Jytte; Stoltze, Kaj; Skjødt, Henrik; Danneskiold-Samsøe, Bente; Locht, Henning; Bendtzen, Klaus; Holmstrup, Palle

    2006-02-01

    Periodontitis shares several clinical and pathogenic characteristics with chronic arthritis, and there is some degree of coexistence. The aims of this study were to elucidate whether patients with localized aggressive periodontitis (LAgP), generalized aggressive periodontitis (GAgP), juvenile idiopathic arthritis (JIA), and rheumatoid arthritis (RA) share periodontal and hematological characteristics distinguishing them from individuals free of diseases. The study population consisted of white adults (or=4 mm, CAL>or=2 mm, and ABL>or=2 mm compared to controls. The percentage of sites with CAL>or=2 mm significantly correlated with the levels of IgM-RF and IgA-RF. Missing teeth in JIA and RA patients were not lost due to periodontitis. Patients with GAgP showed higher levels of leukocytes, including neutrophils, and CRP compared to controls. In part, JIA and RA patients showed similar results. Young adults with RA may develop periodontal destruction, and these patients require professional attention. Both differences and similarities in periodontal and hematological variables were seen in individuals with periodontitis, JIA, and RA.

  19. Characteristics and relationship of periodontal disease with juvenile idiopathic and rheumatoid arthritis.

    PubMed

    Vahabi, Surena; Rostamian, Abdolrahman; Baniebrahimi, Ghazaleh

    2015-01-01

    Rheumatoid arthritis (RA) is the most prevalent chronic inflammatory disease of the joints. It is correlated with periodontal disease due to similar factors that exist in both diseases. The present study assessed the relationship of periodontal disease with RA and juvenile idiopathic arthritis (JIA). In this case-control study, 30 RA and 30 JIA patients along with similar number of matched controls were selected among patients referred to Imam Khomeini Hospital, Tehran, Iran. Periodontal parameters including pocket depth (PD), clinical attachment level (CAL), O'Leary and Bay plaque index (PI) and bleeding on probing (BOP) were determined in cases and controls. Erythrocyte sedimentation rate, number of painful and inflamed joints and severity of disease were evaluated in RA and JIA patients. Mann-Whitney U-test nonparametric, Spearman and Pearson's correlation coefficients, and Chi-square tests were used as statistical analysis (α = 0.05). PD (4.17 vs. 3.6 mm; P < 0.0001), CAL (4.89 vs. 4.18 mm; P < 0.002), percentage of sites with PD >4 mm (58.83% vs. 44.33%; P < 0.002), percentage of sites with CAL >3 mm (74.13% vs. 64.4%; P < 0.001), percentage of sites with BOP (9.67% vs. 6.87%; P < 0.0001) and PI index (85.73% vs. 80.63%; P < 0.0001) were significantly higher in RA patients than controls. In this group, direct and significant correlations were found between serologic findings, disease severity and number of painful and inflamed joints with periodontal factors. In JIA patients, no significant relationships were found between JIA findings and periodontal parameters. Considering the limitations of this study, there was a relationship between RA and periodontal disease. Severity of periodontal disease increases in patients with RA, while no increased risk of periodontal disease or its severity was observed among JIA patients.

  20. Diagnostic performance of anti-citrullinated protein/peptide antibodies in juvenile idiopathic arthritis.

    PubMed

    Pang, S Y; Liu, H Y; Huang, Y J; Liu, Y F; Dai, Y M; Zeng, P; Zeng, H S

    2016-05-13

    The prevalence rates of anti-citrullinated protein/peptide antibodies (ACPAs) were investigated in a cohort of juvenile idiopathic arthritis (JIA) patients, and their diagnostic performances were compared. ACPAs, including anti-cyclic citrullinated peptide IgG (anti-CCP), anti-CCP IgG/IgA (anti-CCP3.1), citrullinated recombinant rat filaggrin antibodies (CPA), anti-mutated citrullinated vimentin (anti-MCV), and antibodies to citrullinated human IgG-derived peptides (RA/CP), were measured in the sera from 81 JIA patients. Serum samples from 55 children with other joint diseases or viral infections and 49 healthy donors were tested as controls. Of the 81 JIA patients, 7 (8.6%), 8 (9.9%), 17 (21.0%), 23 (28.4%), and 18 (22.2%) were found to be positive for anti-CCP, anti-CCP3.1, CPA, anti-MCV, and RA/CP, respectively, with specificities of 98.1, 95.1, 93.3, 84.6, and 86.5%. Analysis by subtype revealed that 7/7 (100%) of RF-positive polyarticular JIA patients tested positive at high serum levels for anti-MCV or RA/CP, and 5/7 (71.4%) were positive for anti-CCP, anti- CCP3.1, or CPA (P < 0.001, compared with controls). Eighteen of 81 JIA patients demonstrated joint erosions on radiographs and erosive arthritis occurred more often in ACPAs positive patients (P < 0.01). Our findings indicate that although ACPAs are not satisfactory screening biomarkers for JIA due to low sensitivity, ACPA measurement can aid in diagnosing RF-positive polyarticular JIA and identifying JIA patients with severe bone involvement. The diagnostic performance of each ACPA in JIA is different, and the careful selection of assays is necessary.

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

    PubMed Central

    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

    2012-01-01

    Objectives 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. Methods 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. Results 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. Conclusions 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. PMID:22294642

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

    PubMed Central

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

    2010-01-01

    Background There is strong evidence suggesting that juvenile idiopathic arthritis (JIA) shares many susceptibility loci with other autoimmune diseases. Objective To investigate variants robustly associated with type 1 diabetes (T1D) or coeliac disease (CD) for association with JIA. Methods 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. Results One SNP in the LPP gene, rs1464510, showed significant association with JIA (ptrend=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 (ptrend=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 (ptrend=0.005, OR=1.88, 95% CI 1.2 to 2.94). Conclusions 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. PMID:20647273

  3. 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

  4. Self-Reported Pain and Disease Symptoms Persist in Juvenile Idiopathic Arthritis Despite Treatment Advances

    PubMed Central

    Bromberg, Maggie H.; Connelly, Mark; Anthony, Kelly K.; Gil, Karen M.; Schanberg, Laura E.

    2014-01-01

    Objective To use electronic diaries (e-diaries) to determine whether pain, stiffness, and fatigue continue to be common, disabling symptoms in children with juvenile idiopathic arthritis (JIA) despite the use of aggressive treatments in contemporary medical management. Methods Fifty-nine children with JIA (ages 8–18 years) provided ratings of pain, stiffness, and fatigue intensity and functional limitations using a smartphone e-diary 3 times each day for 1 month. Medication information was collected via parent report and checked for accuracy by chart review. Descriptive analyses were conducted to determine typical symptom intensity, frequency, and variability. Multilevel modeling was used to analyze associations between symptoms and functional outcomes and between medication use and symptom intensity. Results Children reported moments of pain in 66% of e-diary entries. No children were entirely pain-free across the reporting period. In 31% of all e-diary entries the visual analog scale score for pain was >40 (high pain intensity), with 86% of children reporting a high level of pain at least once during the study period. The mean ratings of pain, stiffness, and fatigue intensity were in the mild-to-moderate range. Medication class was not a reliable predictor of differences in symptom intensity, even though 79% of children were prescribed a disease-modifying antirheumatic drug and 47% were prescribed a biologic agent. Moments of higher pain intensity and higher stiffness intensity were each uniquely predictive of higher concurrent functional limitations. Conclusion Self-reported pain, stiffness, and fatigue continue to be common in children with JIA, despite contemporary advances in treatment strategies, including use of biologic agents. These findings are surprisingly consistent with previous results from research using daily paper diaries in the pre-biologics era. There remains a pressing and ongoing need to optimize pain and symptom management in JIA. PMID

  5. Synovial membrane protein expression differs between juvenile idiopathic arthritis subtypes in early disease

    PubMed Central

    2014-01-01

    Introduction Juvenile idiopathic arthritis (JIA) is the most common rheumatological disease of childhood with a prevalence of around 1 in 1,000. Without appropriate treatment it can have devastating consequences including permanent disability from joint destruction and growth deformities. Disease aetiology remains unknown. Investigation of disease pathology at the level of the synovial membrane is required if we want to begin to understand the disease at the molecular and biochemical level. The synovial membrane proteome from early disease-stage, treatment naive JIA patients was compared between polyarticular and oligoarticular subgroups. Methods Protein was extracted from 15 newly diagnosed, treatment naive JIA synovial membrane biopsies and separated by two dimensional fluorescent difference in-gel electrophoresis. Proteins displaying a two-fold or greater change in expression levels between the two subgroups were identified by matrix assisted laser desorption ionization-time of flight mass spectrometry with expression further verified by Western blotting and immunohistochemistry. Results Analysis of variance analysis (P ≤ 0.05) revealed 25 protein spots with a two-fold or greater difference in expression levels between polyarticular and oligoarticular patients. Hierarchical cluster analysis with Pearson ranked correlation revealed two distinctive clusters of proteins. Some of the proteins that were differentially expressed included: integrin alpha 2b (P = 0.04); fibrinogen D fragment (P = 0.005); collagen type VI (P = 0.03); fibrinogen gamma chain (P = 0.05) and peroxiredoxin 2 (P = 0.02). The identified proteins are involved in a number of different processes including platelet activation and the coagulation system. Conclusions The data indicate distinct synovial membrane proteome profiles between JIA subgroups at an early stage in the disease process. The identified proteins also provide insight into differentially perturbed pathways

  6. Juvenile idiopathic arthritis patients with low inflammatory activity have increased adiposity.

    PubMed

    Grönlund, M-M; Kaartoaho, M; Putto-Laurila, A; Laitinen, K

    2014-01-01

    The aim of this study was to assess the effect of juvenile idiopathic arthritis (JIA), its subtypes and disease activity on anthropometric measurements, body composition, and nutritional parameters. A cross-sectional cohort of 40 JIA patients, aged 3-10 years, was compared with 40 healthy children matched for age and gender. Concentrations of nutritional and inflammatory biomarkers in the blood, anthropometric measures, and clinical status were recorded and the parents filled in a 7-day food diary and completed the Childhood Health Assessment Questionnaire (CHAQ). The JIA patients had low disease activity: 60% had inactive disease, the median CHAQ score was 0.125, and the median erythrocyte sedimentation rate (ESR) was 6 mm/h. Significantly higher values for central and peripheral adiposity were found in JIA patients compared with in healthy controls [waist circumference mean (SD) 55.9 (4.9) vs. 53.4 (3.7) cm, p < 0.0001, and biceps skinfold thickness 6.2 (2.3) vs. 5.3 (1.7) cm, p = 0.035, respectively], and obesity/overweight was more common (30% vs. 12.5%, p = 0.056, respectively) despite no differences in weight-for-height. The intake of energy (kcal/day) was significantly higher in the JIA patients (p = 0.036). The nutritional biomarkers were comparable in both groups. The JIA subtype and disease activity did not affect body composition, energy intake, or the nutritional biomarkers. Even JIA patients with low disease activity have a higher central and peripheral adiposity and a higher energy intake than their healthy peers. Neither disease subtype nor disease activity had any association with changes in body composition.

  7. 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

  8. Effectiveness and safety of TNF inhibitors in adults with juvenile idiopathic arthritis

    PubMed Central

    McErlane, Flora; Foster, Helen E; Lunt, Mark; Watson, Kath D; Hyrich, Kimme L

    2016-01-01

    Introduction Many children with juvenile idiopathic arthritis (JIA) continue to have active disease into adulthood. Adults with JIA are a heterogeneous group, and the effects of tumour necrosis factor inhibitor (TNFi) therapies are not well described. This analysis aims to describe treatment outcomes among patients with JIA starting TNFi for the first time in adulthood. Methods Patients with arthritis onset <16 years starting their first TNFi therapy were identified from the British Society of Rheumatology Biologics Register. Disease activity outcomes (using 28-joint Disease Activity Score (DAS28) and Health Assessment Questionnaire (HAQ)) are presented at 1 year after start of therapy according to disease pattern. Incidence rates (IR) of adverse events per 1000 person-years (pyrs) were calculated. Outcomes in patients with polyarticular JIA were compared with a cohort (weighted for age and gender) of patients with rheumatoid arthritis (RA). Results In 443 adults with JIA starting a first TNFi, disease activity over 1 year improved across all measures. There were 58 first serious infections (IR 22.3/1000 pyrs); 4 cardiovascular events (IR 1.4/1000 pyrs); 11 uveitis events (IR 4.0/1000 pyrs) and 16 malignancies (IR 3.9/1000 pyrs). Compared with the weighted RA cohort, disease activity improvement was similar; malignancy rates were lower and uveitis rates much higher. While crude IR were similar, JIA patients had a lower risk of serious infection (HR 0.5 (95% CI 0.3 to 0.9)). Conclusions This is the largest study to describe disease activity and safety outcomes in adults with JIA receiving TNFi. Disease activity improved after 1 year in all disease patterns, suggesting TNFi is an effective therapy in this population. PMID:27843575

  9. 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

  10. Participation in Leisure Activities by Children and Adolescents with Juvenile Idiopathic Arthritis.

    PubMed

    Cavallo, Sabrina; Majnemer, Annette; Duffy, Ciarán M; Feldman, Debbie Ehrmann

    2015-09-01

    To describe leisure activities of children and adolescents with juvenile idiopathic arthritis (JIA) in terms of diversity, intensity, and enjoyment, and to identify potential determinants. One hundred seven children and adolescents aged 8-17 years diagnosed with JIA and their families participated in this cross-sectional study. Participants answered the Children's Assessment of Participation and Enjoyment, which measures involvement in leisure (recreation, active physical, social, skill-based, self-improvement). Disease characteristics and sociodemographic factors were abstracted from the child's medical file. In terms of intensity, individuals with JIA were more often engaged in informal rather than formal leisure activities [t(106) = 45.5, p < 0.0001]. When intensity scores were compared across activity type, results showed that participants with JIA were most often involved in social and recreational activities (p < 0.001). The level of enjoyment was highest for social activities and lowest for self-improvement activities (p < 0.001). Those with active arthritis displayed less diverse (p = 0.016) and less intense (p = 0.011) participation in active physical activities, and less frequent involvement in informal activities (p = 0.043) compared with those who were asymptomatic. Children and adolescents with JIA tend to participate more in sedentary types of activities. Greater disease activity may dissuade children and adolescents from participating in more active pursuits, which places them at greater risk for adopting sedentary lifestyles. The identification of determinants of leisure activities in children and adolescents with arthritis may allow healthcare professionals to assess children's health needs with more precision and promote a healthier lifestyle.

  11. Agreement among musculoskeletal pediatric specialists in the assessment of radiographic joint damage in juvenile idiopathic arthritis.

    PubMed

    Rodriguez-Lozano, Ana-Luisa; Giancane, Gabriella; Pignataro, Rossana; Viola, Stefania; Valle, Maura; Gregorio, Sandro; Norambuena, Ximena; Ioseliani, Maka; Pistorio, Angela; Magnaguagno, Francesca; Riganti, Simone; Martini, Alberto; Ravelli, Angelo

    2014-01-01

    To evaluate agreement among musculoskeletal pediatric specialists in assessing radiographic joint damage in juvenile idiopathic arthritis (JIA). Two pediatric rheumatologists, 2 pediatric radiologists, and 2 pediatric orthopedic surgeons evaluated independently 60 radiographs of both wrists and hands of children with polyarticular-course JIA. Films were scored using an adapted and simplified version of the Larsen score, ranging from 0-5. Study radiographs were selected from 568 films used in a previous study aimed to validate an adapted pediatric version of the Sharp/van der Heijde (SHS) score. To enable comparison of specialists' scores with the adapted SHS score, the 60 radiographs were divided into 6 classes of severity of damage based on quintiles of the adapted SHS score. Agreement was evaluated in terms of absolute agreement and through weighted kappa statistics. The pediatric radiologists tended to assign lower scores and to provide more frequently scores of 0 than did the other specialists. Weighted kappa for the 3 pairs of specialists ranged from 0.67-0.69, indicating substantial agreement. Absolute agreement ranged from 51.3-55.7%, depending on the pair of specialists examined. Both absolute and weighted kappa concordance between specialists' scores and the adapted SHS score were poorer for the pediatric radiologist than for the other specialists. We observed fair agreement in the assessment of radiographic damage among pediatric specialists involved in the care of children with JIA. The radiologists tended to be more reserved than the rheumatologists and orthopedic surgeons in labeling radiographs as damaged or in considering changes as important. Copyright © 2014 by the American College of Rheumatology.

  12. [Three cases with familial Mediterranean fever misdiagnosed as juvenile idiopathic arthritis].

    PubMed

    Li, J; Zhang, Y; Wang, W; Zhong, L Q; Song, H M

    2017-05-04

    Objective: To explore the key points of diagnosis and treatment of familial Mediterranean fever(FMF). Method: The clinical data of 3 cases with FMF misdiagnosed as Juvenile idiopathic arthritis(JIA)seen from January 2014 to June 2016 in Peking Union Medical College Hospital were retrospectively collected. The clinical manifestations, gene mutation characteristics, treatment and prognosis were also evaluated. Result: Two cases were male and 1 was female. The mean age of onset was 17 months (3 months to 36 months), while the average age of diagnosis was 6 years and 8 months (24 months to 11 years). All the 3 cases presented with periodic fever, red rash and arthritis.Two of them suffered from anemia, 2 of them showed lymphadenopathy, and 1 of them presented with hepatosplenomegaly. All of the 3 cases were diagnosed as JIA by excluding infectious diseases and neoplastic diseases and respondiug poorly to anti-infection treatment, but they benefitted little from glucocorticoids and a variety of immunosuppressive therapy. The mutations of MEFV gene were found in 3 cases by gene detection, and all of them were complex heterozygous mutations. Four reported pathogenic mutations were found: R202Q, E148Q, L110P, P369S. All the 3 cases are currently receiving oral colchicine (in accordance with the initial dose of children under the age of 5 recommended ≤ 0.5 mg/d, 5 to 10 years old children 0.5-1.0 mg/d, 10 years old children and older children 1.0-1.5 mg / d) , and the symptoms were significantly improved. Conclusion: The familial Mediterranean fever can be characterized by repeated remittent fever, red rash, arthritis, and is easy to be confused with JIA in clinical manifestation.In this paper, 3 cases were diagnosed as complex heterozygous MEFV gene mutation by gene analysis.During the 6 months follow-up, all of the 3 patients responded well to colchicine.

  13. 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

  14. 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.

  15. Ultrasonography and color Doppler of proximal gluteal enthesitis in juvenile idiopathic arthritis: a descriptive study

    PubMed Central

    2011-01-01

    Background The presence of enthesitis (insertional inflammation) in patients with juvenile idiopathic arthritis (JIA) is difficult to establish clinically and may influence classification and treatment of the disease. We used ultrasonography (US) and color Doppler (CD) imaging to detect enthesitis at the small and deep-seated proximal insertion of the gluteus medius fascia on the posterior iliac crest where clinical diagnosis is difficult. The findings in JIA patients were compared with those obtained in healthy controls and with the patients' MRI results. Methods Seventy-six proximal gluteus medius insertions were studied clinically (tenderness to palpation of the posterior iliac crest) and by US and CD (echogenicity, thickness, hyperemia) in 38 patients with JIA and in 38 healthy controls, respectively (median age 13 years, range 7-18 years). In addition, an additional MRI examination of the sacroiliac joints and iliac crests was performed in all patients. Results In patients with focal, palpable tenderness, US detected decreased echogenicity of the entheses in 53% of the iliac crests (bilateral in 37% and unilateral in 32%). US also revealed significantly thicker entheses in JIA patients compared to healthy controls (p < 0.003 left side, p < 0.001 right side). There was no significant difference in thickness between the left and right sides in individual subjects. Hyperemia was detected by CD in 37% (28/76) of the iliac crests and by contrast-enhanced MRI in 12% (6/50). Conclusions According to US, the gluteus medius insertion was thicker in JIA patients than in controls, and it was hypoechoic (enthesitis) in about half of the patients. These findings may represent chronic, inactive disease in some of the patients, because there was only limited Doppler flow and MRI contrast enhancement. The present study indicates that US can be useful as an adjunct to clinical examination for improved assessment of enthesitis in JIA. This may influence disease classification

  16. Polymorphisms of genes encoding interleukin-4 and its receptor in Iranian patients with juvenile idiopathic arthritis.

    PubMed

    Ziaee, Vahid; Rezaei, Arezou; Harsini, Sara; Maddah, Marzieh; Zoghi, Samaneh; Sadr, Maryam; Moradinejad, Mohammad Hassan; Rezaei, Nima

    As cytokines, including interleukin-4 (IL-4), seem to have a pivotal role in the pathogenesis of juvenile idiopathic arthritis (JIA), this study is aimed at investigating of association of polymorphisms in IL-4 and IL-4 receptor α (IL-4RA) genes with susceptibility to JIA. A case-control study was conducted on 53 patients with JIA and 139 healthy unrelated controls. Single nucleotide polymorphisms of IL-4 gene at positions -1098, -590, and -33, as well as IL-4RA gene at position +1902 were genotyped using polymerase chain reaction with sequence-specific primers method and compared between patients and healthy individuals. At the allelic level, C allele at IL-4 -33 was found to be more frequent in patients compared to control (P value <0.01). At the genotypic level, CC genotype at IL-4 -590 (P value <0.01), together with CC and TT genotypes at IL-4 -33 (P value <0.01), were significantly higher in patients with JIA, while TC genotypes at IL-4 -590 and -33 positions were found to be lower in case group (P value <0.01). At the haplotypic level, IL-4 (positions -1098, -509, -33) TTC, GCC, and TTT haplotypes were significantly lower than controls (P value <0.01, P value = 0.03, and P value = 0.04, respectively). Although, TCC haplotype at the same positions was found to be higher in patients (P value <0.01). Polymorphic site of +1902 IL-4RA gene did not differ between cases and controls. Polymorphisms in promoter region of IL-4 but not IL-4RA genes confer susceptibility to JIA and may predispose individuals to adaptive immune responses.

  17. Association of interleukin-1 family gene polymorphisms with juvenile idiopathic arthritis in Iranian population.

    PubMed

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

    Cytokines, including interleukin-1 (IL-1), seem to contribute towards the pathogenesis of juvenile idiopathic arthritis (JIA), so this study was designed to evaluate the associations of IL-1 gene cluster and IL-1 receptor (IL-1R) gene single nucleotide polymorphisms (SNPs) with JIA proneness in Iranian population. Genomic DNA of 55 Iranian patients with JIA and 140 controls were extracted and typed for IL-1α gene at position -889, IL-1β gene at positions -511 and +3962, IL-1R gene at position Pst-I 1970, and interleikin-1 receptor antagonist (IL-1Ra) gene at position Mspa-I 11100, using polymerase chain reaction with sequence-specific primers method, and compared between patients and controls. The CC genotype of IL-1Ra at Mspa-I 11100 position was found to be more frequent in patients with JIA compared to healthy individuals (P=0.03), although the CT genotype at the same position was significantly higher in the control group in comparison with patients with JIA (P=0.02). No significant differences were observed between the two groups of case and control for IL-1α (-889 C/T), IL-1β (-511 C/T and +3962 C/T) and IL-1R (Pst-1 1970 C/T). The results of the present investigation suggest that certain IL-1Ra gene variants are associated with individuals' susceptibility to JIA. Nevertheless, further studies are required to establish the results of the current study. Copyright © 2016 SEICAP. Published by Elsevier España, S.L.U. All rights reserved.

  18. Patient-reported joint count in juvenile idiopathic arthritis: the reliability of a manikin format.

    PubMed

    Dijkstra, Maryanne E; Anink, Janneke; van Pelt, Philomine A; Hazes, Johanna M; van Suijlekom-Smit, Lisette W A

    2015-03-01

    To evaluate the reliability of a manikin format, patient-reported joint count in juvenile idiopathic arthritis (JIA), and to detect changes in agreement at a second visit. Patients with JIA aged 12-21 were asked to mark joints with active arthritis on a manikin before their regular clinic visit. The physician then performed a joint count without having seen the patient's assessment. Agreement between scores of physician-reported and patient-reported joint counts was assessed using ICC. Kappa statistics were used to assess reliability of scoring individual joints. The study included 75 patients with JIA. In general, patients had a low number of active joints (median 1 joint, indicated by the physician). ICC was moderate (0.61) and κ ranged from 0.3-0.7. At the second visit, κ were similar; the ICC was 0.19. When a patient scored 0 joints, the physician confirmed this 93%-100% of the time. When the patient marked ≥ 1 joints, the physician confirmed arthritis 59%-76% of the time. Sensitivity to change was moderate. Agreement between physician and patient on the number of joints with active arthritis was reasonable. Untrained patients tended to overestimate the presence of arthritis when they marked active joints on a manikin-format joint count. When the patient indicated absence of arthritis, the physician usually confirmed this. As the agreement did not improve at followup, future research should focus on the possibility of achieving this through training. For now, the patient-reported joint count cannot replace the physicians' joint count in clinical practice; it may be used in epidemiological studies with caution.

  19. Idiopathic transient osteoporosis of the talus: a cause for unexplained foot and ankle pain.

    PubMed

    Limaye, Rajiv; Tripathy, Sujit Kumar; Pathare, Sanjay; Saeed, Kamran

    2012-01-01

    A 53-year-old woman was investigated for several neoplastic, inflammatory, and infective conditions for her left foot, and ankle pain associated with swelling, which she developed unexpectedly without history of trauma or infection. Gross osteopenia in the talus raised the possibilities of several differential diagnoses, but a magnetic resonance imaging scan showed diffuse bone marrow edema in the talus. With negative infective and inflammatory markers, the condition was ultimately labeled as "transient osteoporosis." She was reassured and followed up regularly. At the end of 12 months, she was completely asymptomatic, and her radiograph and magnetic resonance images showed significant improvement, with a normal-appearing talus and ankle joint, and there was complete resolution of bone marrow edema. Although "transient osteoporosis" of the foot is an uncommon condition, clinicians should be aware of this. Unexplained foot pain, with osteopenic bone and diffuse bone marrow edema on magnetic resonance imaging scan, is a feature of this condition. However, the diagnosis is established once other causes are excluded. The condition is self-limiting, and watchful expectancy of a normal recovery is the mainstay of treatment. Copyright © 2012 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

  20. 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

  1. Three-dimensional morphological condylar and mandibular changes in a patient with juvenile idiopathic arthritis: interdisciplinary treatment.

    PubMed

    Farronato, G; Bellintani, C; Garagiola, U; Cressoni, P; Sarzi Puttini, P; Atzeni, F; Cazzola, M

    2014-11-06

    Temporomandibular joint (TMJ) involvement is common but usually delayed in patients with juvenile idiopathic arthritis (JIA). We describe the case of a JIA patient with bilateral TMJ involvement, mandibular retrognathia, bone erosion, and severely restricted mouth opening. The use of cone beam computed tomography and a 3D diagnostic protocol in young patients with JIA provides reliable, accurate and precise quantitative data and images of the condylar structures and their dimensional relationships. Analgesics and conventional disease modifying antirheumatic drugs were ineffective, but interdisciplinary treatment with etanercept and a Herbst functional appliance improved functional TMJ movement and bone resorption.

  2. The novel use of combined IL-1 and IL-6 inhibition in a patient with severe, aggressive, erosive, systemic-onset juvenile idiopathic arthritis.

    PubMed

    Lee, Martin; Isaacs, John

    2017-03-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a form of juvenile idiopathic arthritis (JIA) that typically presents with prominent systemic features and accounts for approximately 10-15% of children with JIA. Pro-inflammatory cytokine pathways are thought to be involved in its pathogenesis, including interleukin-1 (IL-1) and interleukin-6 (IL-6), and laboratory tests demonstrate a prominent inflammatory response with high CRP and ferritin levels. We present a case of severe, aggressive, erosive SoJIA in a 17-year-old male resistant to multiple biologic therapies treated with a novel combination of IL-1 and IL-6 blockade along with subcutaneous methotrexate.

  3. The novel use of combined IL-1 and IL-6 inhibition in a patient with severe, aggressive, erosive, systemic-onset juvenile idiopathic arthritis

    PubMed Central

    Lee, Martin; Isaacs, John

    2017-01-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a form of juvenile idiopathic arthritis (JIA) that typically presents with prominent systemic features and accounts for approximately 10–15% of children with JIA. Pro-inflammatory cytokine pathways are thought to be involved in its pathogenesis, including interleukin-1 (IL-1) and interleukin-6 (IL-6), and laboratory tests demonstrate a prominent inflammatory response with high CRP and ferritin levels. We present a case of severe, aggressive, erosive SoJIA in a 17-year-old male resistant to multiple biologic therapies treated with a novel combination of IL-1 and IL-6 blockade along with subcutaneous methotrexate. PMID:28293458

  4. Subtype specific genetic associations for juvenile idiopathic arthritis: ERAP1 with the enthesitis related arthritis subtype and IL23R with juvenile psoriatic arthritis

    PubMed Central

    2011-01-01

    Introduction Juvenile idiopathic arthritis (JIA) is an umbrella term for all chronic childhood arthropathies and can be divided into seven subtypes. It includes the enthesitis related arthritis (ERA) subtype which displays symptoms similar to ankylosing spondylitis (AS) and juvenile-onset psoriatic arthritis which has similarities to psoriatic arthritis (PsA) and psoriasis (Ps). We, therefore, hypothesized that two well-established susceptibility loci for AS and Ps, ERAP1 and IL23R, could also confer susceptibility to these JIA subtypes. Methods Single nucleotide polymorphisms (SNPs) in ERAP1 (rs30187) and IL23R (rs11209026) were genotyped in JIA cases (n = 1,054) and healthy controls (n = 5,200). Genotype frequencies were compared between all JIA cases and controls using the Cochrane-Armitage trend test implemented in PLINK. Stratified analysis by ILAR subtype was performed. Results The ERA subtype showed strong association with ERAP1 SNP (P trend = 0.005). The IL23R SNP showed significant association in the PsA subtype (P trend = 0.04). The SNPs were not associated with JIA overall or with any other subtype. Conclusions We present evidence for subtype specific association of the ERAP1 gene with ERA JIA and the IL23R gene with juvenile-onset PsA. The findings will require validation in independent JIA datasets. These results suggest distinct pathogenic pathways in these subtypes. PMID:21281511

  5. Agreement between proxy and adolescent assessment of disability, pain, and well-being in juvenile idiopathic arthritis.

    PubMed

    Lal, Sham D; McDonagh, Janet; Baildam, Eileen; Wedderburn, Lucy R; Gardner-Medwin, Janet; Foster, Helen E; Chieng, Alice; Davidson, Joyce; Adib, Navid; Thomson, Wendy; Hyrich, Kimme L

    2011-02-01

    Adolescents with juvenile idiopathic arthritis have demonstrated substantial disagreement with their proxy's assessment of their disability, pain, and well-being. Our objective was to describe the clinical and psychological factors associated with discordance. This analysis included 204 proxy-adolescent (median age, 13 years) dyads that completed a Childhood Health Assessment Questionnaire for disability with 100-mm visual analogue scales for pain and well-being. Depressive symptoms in adolescents were measured by the Mood and Feelings Questionnaire and in proxies the General Health Questionnaire. Disagreement was assessed using Bland-Altman plots. Associations with discordance were identified using logistic regression analyses. There was higher agreement for disability (84%) than for pain (71%) and well-being (66%). Regression analyses found no association between age, sex, or disease duration and disagreement. However, relationships between disease activity and disagreement in outcomes were identified. Independent associations were found between increasing Mood and Feelings Questionnaire scores and disagreement in pain and well-being. Proxy and adolescent reports of pain and well-being are more likely to disagree in those with severe disease. Adolescents who report depressive symptoms are also more likely to disagree with their proxy. The reasons for these are multifactorial, and considerations of both reports are important when assessing outcomes in juvenile idiopathic arthritis. Copyright © 2011 Mosby, Inc. All rights reserved.

  6. Non-HLA gene polymorphisms in juvenile idiopathic arthritis: associations with disease outcome.

    PubMed

    Alberdi-Saugstrup, M; Enevold, C; Zak, M; Nielsen, S; Nordal, E; Berntson, L; Fasth, A; Rygg, M; Müller, K

    2017-09-01

    To test the hypothesis that non-HLA single-nucleotide polymorphisms (SNPs) associated with the risk of juvenile idiopathic arthritis (JIA) are risk factors for an unfavourable disease outcome at long-term follow-up. The Nordic JIA cohort is a prospective multicentre study cohort of patients from the Nordic countries. In all, 193 patients met the inclusion criteria of having an 8 year follow-up assessment and available DNA sample. Seventeen SNPs met the inclusion criteria of having significant associations with JIA in at least two previous independent study cohorts. Clinical endpoints were disease remission, actively inflamed joints and joints with limitation of motion (LOM), articular or extra-articular damage, and history of uveitis. Evidence of associations between genotypes and endpoints were found for STAT4, ADAD1-IL2-IL21, PTPN2, and VTCN1 (p = 0.003-0.05). STAT4_rs7574865 TT was associated with the presence of actively inflamed joints [odds ratio (OR) 20.6, 95% confidence interval (CI) 2.2-> 100, p = 0.003] and extra-articular damage (OR 7.9, 95% CI 1-56.6, p = 0.057). ADAD1_rs17388568 AA was associated with a lower risk of having joints with LOM (OR 0.1, 95% CI 0-0.55, p = 0.016). PTPN2_rs1893217 CC was associated with a lower risk of having joints with LOM (OR 0.2, 95% CI 0-0.99, p = 0.026), while VTCN1_rs2358820 GA was associated with uveitis (OR 3.5, 95% CI 1-12.1, p = 0.029). This exploratory study, using a prospectively followed JIA cohort, found significant associations between long-term outcome and SNPs, all previously associated with development of JIA and involved in immune regulation and signal transduction in immune cells.

  7. 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

  8. HLA II class alleles in juvenile idiopathic arthritis patients with and without temporomandibular joint arthritis.

    PubMed

    Dāvidsone, Zane; Eglīte, Jeļena; Lazareva, Arina; Dzelzīte, Sarmīte; Šantere, Ruta; Bērziņa, Dace; Staņēviča, Valda

    2016-04-19

    Temporomandibular joint (TMJ) arthritis is seen very often (38-87 %) in children with juvenile idiopathic arthritis (JIA). With contrast enhanced magnetic resonance imaging (MRI) we can detect more cases of TMJ arthritis than ever before. Previous studies show that HLA II class alleles may have protective or risk importance in JIA subtypes. Our objective is to identify HLA II class alleles of risk and protection in JIA patients with TMJ arthritis. During the period from 2010 to 2015 MRI for TMJ was performed in 85 JIA patients who were genotyped for HLA- DRB1; DQB1 and DQA1 using RT-PCR with sequence-specific primers. As a control group, data of 100 individuals were taken from the genetic bank of RSU Joint Laboratory of Clinical Immunology and Immunogenetics. Associations of DRB1; DQB1; DQA1 alleles in patients were examined individually using the χ (2) test. P-value (<0.05) and odds ratio were calculated using EPI INFO 6.0 software. Out of 85 JIA patients with mean age of 13.7 ± 3.0 years (range 6.9-17.9 years), 59 (69 %) were girls and 26 (31 %) were boys. The mean duration of the disease was 3.07 ± 2.35 years (range 0.2-11.0 year). JIA subtypes were as follows: seronegative polyarthritis 51 (60 %), seropositive polyarthritis 6(7 %), oligoarthritis extended 7(8 %), oligoarthritis persistent 2 (2 %) arthritis with enthesitis 14 (17 %), undifferentiated 3 (4 %) and 2 (2 %) systemic arthritis. Two groups where separated after TMJ MRI exam: first with at least two signs of active inflammation and/or any structural damage (n = 62); second with no pathologic signs or with slight contrast enhancement (n = 23). We discovered that there are risk alleles that are found in all JIA patient's groups (MRI positive and negative groups) versus controls such as DRB1*07:01, DQB1*03:03; DQB1*05:01. Also some protective alleles as DRB1*18:01, DQB1*06:02-8 were found in overall JIA group. Alleles DRB1*12:01, DQB1*03:01; DQA1*05:01 were found to be

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

    PubMed Central

    2017-01-01

    Objectives 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. Material and methods 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. Results 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). Conclusions 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. PMID:28386137

  10. 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

  11. Are panoramic radiographs predictive of temporomandibular joint synovitis in children with juvenile idiopathic arthritis?

    PubMed

    Abramowicz, Shelly; Simon, Lisa E; Susarla, Harlyn K; Lee, Edward Y; Cheon, Jung-Eun; Kim, Susan; Kaban, Leonard B

    2014-06-01

    To identify specific panoramic radiographic findings associated with temporomandibular joint (TMJ) synovitis in children with juvenile idiopathic arthritis (JIA). This was a retrospective study of children with JIA evaluated at Boston Children's Hospital. Patients were included if they had a confirmed diagnosis of JIA, a panoramic radiograph, and a contemporaneous TMJ magnetic resonance imaging (MRI) study with contrast. Medical records and imaging studies were reviewed to document demographic, panoramic (accentuated antegonial notch, short ramus and condyle unit [RCU] length, and abnormal condyle morphology: decreased condyle anteroposterior or superoinferior dimension) and MRI findings. The outcome variable was the presence or absence of TMJ synovitis on MRI. Descriptive and bivariate statistics and logistic regression models were used to identify associations (significant at P ≤ .05). Thirty patients (21 girls) with a mean age of 11.1 years (range, 5 to 16 yr) met the inclusion criteria. Of these, 15 patients had MRI scans positive for synovitis (bilateral in 18 joints in 9 patients and unilateral in 6 joints in 6 patients). The remaining 15 patients did not have evidence of synovitis on MRI. In the synovitis group, 18 of 24 joints (75%) showed abnormal panoramic findings (abnormal condyle morphology in 18 joints, accentuated antegonial notch in 9 joints, or short RCU length in 5 joints). In the nonsynovitis group, 15 of 36 joints (42%) showed abnormal panoramic findings (abnormal condyle morphology in 12 joints, accentuated antegonial notch in 6 joints, or short RCU length in 4 joints). Abnormal condyle morphology and accentuated antegonial notching on panoramic radiographs were found to be significantly correlated with synovitis (P = .0005 and .044, respectively). In a logistic regression model, abnormal condyle morphology was significantly associated with an increase in likelihood of TMJ synovitis versus those joints with normal condyle morphology (P = .007

  12. 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.

  13. 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

  14. Juvenile idiopathic arthritis in two tertiary centres in the Western Cape, South Africa

    PubMed Central

    2012-01-01

    Background Juvenile idiopathic arthritis (JIA) is a disease that shows wide variations between differing populations. Since the recent international consensus on classification criteria, JIA has been widely described in many countries and population groups. There has been almost no data that describes JIA in an African, specifically Sub-Saharan African, setting. Therefore, the aim of this study is to describe disease characteristics, disease course, and functional disability in two tertiary centres in the Western Cape, South Africa and compare the findings to other JIA populations. Methods Eighty-six children were recruited during random clinic visits to rheumatology clinics at Tygerberg and Groote Schuur Hospital between April 2010 and April 2011. Children were diagnosed using International League of Associations for Rheumatology (ILAR) 2001 classification criteria. Consent was obtained and medical records examined. The Childhood Health Assessment Questionnaires (CHAQ) and visual analogue scales (VAS) for pain and general well-being were completed and all children were examined by a researcher in conjunction with a paediatric rheumatologist. HIV status as well as tuberculosis disease and treatment were investigated. Results A total of 86 children were enrolled. Eight children were excluded (2 HIV arthropathy, 1 TB arthritis, 1 SLE, 4 with insufficient data), leaving a total of 78 patients. There was an equal female to male ratio-39 males and 39 females. There were 6 systemic JIA patients (7.69%), 17 persistent oligoarthritis (21.79%), 4 extended oligoarthritis (5.12%), 11 polyarthritis rheumatoid factor (RF) positive (14.10%), 21 polyarthritis RF negative (26.9%), 1 psoriatic arthritis (1.28%), and 18 enthesitis-related arthritis (23%). The median CHAQ for the group was 0.5 (IQR 0.1-1.25), the median VAS for pain was 18 mm (IQR 4–42) and median VAS for general well-being was 25 mm (IQR 3–49). Enthesitis-related arthritis and polyarthritis disease subtypes in

  15. HLA B27 allele types in homogeneous groups of juvenile idiopathic arthritis patients in Latvia

    PubMed Central

    2010-01-01

    Juvenile idiopathic arthritis (JIA) is a heterogeneous condition and therapeutic strategies vary in different JIA types. The routinely accepted practice to start with Sulphasalazine (SS) as the first line treatment in patients with HLA B27 positive JIA proves to be ineffective in a large proportion of children. Objective to investigate HLA B27 positive JIA patients clinical characteristics, determined HLA B27 allele types and their connection with antirheumatic treatment in homogenous patient groups. Materials and methods 56 patients diagnosed with JIA and observed over the period 2006 to 2009 included in the study. HLAB27 allele types were determined using PCR method. Results In HLA B27 positive JIA patients mean disease onset was 12.34 ± 3.3 years. Most common (44%) JIA type was enthesitis related arthritis. Positive response to the treatment with SS was found in 32% of patients, Methotrexate (MTX) - in 43%, combined treatment - SS with MTX was effective in 12.5%. 12.5% of patients required combination MTX with Enbrel. Eight HLA B27 allele types were found in JIA patients in Latvia: *2702, *2703, *2704, *2705, *2710, *2715, *2717, *2728. The most common was *2705 - in 55% of cases. Among all the patients enthesitis related arthritis most commonly occurred in patients with HLAB*2705 allele (OR = 2.01, p < 0.02), oligoarthritis in patients with *2710 allele (OR = 3.0, p < 0.04) and polyarthritis with *2717 allele (OR = 3.0, p < 0.05). In patients with *2705 allele effective treatment was MTX (OR = 1.13, p < 0.03) and MTX with SS (OR = 2.02, p < 0.05), but in patients having *2703 allele - MTX with Enbrel (OR = 2.94, p < 0.02). Conclusions There are 8 different HLA B27 alleles in JIA patients in Latvia and the most common is *2705, but in order to assert them to be disease associated alleles, more extensive studies are needed, including control group of HLA B27 positive healthy individuals. Standard treatment approach with SS proves to be unsatisfactory in the

  16. Intraarticular corticosteroid injections of the temporomandibular joint in juvenile idiopathic arthritis.

    PubMed

    Ringold, Sarah; Torgerson, Troy R; Egbert, Mark A; Wallace, Carol A

    2008-06-01

    To describe the clinical and radiographic outcomes in a series of patients with juvenile idiopathic arthritis (JIA) who underwent one or more intraarticular corticosteroid (IAS) injections of the temporomandibular joint (TMJ) performed without imaging guidance. Retrospective chart review was performed for all patients with JIA diagnosed and treated at our institution between January 1, 2000, and January 1, 2006, who underwent one or more IAS injections of their TMJ. IAS injections were performed by the same oral and maxillofacial surgeon without imaging guidance, using either triamcinolone acetonide or triamcinolone hexacetonide. The primary outcomes assessed were maximal incisal opening (MIO) measurements, patient-reported symptoms, physical examination findings, and imaging results. Twenty-five patients were identified. Twenty-one (84%) had radiographic evidence of TMJ disease when TMJ disease was first suspected by their physician. The 25 patients underwent 74 IAS injections on 47 separate occasions. When baseline MIO measurements were compared to the last MIO measurements of the study period, there was a mean increase in MIO of 6.9 mm (p = 0.002; 95% CI 3, 10.7). There was a mean increase in MIO of 3.8 mm following each IAS injection (p = 0.003; 95% CI 1.4, 6.2). Patients who underwent multiple IAS injections had a mean increase in MIO after first injection of 6.6 mm (p < 0.001; 95% CI 4.1, 9.1); however, the mean increase in MIO after subsequent injections was 0.4 mm (p = 0.8; 95% CI -3.5, 4.4). One patient developed subcutaneous atrophy at the injection site. Two patients developed small, asymptomatic intraarticular calcifications. No additional adverse events were reported. In this patient population, there was an overall increase in MIO measurements following initial IAS injection and during the study period. Patients tended to have minimal response to subsequent injections. IAS injections performed without imaging guidance by an experienced oral and

  17. Effect of strengthening versus balance-proprioceptive exercises on lower extremity function in patients with juvenile idiopathic arthritis: a randomized, single-blind clinical trial.

    PubMed

    Baydogan, Saime Nilay; Tarakci, Ela; Kasapcopur, Ozgur

    2015-06-01

    The aim of this study was to investigate the effects of two exercise programs on lower extremity function in patients with juvenile idiopathic arthritis. Thirty patients with juvenile idiopathic arthritis participated in this study. Pain, passive range of motion, muscle strength, balance, and functional abilities were assessed with the Numeric Rating Scale, goniometer, handheld dynamometer, Flamingo Balance Test, Functional Reach Test, 10-meter walking test, 10-stair climbing test, and Childhood Health Assessment Questionnaire. Participants were randomly assigned to the strengthening exercise group (group 1, n = 15) or the proprioceptive-balance exercise group (group 2, n = 15). Intragroup analysis showed statistically significant improvements in all outcome measures except muscle strength in the hip and ankle after strengthening exercises in group 1. However, statistically significant improvements were found in all outcome measures after the proprioceptive-balance exercises in group 2. Intergroup analysis showed statistically significant improvement in all outcome measures in group 2 except for the Numeric Rating Scale, Childhood Health Assessment Questionnaire, and passive range of motion scores and hip extension and knee flexion muscle strengths. This study demonstrates that exercise treatment significantly improves musculoskeletal symptoms in patients with juvenile idiopathic arthritis. However, balance-proprioceptive exercises prove to be effective more than strengthening exercises for improving lower extremity function such as walking, climbing stairs, and balance in patients with juvenile idiopathic arthritis.

  18. 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.

  19. Severe idiopathic hypocalcemia in a juvenile western lowland gorilla, Gorilla gorilla gorilla.

    PubMed

    Chatfield, Jenifer; Stones, Greeley; Jalil, Tania

    2012-03-01

    A 6-mo-old, male western lowland gorilla (Gorilla gorilla gorilla) was evaluated because of tetany of both hands. The gorilla had alternating periods of constipation, diarrhea, and bloating since birth. A diagnosis of idiopathic hypocalcemia was based on severe hypocalcemia, a normal vitamin D level, response to oral calcium and vitamin D therapy, and eventual resolution. Idiopathic hypocalcemia, an uncommon disease in neonatal humans, should be considered in young gorillas with persistent gastrointestinal problems or acute tetany.

  20. Disk abnormality coexists with any degree of synovial and osseous abnormality in the temporomandibular joints of children with juvenile idiopathic arthritis.

    PubMed

    Kirkhus, Eva; Arvidsson, Linda Z; Smith, Hans-Jørgen; Flatø, Berit; Hetlevik, Siri O; Larheim, Tore A

    2016-03-01

    MRI manifestation of temporomandibular joint arthritis is frequently reported in children with juvenile idiopathic arthritis. However, little attention has been paid to temporomandibular joint disk abnormalities. To assess combinations of MRI findings in the symptomatic temporomandibular joint in children with juvenile idiopathic arthritis with focus on disk abnormalities. This was a retrospective study of 46 patients with juvenile idiopathic arthritis, mean age 12 years (range: 5-17 years). Mean disease duration was 70 months (standard deviation: 61 months). MR images of 92 temporomandibular joints were scored for thickness of abnormally enhancing synovium (synovitis), joint effusion, bone marrow oedema, abnormal bone shape, bone erosion and disk abnormalities. The 92 temporomandibular joints were categorized as A: No synovitis and normal bone shape (30/92; 33%), B: Synovitis and normal bone shape (14/92: 15%), C: Synovitis and abnormal bone shape (38/92; 41%) and D: No synovitis but abnormal bone shape (10/92; 11%). Thirty-six of the 46 patients (78%) had synovitis and 33/46 (72%) had abnormal bone shape, most frequently in combination (30/46; 65%). Disk abnormalities (flat disk, fragmented disk, adherent disk and displaced disk) were found in 29/46 patients (63%). Disk abnormalities were found in all categories of juvenile idiopathic arthritis involved temporomandibular joints (B: 8/14 [57%]; C: 25/38 [66%] and D: 7/10 [70%]). Disk displacement was found in half of the joints (7/14) in category B. Synovitis was most pronounced in this category. Disk abnormalities were frequent. Disk displacement also occurred in joints with early temporomandibular joint arthritis, i.e., with normal bone shape. Other disk abnormalities were found in joints with bone abnormalities. Attention should be paid to disk abnormalities both in early and long-standing temporomandibular joint arthritis in children with juvenile idiopathic arthritis.

  1. Usability Testing of an Online Self-management Program for Adolescents With Juvenile Idiopathic Arthritis

    PubMed Central

    McGrath, Patrick; Hodnett, Ellen; Feldman, Brian; Duffy, Ciaran; Huber, Adam; Tucker, Lori; Hetherington, Ross; Tse, Shirley; Spiegel, Lynn; Campillo, Sarah; Gill, Navreet; White, Meghan

    2010-01-01

    Background A new bilingual (English and French) Internet-based self-management program, Teens Taking Charge: Managing Arthritis Online, for adolescents with arthritis and their parents was developed following a needs assessment. Objectives This study explored the usability (user performance and satisfaction) of the self-management program for youth with juvenile idiopathic arthritis (JIA) and their parents to refine the health portal prototype. Methods A qualitative study design with semi-structured, audio taped interviews and observation by a trained observer was undertaken with two iterative cycles to determine the usability (ease of use, efficiency, errors, and user satisfaction) of the user interface and content areas of the intervention. A purposive sample of English-speaking (n = 11; mean age = 15.4, standard deviation [SD] 1.7) and French-speaking (n = 8; mean age = 16.0, SD 1.2) adolescents with JIA and one of their respective parents/caregivers were recruited from 2 Canadian tertiary care centers. Descriptive statistics and simple content analyses were used to organize data into categories that reflected the emerging usability themes. Results All of the participants had access to a computer/Internet at home; however, adolescents were more comfortable using the computer/Internet than their parents. Adolescents and parents provided similar as well as differing suggestions on how the website user interface could be improved in terms of its usability (navigation; presentation and control usage errors; format and layout; as well as areas for further content development). There were no major differences in usability issues between English- and French-speaking participants. Minor changes to the website user interface were made and tested in a second cycle of participants. No further usability problems were identified in the second iterative cycle of testing. Teens and parents responded positively to the appearance and theme of the website (ie, promoting self

  2. 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.

  3. Extremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases

    PubMed Central

    Xia, Y.; Cui, P.; Li, Q.; Liang, F.; Li, C.; Yang, J.

    2017-01-01

    The aim of this research was to explore whether IL-18 can be a serological marker for the diagnosis of systemic-onset juvenile idiopathic arthritis (sJIA). A total of 23 sJIA patients (13 males, median age 8.2), 20 acute lymphoblastic leukemia (ALL) patients, 18 patients with severe infections (SIF), 26 Kawasaki disease (KD) patients, 18 juvenile idiopathic arthritis (JIA) patients, and 25 healthy control patients were selected for this study. Enzyme-linked immunosorbent assays (ELISAs) were used to determine the serum concentrations of the S100A8, S100A9, and IL-6 proteins. The serum IL-18 levels were detected by a cytometric bead array (CBA). The serum IL-6 concentrations in various disease groups were significantly higher than that in the healthy control group. The IL-6 concentrations exhibited no significant difference between disease groups. The S100A8 level in the sJIA group was significantly higher than those of the ALL, JIA, and healthy control groups but showed no significant difference compared to the SIF and KD groups. The S100A9 serum concentration in the sJIA group was significantly higher than those in the ALL and healthy control groups and exhibited no significant difference from the SIF, KD, and JIA groups. The IL-18 level of the sJIA group was significantly higher than that of the other febrile disease groups. The IL-18 serum concentration may be used as a biological serum marker to distinguish sJIA from other febrile diseases. PMID:28225869

  4. 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.

  5. Effects of the use of growth hormone in children and adolescents with juvenile idiopathic arthritis: a systematic review.

    PubMed

    Frittoli, Renan Bazuco; Longui, Barbara Sugui; Silva, Amanda Meireles; Filho, Antônio de Azevedo Barros; Monteiro, Maria Ângela Reis de Góes; Appenzeller, Simone

    Children with juvenile idiopathic arthritis (JIA) often have impaired growth and short stature. There is evidence that the therapeutic use of growth hormone (GH) is useful and safe in these patients. To analyze the effects of GH use in patients with JIA. A systematic review of the literature over the last 18 years in Medline and Embase databases. The criteria were analyzed independently by the researchers. We used the following keywords: "growth hormone", "arthritis, juvenile", "arthritis, rheumatoid", "child" and "adolescent". Among the 192 identified articles, 20 corresponded to the inclusion criteria. Seventeen longitudinal studies and 3 case reports were found. Most studies analyzed observed increased growth, muscle mass and bone mass using GH. Adverse effects observed were glucose intolerance, diabetes, bone deformities, osteonecrosis, reactivation of the disease and low final height. The majority of studies reported positive effects after the therapeutic use of GH, but some variability in response to treatment was observed. The combination of growth hormone with other drugs seems to be a good option. Copyright © 2016. Published by Elsevier Editora Ltda.

  6. Effects of the use of growth hormone in children and adolescents with juvenile idiopathic arthritis: a systematic review.

    PubMed

    Frittoli, Renan Bazuco; Longui, Barbara Sugui; Silva, Amanda Meireles; Filho, Antônio de Azevedo Barros; Monteiro, Maria Ângela Reis de Góes; Appenzeller, Simone

    2016-07-09

    Children with Juvenile Idiopathic Arthritis (JIA) often have impaired growth and short stature. There is evidence that the therapeutic use of growth hormone (GH) is useful and safe in these patients. To analyze the effects of GH use in patients with JIA. A systematic review of the literature over the last 18 years in Medline and Embase databases. The criteria were analyzed independently by the researchers. We used the following keywords: "growth hormone", "arthritis, juvenile", "arthritis, rheumatoid", "child" and "adolescent". Among the 192 identified articles, 20 corresponded to the inclusion criteria. Seventeen longitudinal studies and 3 case reports were found. Most studies analyzed observed increased growth, muscle mass and bone mass using GH. Adverse effects observed were glucose intolerance, diabetes, bone deformities, osteonecrosis, reactivation of the disease and low final height. The majority of studies reported positive effects after the therapeutic use of GH, but some variability in response to treatment was observed. The combination of growth hormone with other drugs seems to be a good option. Copyright © 2016. Published by Elsevier Editora Ltda.

  7. 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

  8. [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.

  9. Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-up study.

    PubMed

    Ekelund, Maria; Aalto, Kristiina; Fasth, Anders; Herlin, Troels; Nielsen, Susan; Nordal, Ellen; Peltoniemi, Suvi; Rygg, Marite; Zak, Marek; Berntson, Lillemor

    2017-02-22

    To study the impact of psoriasis and features associated with psoriasis on classification and outcome in a population-based follow-up cohort of children with juvenile idiopathic arthritis (JIA). In all, 440 children with JIA were followed for a median of 8 years in a prospective Nordic population-based cohort study. Data for remission was available for 427 of these children. The presence of psoriasis, psoriasis-like rash, dactylitis, nail pitting, enthesitis, tenosynovitis and heredity was assessed in relation to ILAR classification and remission. Clinical findings associated with psoriasis developed consecutively during the 8-year period. Six of 14 children with psoriasis were not classified as juvenile psoriatic arthritis according to the ILAR criteria at 8 year follow-up. Dactylitis was more common in children with early onset of JIA. After 8 years we found a cumulative median number of eleven arthritic joints in children with psoriasis or psoriasis-like rash compared with six in the rest of the cohort (p = 0.02). Also, the chance for not being in remission after 8 years increased significantly in patients with psoriasis, psoriasis-like rash or at least two of: 1) first-degree heredity for psoriasis or psoriatic arthritis, 2) dactylitis or 3) nail pitting, compared with the rest of the group (OR 3.32, p = 0.010). Our results indicate a more severe disease over time in psoriasis-associated JIA, as features of psoriasis develop during the disease course. This group is a major challenge to encompass in a future JIA classification in order to facilitate early tailored treatment.

  10. How common is clinically inactive disease in a prospective cohort of patients with juvenile idiopathic arthritis? The importance of definition.

    PubMed

    Shoop-Worrall, Stephanie J W; Verstappen, Suzanne M M; Baildam, Eileen; Chieng, Alice; Davidson, Joyce; Foster, Helen; Ioannou, Yiannis; McErlane, Flora; Wedderburn, Lucy R; Thomson, Wendy; Hyrich, Kimme L

    2017-08-01

    Many criteria for clinically inactive disease (CID) and minimal disease activity (MDA) have been proposed for juvenile idiopathic arthritis (JIA). It is not known to what degree each of these criteria overlap within a single patient cohort. This study aimed to compare the frequency of MDA and CID across different criteria in a cohort of children with JIA at 1 year following presentation. The Childhood Arthritis Prospective Study recruits children at initial presentation to paediatric or adolescent rheumatology in seven UK centres. Children recruited between October 2001 and December 2013 were included. The proportions of children with CID and MDA at 1 year were calculated using four investigator-defined and eight published composite criteria. Missing data were accounted for using multiple imputation under different assumptions. In a cohort of 1415 children and adolescents, 67% patients had no active joints at 1 year. Between 48% and 61% achieved MDA and between 25% and 38% achieved CID using published criteria. Overlap between criteria varied. Of 922 patients in MDA by either the original composite criteria, Juvenile Arthritis Disease Activity Score (JADAS) or clinical JADAS cut-offs, 68% were classified as in MDA by all 3 criteria. Similarly, 44% of 633 children with CID defined by either Wallace's preliminary criteria or the JADAS cut-off were in CID according to both criteria. In a large JIA prospective inception cohort, a majority of patients have evidence of persistent disease activity after 1 year. Published criteria to capture MDA and CID do not always identify the same groups of patients. This has significant implications when defining and applying treat-to-target strategies. 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/.

  11. 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

  12. Blood gene expression profiling in pediatric systemic lupus erythematosus and systemic juvenile idiopathic arthritis: from bench to bedside.

    PubMed

    Gilbert, Mileka; Punaro, Marilynn

    2014-01-01

    Blood gene expression profiling has led to major advances in the field of rheumatology over the last few decades. Specifically, DNA microarray technology has been integral in increasing our knowledge of key players in the pathogenesis of some rare pediatric rheumatic diseases. Our group, using microarray analysis, identified the interferon (IFN) gene signature in pediatric systemic lupus erythematosus (SLE) and has published data that suggest high doses of intravenous corticosteroid treatment may have benefit over strictly oral regimens. Additionally, DNA microarray technology led to our discovery that the interleukin (IL)-1 gene signature is associated with systemic juvenile idiopathic arthritis (sJIA) and to the use of IL-1 blockade with anakinra in this disease. We also reported the biologic rationale for use of anakinra early in the disease course. Anakinra is now being used as first-line treatment in sJIA in multiple centers. Herein, we review how information obtained from blood gene expression profiling has changed our clinical practice.

  13. Ankylosis of the Temporomandibular Joint and Reconstruction With a Costochondral Graft in a Patient With Juvenile Idiopathic Arthritis.

    PubMed

    Felix, Valtuir Barbosa; Cabral, Dhayanna Rolemberg Gama; de Almeida, Alana Beatriz Wanderley; Soares, Elionai Dias; de Moraes Fernandes, Katharina Jucá

    2017-01-01

    Juvenile idiopathic arthritis (JIA), the most common inflammatory autoimmune rheumatic disease in children, consists of a heterogeneous group of diseases with 7 distinct subtypes. Involvement of the temporomandibular joint (TMJ) in JIA varies from 17% to 87%, and can alter craniofacial growth due to damage to the condylar growth center. This study was a literature review and clinical report of bilateral ankylosis of the TMJ in a 13-year-old patient with polyarticular JIA. Temporomandibular joint reconstruction with a costochondral graft was carried out. The surgery was uneventful and the patient developed a mouth opening of 40 mm during the postoperative period of 24 months. The authors concluded that treatment of TMJ ankylosis should be surgical with removal of the ankylotic mass, and when necessary, joint reconstruction in patients undergoing a growth phase. Costochondral graft is still the gold standard due to its biological similarity and growth potential in patients with JIA. Research and early diagnosis of TMJ diseases should be carried out, because the earlier the identification of the disease, the better the chances of reducing its devastating effects, thus avoiding the worst possible outcome: TMJ ankylosis.

  14. Oral health and orthodontic considerations in children with juvenile idiopathic arthritis: review of the literature and report of a case.

    PubMed

    Synodinos, Philippos N; Polyzois, Ioannis

    2008-01-01

    Juvenile idiopathic arthritis (JIA) is a severe disease of childhood, which comprises a diverse group of distinct clinical entities of unclear aetiology. Some abnormality of the immune system is present in all JIA cases. In its most severe clinical form, JIA may show localised and/or systemic complications, including functional impairment of the affected sites. This may result in variable growth and developmental anomalies. In many JIA cases, where the temporomandibular joint (TMJ) is affected, mandibular growth may be restricted, thus leading to the development of mandibular hypoplasia and/or retrognathism. As a result, it is not uncommon for JIA patients to present with skeletal Class II and open bite malocclusions. Furthermore, in JIA cases with unilateral TMJ involvement, craniofacial asymmetry may occur. In such cases, early orthodontic intervention facilitates both the skeletal and the occlusal rehabilitation. Increased prevalence of dental caries and periodontal disease in JIA cases may be attributed to a combination of aetiological factors, including difficulties in executing good oral hygiene, unfavourable dietary practices and side effects from the long-term administration of medication. In addition, an association between periodontal disease and JIA has been reported based on their similar pattern of clinical disregulation of the inflammatory process. This paper presents a brief description of JIA, with special reference to dental health and orthodontic treatment considerations. In addition, a case is presented where the appropriate orthodontic intervention led to the establishment of a normally functioning, as well as an aesthetically pleasing, occlusion.

  15. Behavioral Problems in Juvenile Idiopathic Arthritis: A Controlled Study to Examine the Risk of Psychopathology in a Chronic Pediatric Disorder

    PubMed Central

    Chamanara, Elham; Raeeskarami, Seyed-Reza

    2016-01-01

    Children with juvenile idiopathic arthritis (JIA) are prone to the problems that can delay their psychosocial development; however, the existing literature has not reached a consensus on the psychological problems related to JIA. A total of 51 children and adolescents with JIA and 75 healthy controls aged 6 to 18 years were examined using the Child Behavioral Checklist (CBCL). Our results represented that 70 percent of JIA group reached “borderline clinical” range or “clinical” range in internalizing problems, while this percentage in the control group was 18 percent. In addition, our results indicated that JIA group has gotten significantly higher scores (more than twofold) in externalizing behaviors compared to control group. Furthermore, children with JIA showed higher rate of anxiety/depression, withdrawal/depression, somatic complaints, rule breaking behaviors, and aggressive behaviors as well as thought and social problems compared to control group (p < 0.001). As a conclusion, children and adolescents with JIA compared to healthy controls may show higher rate of both internalizing and externalizing problems. Furthermore, our novel findings on externalizing, social, and thought problems in JIA warrant further investigation on affected children who may be at greater risk of future psychopathologies. PMID:27656678

  16. Juvenile idiopathic arthritis and rheumatoid arthritis: bacterial diversity in temporomandibular joint synovial fluid in comparison with immunological and clinical findings.

    PubMed

    Olsen-Bergem, H; Kristoffersen, A K; Bjørnland, T; Reseland, J E; Aas, J A

    2016-03-01

    Temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) occurs in up to 80% of affected children. The purpose of this study was to investigate the presence of bacterial DNA in synovial fluid, and to compare this with clinical and immunological findings in children with JIA, adults with persistent JIA, and adults with rheumatoid arthritis, in order to detect whether bacteria contribute to inflammation in TMJ arthritis. Synovial fluid and skin swab samples were collected from 30 patients (54 TMJs). Bacterial detection was performed using 16S rRNA pyrosequencing. Bacterial DNA was detected in 31 TMJs (57%) in 19 patients (63%). A positive statistically significant correlation was registered between bacterial DNA detected in TMJ synovial fluid and the following factors: total protein concentration in synovial fluid, interleukin 1β, tumour necrosis factor alpha, adrenocorticotropic hormone, and adiponectin, as well as the duration of the general medical disease. Fourteen different bacterial species were detected in synovial fluid. Bacterial DNA in TMJ synovial fluid without contamination was detected in more than 50% of the patients. Studies are needed to evaluate the consequences of this bacterial DNA in synovial fluid with regard to TMJ arthritis.

  17. 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.

  18. Diabetes, coeliac disease, multiple sclerosis and chronic arthritis in first-degree relatives of patients with juvenile idiopathic arthritis.

    PubMed

    Pohjankoski, Heini; Kautiainen, Hannu; Kotaniemi, Kaisu; Korppi, Matti; Savolainen, Anneli

    2012-07-01

    To evaluate the occurrence of autoimmune diseases in first-degree relatives of children with juvenile idiopathic arthritis (JIA) and to compare the figures with published population data. Families of the 362 children with recently diagnosed JIA admitted to Rheumatism Foundation Hospital, Finland, from 1996 to 2001 were contacted by questionnaires regarding autoimmune diseases in family members. Data were collected on type 1 diabetes, coeliac disease, multiple sclerosis and chronic arthritis, consisting mainly of JIA, rheumatoid arthritis, spondyloarthropathy or psoriatic arthritis. In all, 21.4% of the 355 families with a patient with JIA had members with type 1 diabetes, coeliac disease, multiple sclerosis or chronic arthritis. Thirty-three mothers and 23 fathers had type 1 diabetes, coeliac disease, multiple sclerosis or chronic arthritis in 15.2% (95% CI 11.6-19.4) of the families, and 23 mothers and 15 fathers had chronic arthritis in 10.7% (95% CI 7.7-14.5) of the families. When compared with available research data, the prevalences of rheumatoid arthritis, spondyloarthropathy, psoriatic arthritis, paediatric type 1 diabetes and JIA (in siblings) were increased in JIA families. Coeliac disease was as prevalent as in the population. Autoimmune diseases cluster in families with a child with JIA. © 2012 The Author(s)/Acta Paediatrica © 2012 Foundation Acta Paediatrica.

  19. Intravenous Laser Blood Irradiation Increases Efficacy of Etanercept in Selected Subtypes of Juvenile Idiopathic Arthritis: An Innovative Clinical Research Approach

    PubMed Central

    Weber, Michael; Ailioaie, Laura Marinela; Ailioaie, Constantin

    2013-01-01

    This single-blind, placebo-controlled study assesses the efficacy of synergic administration of intravenous laser blood irradiation (ILBI) and etanercept in selected subtypes of juvenile idiopathic arthritis (JIA). Etanercept is a tumor necrosis factor alpha blocking agent with recognized importance in JIA. Laser radiation has immunomodulatory effects in animal and human studies. Fourteen patients (Group I) received ILBI and 9 patients (Group II) received placebo laser. ILBI was performed in addition to ongoing JIA medication, including etanercept. ILBI was administrated in 3 sets of 5 consecutive daily sessions, with a 7-week interval between every set of sessions. Evaluation was performed using ACR (American College of Rheumatology) Pediatric Criteria (ACR Pedi) at study enrollment and at 10 and 20 weeks, respectively. After 10 weeks, 85.7% of the patients in Group I fulfilled Pedi 30 criteria, compared to only 55.6% of the patients in Group II. After 20 weeks, all patients in both groups had a Pedi 30 response. In Group I, 92.8% of the subjects met the Pedi 50 response, compared to only 55.6% in the placebo group. One patient in Group I responded best, fulfilling Pedi 70 criteria. If applied synergistically, ILBI and etanercept would have an increased efficacy in promoting JIA remission. PMID:23990845

  20. Network analysis and juvenile idiopathic arthritis (JIA): a new horizon for the understanding of disease pathogenesis and therapeutic target identification.

    PubMed

    Donn, Rachelle; De Leonibus, Chiara; Meyer, Stefan; Stevens, Adam

    2016-07-02

    Juvenile idiopathic arthritis (JIA) is a clinically diverse and genetically complex autoimmune disease. Currently, there is very limited understanding of the potential underlying mechanisms that result in the range of phenotypes which constitute JIA.The elucidation of the functional relevance of genetic associations with phenotypic traits is a fundamental problem that hampers the translation of genetic observations to plausible medical interventions. Genome wide association studies, and subsequent fine-mapping studies in JIA patients, have identified many genetic variants associated with disease. Such approaches rely on 'tag' single nucleotide polymorphisms (SNPs). The associated SNPs are rarely functional variants, so the extrapolation of genetic association data to the identification of biologically meaningful findings can be a protracted undertaking. Integrative genomics aims to bridge the gap between genotype and phenotype.Systems biology, principally through network analysis, is emerging as a valuable way to identify biological pathways of relevance to complex genetic diseases. This review aims to highlight recent findings in systems biology related to JIA in an attempt to assist in the understanding of JIA pathogenesis and therapeutic target identification.

  1. Multidisciplinary approach in the management of absolute trismus with bilateral temporomandibular joint replacements for a patient with juvenile idiopathic arthritis.

    PubMed

    Fanaras, Nikolaos; Parry, Nicholas S; Matthews, N Shaun

    2014-11-01

    Juvenile idiopathic arthritis (JIA) is an exclusion diagnosis that gathers together all forms of arthritis that begin before the age of 16 years, persist for more than 6 weeks and are of unknown origin. We present the case of a 42 year old woman with a 20 year history of absolute trismus, secondary to bilateral temporomandibular joint (TMJ) ankylosis caused by JIA. The trismus resulted in grossly compromised oral hygiene and limited the patient to a semi-solid diet. JIA also affected her neck leading to a severe cervico-thoracic kyphosis. The patient who had been wheelchair bound developed severe lymphoedema of both lower limbs, complicating the pre-operative work up further. This particularly challenging case required input from specialists in anaesthetics, neurosurgery, special care dentistry, intensive care and maxillofacial surgery. Treatment consisted of ankylosis release, dental clearance and bilateral alloplastic replacement of her TMJs with custom implants. A full range of hinge movement and good functional outcome was achieved. This case presents the multidisciplinary approach to a severely compromised patient and illustrates the pre-, intra- and postoperative management of bilateral TMJ ankylosis with bespoke implants. Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  2. Whole Blood Gene Expression Profiling Predicts Therapeutic Response at Six Months in Patients With Polyarticular Juvenile Idiopathic Arthritis

    PubMed Central

    Jiang, Kaiyu; Sawle, Ashley D; Frank, M Barton; Chen, Yanmin; Wallace, Carol A; Jarvis, James N

    2014-01-01

    Objective To determine whether gene expression profiles identified in peripheral whole blood samples could be used to determine therapeutic outcome in a cohort of children with newly diagnosed polyarticular juvenile idiopathic arthritis (JIA). Methods Whole blood samples from the Trial of Early Aggressive Therapy (TREAT) in JIA patients were analyzed on Illumina microarrays, and differential gene expression was compared to expression in healthy controls. Microarray results were validated by real-time quantitative polymerase chain reaction in an independent cohort of samples. Pathway analysis software was used to characterize gene expression profiles. Support vector machines were used to develop predictive models for different patient classes. Results Differential gene expression profiles for rheumatoid factor (RF)–positive and RF-negative patients were remarkably similar. Pathway analysis revealed a broad range of affected pathways, consistent with current mechanistic theories. Modeling showed that the prognosis at 6 months was strongly linked to gene expression at presentation, irrespective of treatment. Conclusion Gene expression is linked to therapeutic outcome, and gene expression in the peripheral blood may be a suitable target for a prognostic test. PMID:24782192

  3. Evaluation of anti-cyclic citrullinated peptide antibodies may be beneficial in RF-negative juvenile idiopathic arthritis patients.

    PubMed

    Spârchez, Mihaela; Miu, Nicolae; Bolba, Claudia; Iancu, Mihaela; Spârchez, Zeno; Rednic, Simona

    2016-03-01

    Despite the high diagnostic and prognostic performance in adult rheumatoid arthritis, the role of antibodies to cyclic citrullinated peptide (anti-CCP) in juvenile idiopathic arthritis (JIA) is controversial. Occurrence of anti-CCP was mainly seen in rheumatoid factor (RF)-positive polyarthritis patients. In the present study, our aim was to investigate the prevalence and significance of anti-CCP for subjects with JIA in our population. We evaluated anti-CCP reactivity in the sera of 70 patients with various subtypes of JIA in a prospective cohort study. Anti-CCP titres were correlated with the evolution of joint involvement and the presence of joint damage. Nine JIA patients were seropositive for anti-CCP with respect to the cut-off value of the test. In our cohort, 34 patients had a polyarticular joint disease, most of them being RF-negative (30/34, 88 %). All four RF-positive polyarthritis patients had high anti-CCP concentrations and an aggressive erosive disease. In the RF-negative JIA patients, anti-CCP reactivity was in lower titres but significantly associated with polyarticular joint involvement (p = 0.016) and also with the presence of joint damage (p < 0.001). Presence of anti-CCP, at both low and high concentration, was significantly associated with a more severe articular disease in our JIA patients. Investigating anti-CCP should clearly be taken into consideration even among patients with JIA subtypes other than RF-positive polyarthritis.

  4. Genetic Screening of Mutations Associated with Fabry Disease in a Nationwide Cohort of Juvenile Idiopathic Arthritis Patients

    PubMed Central

    Gonçalves, Maria J.; Mourão, Ana F.; Martinho, António; Simões, Olívia; Melo-Gomes, José; Salgado, Manuel; Estanqueiro, Paula; Ribeiro, Célia; Brito, Iva; Fonseca, João E.; Canhão, Helena

    2017-01-01

    Fabry’s disease (FD) is a lysosomal storage disorder associated with an alpha-galactosidase A deficiency. The prevalence of FD among juvenile idiopathic arthritis (JIA) patients with established diagnosis is unknown, but as musculoskeletal pain may be an important complaint at presentation, misdiagnosed cases are anticipated. With this study, we aim to calculate the frequency of FD-associated mutations in a cohort of JIA patients. Children with JIA from a national cohort were selected. Clinical and laboratorial information was recorded in the Portuguese rheumatic diseases register (http://Reuma.pt). Molecular genetic testing to detect GLA gene mutations was performed. After the multiplex polymerase chain reactions technique for DNA amplification, direct sequencing of the complete sequence of GLA gene was completed. From a cohort of 292 patients with JIA (188 females, 104 males), mutations were identified in 5 patients (all female). Four patients had the mutation D313Y, a rare GLA variant, which is associated with low enzymatic levels in plasma, but normal lysosomal levels. One patient presented the missense mutation R118C, which was previously described in Mediterranean patients with FD. This is the first screening of FD mutations in a cohort of JIA patients. No “classic” pathogenic FD mutations were reported. The late-onset FD-associated mutation, R118C, was found in a frequency of 0.34% (1/292). PMID:28299312

  5. 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.

  6. Genetic Predictors of Poor Prognosis in Portuguese Patients with Juvenile Idiopathic Arthritis: Data from Reuma.pt

    PubMed Central

    Mourão, Ana Filipa; Santos, Maria José; Mendonça, Sílvia; Oliveira-Ramos, Filipa; Salgado, Manuel; Estanqueiro, Paula; Melo-Gomes, José; Martins, Fernando; Lopes, Ana; Bettencourt, Bruno Filipe; Bruges-Armas, Jácome; Costa, José; Furtado, Carolina; Figueira, Ricardo; Brito, Iva; Branco, Jaime; Fonseca, João Eurico; Canhão, Helena

    2015-01-01

    Introduction. This study aimed to assess the genetic determinants of poor outcome in Portuguese patients with juvenile idiopathic arthritis (JIA). Methods. Our study was conducted in Reuma.pt, the Rheumatic Diseases Portuguese Register, which includes patients with JIA. We collected prospectively patient and disease characteristics and a blood sample for DNA analysis. Poor prognosis was defined as CHAQ/HAQ >0.75 at the last visit and/or the treatment with biological therapy. A selected panel of single nucleotide polymorphisms (SNPs) associated with susceptibility was studied to verify if there was association with poor prognosis. Results. Of the 812 patients with JIA registered in Reuma.pt, 267 had a blood sample and registered information used to define “poor prognosis.” In univariate analysis, we found significant associations with poor prognosis for allele A of TNFA1P3/20 rs6920220, allele G of TRAF1/C5 rs3761847, and allele G of PTPN2 rs7234029. In multivariate models, the associations with TRAF1/C5 (1.96 [1.17–3.3]) remained significant at the 5% level, while TNFA1P3/20 and PTPN2 were no longer significant. Nevertheless, none of associations found was significant after the Bonferroni correction was applied. Conclusion. Our study does not confirm the association between a panel of selected SNP and poor prognosis in Portuguese patients with JIA. PMID:26504858

  7. Genetic Screening of Mutations Associated with Fabry Disease in a Nationwide Cohort of Juvenile Idiopathic Arthritis Patients.

    PubMed

    Gonçalves, Maria J; Mourão, Ana F; Martinho, António; Simões, Olívia; Melo-Gomes, José; Salgado, Manuel; Estanqueiro, Paula; Ribeiro, Célia; Brito, Iva; Fonseca, João E; Canhão, Helena

    2017-01-01

    Fabry's disease (FD) is a lysosomal storage disorder associated with an alpha-galactosidase A deficiency. The prevalence of FD among juvenile idiopathic arthritis (JIA) patients with established diagnosis is unknown, but as musculoskeletal pain may be an important complaint at presentation, misdiagnosed cases are anticipated. With this study, we aim to calculate the frequency of FD-associated mutations in a cohort of JIA patients. Children with JIA from a national cohort were selected. Clinical and laboratorial information was recorded in the Portuguese rheumatic diseases register (http://Reuma.pt). Molecular genetic testing to detect GLA gene mutations was performed. After the multiplex polymerase chain reactions technique for DNA amplification, direct sequencing of the complete sequence of GLA gene was completed. From a cohort of 292 patients with JIA (188 females, 104 males), mutations were identified in 5 patients (all female). Four patients had the mutation D313Y, a rare GLA variant, which is associated with low enzymatic levels in plasma, but normal lysosomal levels. One patient presented the missense mutation R118C, which was previously described in Mediterranean patients with FD. This is the first screening of FD mutations in a cohort of JIA patients. No "classic" pathogenic FD mutations were reported. The late-onset FD-associated mutation, R118C, was found in a frequency of 0.34% (1/292).

  8. Blood gene expression profiling in pediatric systemic lupus erythematosus and systemic juvenile idiopathic arthritis: from bench to bedside

    PubMed Central

    2014-01-01

    Blood gene expression profiling has led to major advances in the field of rheumatology over the last few decades. Specifically, DNA microarray technology has been integral in increasing our knowledge of key players in the pathogenesis of some rare pediatric rheumatic diseases. Our group, using microarray analysis, identified the interferon (IFN) gene signature in pediatric systemic lupus erythematosus (SLE) and has published data that suggest high doses of intravenous corticosteroid treatment may have benefit over strictly oral regimens. Additionally, DNA microarray technology led to our discovery that the interleukin (IL)-1 gene signature is associated with systemic juvenile idiopathic arthritis (sJIA) and to the use of IL-1 blockade with anakinra in this disease. We also reported the biologic rationale for use of anakinra early in the disease course. Anakinra is now being used as first-line treatment in sJIA in multiple centers. Herein, we review how information obtained from blood gene expression profiling has changed our clinical practice. PMID:24839407

  9. 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 ...

  10. Transfer from paediatric rheumatology to the adult rheumatology setting: experiences and expectations of young adults with juvenile idiopathic arthritis.

    PubMed

    Hilderson, Deborah; Eyckmans, Leen; Van der Elst, Kristien; Westhovens, Rene; Wouters, Carine; Moons, Philip

    2013-05-01

    Adolescents with juvenile idiopathic arthritis (JIA) are transferred from paediatrics to adult-oriented healthcare when they reach early adulthood. Research on the extent to which patients' expectations about the adult healthcare setting match their actual experience after transfer, may promote successful transfer from paediatrics to adult care. As part of the 'Don't Retard' project ( http://www.kuleuven.be/switch2/rheuma.html ), experiences and expectations of young adults regarding their transfer from paediatric rheumatology to adult rheumatology were explored. A qualitative study was conducted using semi-structured, in-depth interviews of 11 patients with JIA, aged 18 to 30. Data were analysed using procedures inherent to the content analysis approach. For both concepts, experiences and expectations, three main themes emerged: 'preparation', 'parental involvement' and an 'adapted setting for the late-adolescent or early adult'. The need for a gradual process covered the themes 'preparation' and 'parental involvement'. Young people with JIA prefer to have a say in the moment of transfer and in the reduction of parental involvement. The majority of the participants like their parents' presence at the first consultation at the adult rheumatology department. They expect a healthcare setting adapted to their needs and the possibility to meet peers in this setting. Sudden confrontation with older patients with severe rheumatoid arthritis at adult rheumatology was an unsettling experience for some of the young patients and they declared that better preparation is needed. This study enabled us to define three main themes important in transfer. These themes can facilitate healthcare professionals in developing specific interventions to prepare the young people to transfer, to regulate parental involvement and to arrange an adapted setting for them. Since we included patients who were in follow-up at one tertiary care centre, in which both paediatric and adult

  11. TNF-alpha promoter gene polymorphisms in Spanish children with persistent oligoarticular and systemic-onset juvenile idiopathic arthritis.

    PubMed

    Modesto, C; Patiño-García, A; Sotillo-Piñeiro, E; Merino, J; García-Consuegra, J; Merino, R; Rua, M J; Sierrasesúmaga, L; Arnal, C

    2005-01-01

    To explore the possible association/s of the first reported tumour necrosis factor (TNF-alphaTNF-) alpha promoter gene polymorphisms -308, -238, -376 and -163 (G-->A) with systemic (SoJIA) and oligoarticular subtypes of juvenile idiopathic arthritis (JIA); and to test the association between these polymorphisms and the class I/class II HLA alleles in our population. The patient group comprised 29 oligoarticular and 26 systemic Caucasian Spanish children with JIA; 68 healthy volunteers from the same ethnic group and geographical region served as controls. HLA alleles were determined using low-resolution polymerase chain reaction (PCR). TNF-alpha promoter gene polymorphisms were screened using PCR denaturing gradient gel electrophoresis (PCR-DGGE), followed, if positive, by restriction fragment length polymorphism (RFLP) analysis for identification. No statistical association was found between the four polymorphisms studied and JIA. However, the -308 G-->A polymorphism (TNF A2) tended to be more frequent in patients with SoJIA than in the oligoarticular group. TNF A2 was strongly associated with the extended haplotype A1B8DR3 (p = 0.003), and the tandem polymorphism -238/-376 in the presence of B18 and DR3. The TNF A2 allele was more frequent in SoJIA than in the oligoarticular group. TNF A2 can help to create a more inflammatory milieu in this JIA subtype, in combination with other polymorphisms involved in regulatory sequences of key molecules in the inflammatory response. The association of the -308 and -238/-376 polymorphisms with specific alleles of the HLA is reconfirmed.

  12. Alteration of Fecal Microbiota Profiles in Juvenile Idiopathic Arthritis. Associations with HLA-B27 Allele and Disease Status

    PubMed Central

    Di Paola, Monica; Cavalieri, Duccio; Albanese, Davide; Sordo, Maddalena; Pindo, Massimo; Donati, Claudio; Pagnini, Ilaria; Giani, Teresa; Simonini, Gabriele; Paladini, Alessia; Lionetti, Paolo; De Filippo, Carlotta; Cimaz, Rolando

    2016-01-01

    Alteration of gut microbiota is involved in several chronic inflammatory and autoimmune diseases, including rheumatoid arthritis, and gut microbial “pro-arthritogenic” profiles have been hypothesized. Intestinal inflammation may be involved in spondyloarthropathies and in a subset of patients affected by Juvenile Idiopathic Arthritis (JIA), the most common chronic rheumatic disease of childhood. We compared the fecal microbiota composition of JIA patients with healthy subjects (HS), evaluating differences in microbial profiles between sub-categories of JIA, such as enthesitis-related arthritis (JIA-ERA), in which inflammation of entheses occurs, and polyarticular JIA, non-enthesitis related arthritis (JIA-nERA). Through taxon-level analysis, we discovered alteration of fecal microbiota components that could be involved in subclinical gut inflammation, and promotion of joint inflammation. We observed abundance in Ruminococcaceae in both JIA categories, reduction in Clostridiaceae and Peptostreptococcaceae in JIA-ERA, and increase in Veillonellaceae in JIA-nERA, respectively, compared with HS. Among the more relevant genera, we found an increase in Clostridium cluster XIVb, involved in colitis and arthritis, in JIA-ERA patients compared with HS, and a trend of decrease in Faecalibacterium, known for anti-inflammatory properties, in JIA-nERA compared with JIA-ERA and HS. Differential abundant taxa identified JIA patients for the HLA-B27 allele, including Bilophila, Clostridium cluster XIVb, Oscillibacter, and Parvimonas. Prediction analysis of metabolic functions showed that JIA-ERA metagenome was differentially enriched in bacterial functions related to cell motility and chemotaxis, suggesting selection of potential virulence traits. We also discovered differential microbial profiles and intra-group variability among active disease and remission, suggesting instability of microbial ecosystem in autoimmune diseases with respect to healthy status. Similarly to

  13. Body composition in children with juvenile idiopathic arthritis: effect of dietary intake of macronutrient: results from a cross sectional study

    PubMed Central

    Hari, Asmae; Rostom, Samira; Hassani, Asmae; El Badri, Dalal; Bouaadi, Ilham; Barakat, Amina; Chkirat, Bouchra; Elkari, Khalid; Amine, Bouchra; Hajjaj-Hassouni, Najia

    2015-01-01

    Introduction The aim of this study was to evaluate the relationship between macronutrient intake, body composition (lean body mass and fat mass) and bone mineral content in Moroccan children with juvenile idiopathic arthritis (JIA). Methods A cross-sectional study, conducted between May 2010 and June 2011, covering out patient with JIA. The characteristics of patients were collected. The nutritional status was assessed by a food questionnaire including data of food intake during 7 consecutive days using 24-hour dietary recall. Food intake was quantified using the software Bilnut (Bilnut version 2.01, 1991). Dietary intake of macronutrients was expressed as percentage contribution to total energy. Body composition was evaluated with DXA total-body measurements (bone mineral content BMC expressed in g, lean body mass LBM and fat mass FM expressed in kg). Results 33 patients were included. The mean age was 10.4 ± 4.3 years. The median disease duration was 2 (1-4.5) years. The median of LBM, FM and BMC were 19 kg (13.82-33.14), 5 kg (3.38-9.14) and 1044.90 g (630.40-1808.90) respectively. We found a positive correlation between LBM and dietary intake of carbohydrate (r= 0.4; p = 0.03). There were no significant association between LBM and intake of lipids, or protein. Moreover, no association was found between FM, BMC and intake of carbohydrates, lipids and proteins. Conclusion This study suggests that there is a positive correlation between carbohydrates intake and LBM; however, dietary intake does not influence FM and BMC. Prospective studies with larger numbers of patients appear to be needed to confirm our findings. PMID:26161167

  14. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

    PubMed Central

    Ravelli, Angelo; Minoia, Francesca; Davì, Sergio; Horne, AnnaCarin; Bovis, Francesca; Pistorio, Angela; Aricò, Maurizio; Avcin, Tadej; Behrens, Edward M; De Benedetti, Fabrizio; Filipovic, Alexandra; Grom, Alexei A; Henter, Jan-Inge; Ilowite, Norman T; Jordan, Michael B; Khubchandani, Raju; Kitoh, Toshiyuki; Lehmberg, Kai; Lovell, Daniel J; Miettunen, Paivi; Nichols, Kim E; Ozen, Seza; Pachlopnik Schmid, Jana; Ramanan, Athimalaipet V; Russo, Ricardo; Schneider, Rayfel; Sterba, Gary; Uziel, Yosef; Wallace, Carol; Wouters, Carine; Wulffraat, Nico; Demirkaya, Erkan; Brunner, Hermine I; Martini, Alberto; Ruperto, Nicolino; Cron, Randy Q

    2016-01-01

    Objective To identify which laboratory tests that change over time are most valuable for the timely diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA). Methods A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of experts was first asked to evaluate 115 profiles of patients with MAS, which included the values of laboratory tests at the pre-MAS visit and at MAS onset, and the change in values between the two time points. The experts were asked to choose the 5 laboratory tests in which change was most important for the diagnosis of MAS and to rank the 5 selected tests in order of importance. The relevance of change in laboratory parameters was further discussed and ranked by the same experts at a consensus conference. Results Platelet count was the most frequently selected test, followed by ferritin level, aspartate aminotransferase (AST), white cell count, neutrophil count, and fibrinogen and erythrocyte sedimentation rate. Ferritin was most frequently assigned the highest score. At the end of the process, platelet count, ferritin level and AST were the laboratory tests in which the experts found change over time to be most important. Conclusions We identified the laboratory tests in which change over time is most valuable for the early diagnosis of MAS in sJIA. The dynamics of laboratory values during the course of MAS should be further scrutinised in a prospective study in order to establish the optimal cut-off values for their variation. PMID:26848401

  15. Operative stabilization of the remaining mobile segment in ankylosed cervical spine in systemic onset - juvenile idiopathic arthritis: A case report

    PubMed Central

    Suhodolčan, Lovro; Mihelak, Marko; Brecelj, Janez; Vengust, Rok

    2016-01-01

    We describe a case of a 19-year-old young man with oligoarthritis type of juvenile idiopathic arthritis, who presented with several month duration of lower neck pain and progressive muscular weakness of all four limbs. X-rays of the cervical spine demonstrated spontaneous apophyseal joint fusion from the occipital condyle to C6 and from C7 to Th2 with marked instability between C6 and C7. Surgical intervention began with anterolateral approach to the cervical spine performing decompression, insertion of cage and anterior vertebral plate and screws, followed by posterior approach and fixation. Care was taken to restore sagittal balance. The condition was successfully operatively managed with multisegmental, both column fixation and fusion, resulting in pain cessation and resolution of myelopathy. Postoperatively, minor swallowing difficulties were noted, which ceased after three days. Patient was able to move around in a wheelchair on the sixth postoperative day. Stiff neck collar was advised for three months postoperatively with neck pain slowly decreasing in the course of first postoperative month. On the follow-up visit six months after the surgery patient exhibited no signs of spastic tetraparesis, X-rays of the cervical spine revealed solid bony fusion at single mobile segment C6-C7. He was able to gaze horizontally while sitting in a wheelchair. Signs of myelopathy with stiff neck and single movable segment raised concerns about intubation, but were successfully managed using awake fiber-optic intubation. Avoidance of tracheostomy enabled us to perform an anterolateral approach without increasing the risk of wound infection. Regarding surgical procedure, the same principles are obeyed as in management of fracture in ankylosing spondylitis or Mb. Forestrier. PMID:27458558

  16. HLA genotyping as first-line screening tool for coeliac disease in children with juvenile idiopathic arthritis.

    PubMed

    Skrabl-Baumgartner, Andrea; Christine Hauer, Almuthe; Erwa, Wolfgang; Jahnel, Jörg

    2017-07-01

    Coeliac disease (CD) and juvenile idiopathic arthritis (JIA) often coexist. This association warrants assessment for CD in patients with JIA. We evaluated the clinical relevance and cost-effectiveness of human leucocyte antigen (HLA) genotyping in first-line screening for development of CD in children with JIA. 95 patients with JIA were screened for CD using CD-specific antibodies. In case of positivity, a small intestinal biopsy was performed to confirm diagnosis. In addition, HLA genotyping was performed. 110 age-matched and sex-matched Caucasian children from the same geographical area served as controls. CD was diagnosed in 4 of 95 patients with JIA (4.2%), a rate significantly higher compared with controls (p<0.02) and 14 times higher than in the general population. Twenty-six patients (27.4%) had one of the variants of the risk genotypes. All four patients diagnosed with CD had a HLA-DQ2.5 genotype: one was homozygote, the remainder heterozygote. Twenty-two patients are, judging by their HLA genotypes, at risk of developing CD and require repeated serological screening. None of the 69 patients without HLA-DQ2/DQ8 genotypes had CD-specific antibodies. Screening with HLA genotyping becomes cheaper than screening without after the second determination. In our cohort of patients with JIA, lack of HLA-DQ2/DQ8 genotypes identified a majority not at risk of CD in whom repeated serological testing is unnecessary. Genotyping is nowadays the most efficient and cost-effective way to screen for CD risk in JIA. 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/.

  17. Safety of celecoxib and nonselective nonsteroidal anti-inflammatory drugs in juvenile idiopathic arthritis: results of the phase 4 registry

    PubMed Central

    2014-01-01

    Background This study aimed to assess long-term safety and developmental data on juvenile idiopathic arthritis (JIA) patients treated in routine clinical practice with celecoxib or nonselective nonsteroidal anti-inflammatory drugs (nsNSAIDs). Methods Children aged ≥2 to <18 years with rheumatoid-factor–positive or –negative polyarthritis, persistent or extended oligoarthritis, or systemic arthritis were enrolled into this prospective, observational, multicenter standard-of-care registry. Eligible patients were newly or recently prescribed (≤6 months) an nsNSAID or celecoxib. Enrolled patients were followed to the end of the study, whether they remained on the original NSAID, switched, or discontinued therapy altogether. All adverse events (AEs) regardless of severity were captured in the database. Results A total of 274 patients (nsNSAID, n = 219; celecoxib, n = 55) were observed for 410 patient-years of observation. Naproxen, meloxicam, and nabumetone were the most frequently used nsNSAIDs. At baseline, the celecoxib group was older, had a numerically longer median time since diagnosis, and a numerically higher proportion of patients with a history of gastrointestinal-related NSAID intolerance. AEs reported were those frequently observed with NSAID treatment and were similar across groups (nsNSAIDs: 52.0%; celecoxib: 52.9%). Twelve unique patients experienced a total of 18 serious AEs; the most frequent were infections, and none was attributed to NSAID use. Conclusions The safety profile of celecoxib and nsNSAIDs appears similar overall. The results from this registry, ongoing pharmacovigilance, and the phase 3 trial that led to the approval of celecoxib for children with JIA provide evidence that the benefit-risk for celecoxib treatment in JIA remains positive. Trial registration ClinicalTrials.gov identifier NCT00688545. PMID:25057265

  18. Th1-Induced CD106 Expression Mediates Leukocytes Adhesion on Synovial Fibroblasts from Juvenile Idiopathic Arthritis Patients

    PubMed Central

    Luciani, Cristina; Capone, Manuela; Rossi, Maria Caterina; Chillà, Anastasia; Santarlasci, Veronica; Mazzoni, Alessio; Cimaz, Rolando; Liotta, Francesco; Maggi, Enrico; Cosmi, Lorenzo; Del Rosso, Mario; Annunziato, Francesco

    2016-01-01

    This study tested the hypothesis that subsets of human T helper cells can orchestrate leukocyte adhesion to synovial fibroblasts (SFbs), thus regulating the retention of leukocytes in the joints of juvenile idiopathic arthritis (JIA) patients. Several cell types, such as monocytes/macrophages, granulocytes, T and B lymphocytes, SFbs and osteoclasts participate in joint tissue damage JIA. Among T cells, an enrichment of classic and non-classic Th1 subsets, has been found in JIA synovial fluid (SF), compared to peripheral blood (PB). Moreover, it has been shown that IL-12 in the SF of inflamed joints mediates the shift of Th17 lymphocytes towards the non-classic Th1 subset. Culture supernatants of Th17, classic and non-classic Th1 clones, have been tested for their ability to stimulate proliferation, and to induce expression of adhesion molecules on SFbs, obtained from healthy donors. Culture supernatants of both classic and non-classic Th1, but not of Th17, clones, were able to induce CD106 (VCAM-1) up-regulation on SFbs. This effect, mediated by tumor necrosis factor (TNF)-α, was crucial for the adhesion of circulating leukocytes on SFbs. Finally, we found that SFbs derived from SF of JIA patients expressed higher levels of CD106 than those from healthy donors, resembling the phenotype of SFbs activated in vitro with Th1-clones supernatants. On the basis of these findings, we conclude that classic and non-classic Th1 cells induce CD106 expression on SFbs through TNF-α, an effect that could play a role in leukocytes retention in inflamed joints. PMID:27123929

  19. The distribution of juvenile idiopathic arthritis in the eastern Mediterranean: results from the registry of the Turkish Paediatric Rheumatology Association.

    PubMed

    Demirkaya, Erkan; Ozen, Seza; Bilginer, Yelda; Ayaz, Nuray Aktay; Makay, Balahan Bora; Unsal, Erbil; Erguven, Muferet; Poyrazoglu, Hakan; Kasapcopur, Ozgur; Gok, Faysal; Akman, Sema; Balat, Ayse; Cavkaytar, Ozlem; Kaya, Bulent; Duzova, Ali; Ozaltin, Fatih; Topaloglu, Rezan; Besbas, Nesrin; Bakkaloglu, Aysin; Arisoy, Nil; Ozdogan, Huri; Bakkaloglu, Sevcan; Turker, Turker

    2011-01-01

    To analyse the demographics, main clinical and laboratory features and subtype distribution of juvenile idiopathic arthritis (JIA) in an eastern Mediterranean country, based on a multicentre registry. Between March 2008 and February 2009 with this cross-sectional study, consecutive patients seen with JIA in selected centres were registered through a web-based registry. All patients were classified according to the International League of Associations for Rheumatology (ILAR) criteria. There were 634 patients with a mean age of 11.84 ± 4.66 years and the female/male ratio was 1.2. The distributions of JIA patients according to onset of disease were as follows: systemic 92 (14.5%), oligoarticular extended 26 (4.1%), oligoarticular persistent 234 (36.9%), rheumatoid factor (RF) positive polyarthritis 20 (3.2%), RF negative polyarthritis 129 (20.3%), enthesitis-related 120 (18.9%), psoriatic 13(2.1%). The frequency of uveitis was 15.7% among all of the oligoarthritis patients. Anti-nuclear antibody (ANA) was positive mainly among the oligoarticular onset patients. Twenty-one patients also had Familial Mediterranean fever (FMF). Among systemic JIA patients, the frequency of macrophage activation syndrome (MAS) was 15.2% (n=14). At the end of the mean follow-up of 7.6 ± 4.4 years, 305 (48.1%) patients were defined to have inactive disease on medication, and 106 (16.7%) were completely free of any disease symptoms without medication. Enthesitis related arthritis had a high frequency whereas psoriatic arthritis was very rare compared to other series. We suggest that there are certain differences in the characteristics of JIA in our eastern Mediterranean population. Thus, genetic studies need to be assessed in these populations separately and findings of genome wide association studies need to be confirmed in different populations.

  20. 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

  1. High prevalence of methotrexate intolerance in juvenile idiopathic arthritis: development and validation of a methotrexate intolerance severity score.

    PubMed

    Bulatović, Maja; Heijstek, Marloes W; Verkaaik, Marleen; van Dijkhuizen, E H Pieter; Armbrust, Wineke; Hoppenreijs, Esther P A; Kamphuis, Sylvia; Kuis, Wietse; Egberts, Toine C G; Sinnema, Gerben; Rademaker, Carin M A; Wulffraat, Nico M

    2011-07-01

    To design and validate a new questionnaire for identifying patients with methotrexate (MTX) intolerance, and to determine the prevalence of MTX intolerance in patients with juvenile idiopathic arthritis (JIA) using this questionnaire. The MTX Intolerance Severity Score (MISS) questionnaire was constructed, consisting of 5 domains: stomach ache, nausea, vomiting, sore mouth, and behavioral symptoms. The domains each consisted of 3 questions pertaining to the presence of a symptom upon, prior to (anticipatory), and when thinking of (associative) MTX intake. The MISS questionnaire was validated in 86 patients by determining its discriminative power between patients with and those without MTX intolerance, identified as such by a gold standard (physician's opinion). Using the MISS questionnaire, the prevalence of MTX intolerance was determined in 297 JIA patients. The MISS questionnaire discriminated well between MTX-intolerant and MTX-tolerant patients. A cutoff score of 6 yielded the best sensitivity (88%) and specificity (80%). MTX intolerance was found in 150 (50.5%) of 297 patients. Of 220 patients receiving oral MTX, 98 (44.5%) experienced MTX intolerance, whereas 67.5% of 77 patients receiving parenteral MTX experienced intolerance to the drug (P = 0.001). Our findings indicate that the MISS questionnaire is a highly sensitive and specific tool for the diagnosis of MTX intolerance, and that there is a high prevalence of MTX intolerance among JIA patients. The prevalence of intolerance in patients receiving parenteral MTX exceeds that in patients receiving oral MTX. The frequent occurrence of anticipatory and associative symptoms suggests that classic conditioning plays an important role in MTX intolerance. Copyright © 2011 by the American College of Rheumatology.

  2. 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

  3. 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

  4. Altered expression of intestinal human leucocyte antigen D-related and immune signalling molecules in juvenile idiopathic arthritis

    PubMed Central

    Arvonen, M; Vähäsalo, P; Turunen, S; Salo, H M; Mäki, M; Laurila, K; Vaarala, O; Karttunen, T J

    2012-01-01

    We aimed to study intestinal immune activation status in juvenile idiopathic arthritis (JIA) by assessing intestinal human leucocyte antigen (HLA) class II expression and the mRNA expression levels of the pro- and anti-inflammatory mediators and pattern recognition receptors. HLA-D-related (HLA-DR) expression was assessed using immunohistochemical staining of frozen sections in 11 children with JIA and 17 controls. The gene expression levels of the anti- and proinflammatory cytokines, lymphocyte recognition receptors and pattern recognition receptors were studied with reverse transcription–polymerase chain reaction (RT–PCR) in 14 children with JIA and 12 controls. All subjects had various gastrointestinal (GI) symptoms indicating endoscopic examinations, but eventually were not diagnosed with GI disease. In JIA patients, the expression of HLA-DR was increased in the crypt epithelial cells and in the epithelial basement membrane of the ileum when compared with the controls. Positive HLA-DR staining in the ileal mucosa was associated with the presence of high clinical disease activity of JIA and low mRNA expression of anti-inflammatory mediators, such as forkhead box protein P3 (FoxP3), glucocorticoid-induced tumour necrosis factor receptor-related protein (GITR) and transforming growth factor (TGF)-beta. Low ileal expression of interleukin (IL)-10, TGF-β, FoxP3, Toll-like receptor 2 (TLR-2) and TLR-4 transcripts correlated significantly with a high clinical disease activity in the JIA patients. The increased HLA-DR expression suggests enhanced intestinal antigen presentation in JIA. A correlation between clinical disease activity and low gene expression of tolerogenic mediators in the ileum supports the hypothesis that a link exists between the gut immune system and JIA. PMID:23121667

  5. Methotrexate efficacy and tolerability after switching from oral to subcutaneous route of administration in juvenile idiopathic arthritis.

    PubMed

    Żuber, Zbigniew; Turowska-Heydel, Dorota; Sobczyk, Małgorzata; Banach-Górnicka, Marta; Rusnak, Katarzyna; Piszczek, Anna; Mężyk, Elżbieta

    2016-01-01

    Methotrexate (MTX) is one of the most frequently used, highly effective disease-modifying drugs in juvenile idiopathic arthritis (JIA) therapy. The drug can be administered orally or subcutaneously, but the efficacy and tolerance of these two routes of administration raise doubts in JIA patients. The aim of the study was to evaluate MTX efficacy and tolerability after switching from the oral to the subcutaneous route of administration in children with JIA. A single-centre, questionnaire-based assessment of MTX efficacy and tolerance in 126 unselected JIA patients with longer than 6 months of follow-up was performed. In all patients, MTX was initially administered orally. The response to MTX treatment was analysed according to American College of Rheumatology (ACR) paediatric criteria. Six-month MTX therapy was effective (ACR score ≥ 30) in 83 children (65.9%). The oral route of MTX administration was changed to subcutaneous in 32 patients after a mean period of 14 months due to intolerance (n = 20) or reluctance to take the oral formulation (n = 12). This group of children was significantly younger (p = 0.02) but did not differ from the group of children that continued oral treatment in other aspects, including MTX dose. Six months after switching from oral to subcutaneous MTX the ACR score remained unchanged. Three children (9.4%) still reported symptoms of drug intolerance. The switch from oral to subcutaneous MTX may increase the response rate in JIA patients with intolerance of its oral formulation. The reluctance to take oral MTX can be anticipated in early childhood, and should be considered in the individualization of therapy, having also in mind the lower risk of severe gastrointestinal adverse drug reactions.

  6. Mesenchymal stromal cells isolated from children with systemic juvenile idiopathic arthritis suppress innate and adaptive immune responses.

    PubMed

    Calkoen, Friso G J; Brinkman, Danielle M C; Vervat, Carly; van Ostaijen-Ten Dam, Monique M; Ten Cate, Rebecca; van Tol, Maarten J D; Ball, Lynne M

    2013-03-01

    Infusion of mesenchymal stromal cells (MSCs) has been reported to be an effective treatment modality for acute graft-versus-host disease, and MSCs have been considered for use in the treatment of patients with autoimmune diseases. Before contemplating clinical studies with MSCs in patients with systemic juvenile idiopathic arthritis (sJIA), the immunomodulatory capacity of MSCs in this setting needs to be explored. A comparative analysis of bone marrow-derived MSCs from children with sJIA and healthy pediatric controls was performed. MSCs were successfully expanded from 11 patients with sJIA and 10 controls. The phenotype, differentiation and immunomodulatory capacity of these MSCs were compared. The effect of immunosuppressive drugs on MSC function was also investigated. MSCs from patients with sJIA and controls showed no differences in their suppressive effect using control peripheral blood mononuclear cells. Furthermore, the suppression of the response of peripheral blood mononuclear cells from patients with sJIA by autologous sJIA MSCs and allogeneic control MSCs was comparable. The immunosuppressive effect of both groups of MSCs was diminished in the presence of indomethacin (P < 0.05). MSCs from patients with sJIA and controls suppressed interleukin-2-induced natural killer cell activation to a similar extent. In addition, MSCs of patients with sJIA and controls inhibited the differentiation of monocytes to dendritic cells. This is the first explorative study in a significant cohort of patients with sJIA to evaluate the effect of MSCs on adaptive and innate immune responses. The comparable immunosuppressive characteristics of MSCs derived from patients with sJIA to age-matched controls support the potential use of patient-derived MSCs in the treatment of sJIA. Copyright © 2013 International Society for Cellular Therapy. Published by Elsevier Inc. All rights reserved.

  7. Measurement of biomarkers in juvenile idiopathic arthritis patients and their significant association with disease severity: a comparative study.

    PubMed

    Gilliam, B E; Chauhan, A K; Low, J M; Moore, T L

    2008-01-01

    To evaluate in juvenile idiopathic arthritis (JIA) patients a biomarker panel of anti-cyclic citrullinated peptide (anti-CCP) antibodies, cartilage oligomeric matrix protein (COMP), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), IgM rheumatoid factor (RF), IgG RF, and IgA RF and compare to the presence of joint erosions (JE), joint space narrowing (JSN), and synovitis in order to evaluate aggressive disease. Sixty-eight JIA patients (19 RF positive polyarthritis, 23 RF negative polyarthritis, 17 persistent oligoarthritis, and 9 systemic-onset) were evaluated using the biomarker panel and compared to 18 healthy controls. All RF isotypes, anti-CCP antibodies, and COMP were measured by enzyme-linked immunosorbent assays (ELISA). Statistically significant differences and associations were assessed for each biomarker in relation to JE, JSN, and synovitis. Multiple regression analysis was used to find the variables associated with joint damage and synovitis. Patients with JE and JSN had significantly elevated levels of IgA RF, IgM RF, and anti-CCP antibodies. COMP levels were higher in early disease, but also later in disease in patients with no JE or JSN. ESR, CRP, and IgA RF were significantly elevated in patients with active synovitis. Regression analysis showed IgM RF and disease duration to be associated with JE and JSN. Anti-CCP antibodies and COMP were also associated with JSN. CRP and IgA RF were associated with synovitis. Our findings demonstrate the importance of measuring IgM RF and IgA RF by ELISA and anti-CCP antibodies by ELISA, in addition to COMP in the assessment of JIA patients to determine severity of disease.

  8. Incidence and predictors of Uveitis in juvenile idiopathic arthritis in a Nordic long-term cohort study.

    PubMed

    Nordal, Ellen; Rypdal, Veronika; Christoffersen, Terje; Aalto, Kristiina; Berntson, Lillemor; Fasth, Anders; Herlin, Troels; Nielsen, Susan; Peltoniemi, Suvi; Straume, Bjørn; Zak, Marek; Rygg, Marite

    2017-08-18

    The incidence of uveitis associated with juvenile idiopathic arthritis (JIA) varies around the world. Our aim was to investigate the incidence and predictors of uveitis in a Nordic population-based cohort. Consecutive JIA cases from defined geographical areas in Denmark, Finland, Sweden and Norway with disease onset between January 1997 to June 2000 were followed for median 98 months in this prospective longitudinal cohort study. Potential clinical and immunological predictors of uveitis were identified with logistic regression analysis. Uveitis occurred in 89 (20.5%) of the 435 children with regular ophtalmologic follow-up among the 500 included. Chronic asymptomatic uveitis developed in 80 and acute symptomatic uveitis in 9 children. Uveitis developed at a median interval of 0.8 (range - 4.7 to 9.4) years after onset of arthritis. Predictors of uveitis were age < 7 years at JIA onset (Odds ratio (OR) 2.1, 95% confidence interval (CI) 1.3 to 3.5), presence of antihistone antibodies (AHA) > 15 U/ml (OR 4.8 (1.8 to 13.4)) and antinuclear antibodies (ANA) (OR 2.4 (1.5 to 4.0)). Mean combined IgM/IgG AHA was significantly higher in the uveitis group (19.2 U/ml) than in the non-uveitis group (10.2 U/ml) (p = 0.002). Young age at JIA onset predicted uveitis in girls (p < 0.001), but not in boys (p = 0.390). Early-onset arthritis and presence of AHA in girls, as well as presence of ANA in both genders, were significant predictors of chronic uveitis. The high incidence of uveitis in this long-term Nordic JIA cohort may have severe implications in a lifelong perspective.

  9. Early onset pauciarticular arthritis is the major risk factor for naproxen-induced pseudoporphyria in juvenile idiopathic arthritis

    PubMed Central

    Schäd, Susanne G; Kraus, Andrea; Haubitz, Imme; Trcka, Jiri; Hamm, Henning; Girschick, Hermann J

    2007-01-01

    Pseudoporphyria (PP) is characterized by skin fragility, blistering and scarring in sun-exposed skin areas without abnormalities in porphyrin metabolism. The phenylpropionic acid derivative group of nonsteroidal anti-inflammatory drugs, especially naproxen, is known to cause PP. Naproxen is currently one of the most prescribed drugs in the therapy of juvenile idiopathic arthritis (JIA). The prevalence of PP was determined in a 9-year retrospective study of children with JIA and associated diseases. In addition, we prospectively studied the incidence of PP in 196 patients (127 girls and 69 boys) with JIA and associated diseases treated with naproxen from July 2001 to March 2002. We compared these data with those from a matched control group with JIA and associated diseases not treated with naproxen in order to identify risk factors for development of PP. The incidence of PP in the group of children taking naproxen was 11.4%. PP was particularly frequent in children with the early-onset pauciarticular subtype of JIA (mean age 4.5 years). PP was associated with signs of disease activity, such as reduced haemoglobin (<11.75 g/dl), and increased leucocyte counts (>10,400/μl) and erythocyte sedimentation rate (>26 mm/hour). Comedications, especially chloroquine intake, appeared to be additional risk factors. The mean duration of naproxen therapy before the onset of PP was 18.1 months, and most children with PP developed their lesions within the first 2 years of naproxen treatment. JIA disease activity seems to be a confounding factor for PP. In particular, patients with early-onset pauciarticular JIA patients who have significant inflammation appear to be prone to developing PP upon treatment with naproxen. PMID:17266758

  10. Alteration of Fecal Microbiota Profiles in Juvenile Idiopathic Arthritis. Associations with HLA-B27 Allele and Disease Status.

    PubMed

    Di Paola, Monica; Cavalieri, Duccio; Albanese, Davide; Sordo, Maddalena; Pindo, Massimo; Donati, Claudio; Pagnini, Ilaria; Giani, Teresa; Simonini, Gabriele; Paladini, Alessia; Lionetti, Paolo; De Filippo, Carlotta; Cimaz, Rolando

    2016-01-01

    Alteration of gut microbiota is involved in several chronic inflammatory and autoimmune diseases, including rheumatoid arthritis, and gut microbial "pro-arthritogenic" profiles have been hypothesized. Intestinal inflammation may be involved in spondyloarthropathies and in a subset of patients affected by Juvenile Idiopathic Arthritis (JIA), the most common chronic rheumatic disease of childhood. We compared the fecal microbiota composition of JIA patients with healthy subjects (HS), evaluating differences in microbial profiles between sub-categories of JIA, such as enthesitis-related arthritis (JIA-ERA), in which inflammation of entheses occurs, and polyarticular JIA, non-enthesitis related arthritis (JIA-nERA). Through taxon-level analysis, we discovered alteration of fecal microbiota components that could be involved in subclinical gut inflammation, and promotion of joint inflammation. We observed abundance in Ruminococcaceae in both JIA categories, reduction in Clostridiaceae and Peptostreptococcaceae in JIA-ERA, and increase in Veillonellaceae in JIA-nERA, respectively, compared with HS. Among the more relevant genera, we found an increase in Clostridium cluster XIVb, involved in colitis and arthritis, in JIA-ERA patients compared with HS, and a trend of decrease in Faecalibacterium, known for anti-inflammatory properties, in JIA-nERA compared with JIA-ERA and HS. Differential abundant taxa identified JIA patients for the HLA-B27 allele, including Bilophila, Clostridium cluster XIVb, Oscillibacter, and Parvimonas. Prediction analysis of metabolic functions showed that JIA-ERA metagenome was differentially enriched in bacterial functions related to cell motility and chemotaxis, suggesting selection of potential virulence traits. We also discovered differential microbial profiles and intra-group variability among active disease and remission, suggesting instability of microbial ecosystem in autoimmune diseases with respect to healthy status. Similarly to other

  11. Anti-type II collagen antibodies detection and avidity in patients with oligoarticular and polyarticular forms of juvenile idiopathic arthritis.

    PubMed

    Araujo, Galber R; Fonseca, João E; Fujimura, Patricia T; Cunha-Junior, Jair P; Silva, Carlos H M; Mourão, Ana F; Canhão, Helena; Goulart, Luiz R; Gonçalves, João; Ueira-Vieira, Carlos

    2015-05-01

    Juvenile idiopathic arthritis (JIA) refers to a heterogeneous group of illnesses that have in common the occurrence of chronic joint inflammation in children younger than 16 years of age. The diagnosis is made only on clinical assessment. The identification of antibody markers could improve the early diagnosis, optimizing the clinical management of patients. Type II collagen is one potential autoantigen that has been implicated in the process of arthritis development. The aims of our study were to investigate the occurrence of anti-type II collagen antibodies and also to determine the avidity of the antibody-antigen binding. Ninety-six patients with oligoarticular or polyarticular JIA, 13 patients with ankylosing spondylitis (AS) and 61 healthy controls (HC) were tested for anti-type II collagen antibodies by ELISA and avidity ELISA. Sensitivity and specificity were determined by the receiver operating characteristic (ROC) curve analysis. Forty-two JIA patients (44%) were positive for antibodies against type II collagen. Its detection was significantly higher in JIA patients than in AS patients (p=0.006) and HCs (p<0.0001). Furthermore, anti-type II collagen antibody detection was significantly more frequent in patients with JIA of ≤6 months duration (p=0.0007). Antibodies displaying high avidity to type II collagen were associated with disease activity (p=0.004). This study demonstrates that antibodies against type II collagen are present in the serum of patients with oligoarticular and polyarticular JIA, being its presence more prevalent in patients with early disease. It also demonstrates that JIA patients with active disease present antibodies with high avidity against type II collagen.

  12. The many shades of enhancement: timing of post-gadolinium images strongly influences the scoring of juvenile idiopathic arthritis wrist involvement on MRI.

    PubMed

    Rieter, Jasper F M M; de Horatio, Laura Tanturri; Nusman, Charlotte M; Müller, Lil-Sofie Ording; Hemke, Robert; Avenarius, Derk F M; van Rossum, Marion A J; Malattia, Clara; Maas, Mario; Rosendahl, Karen

    2016-10-01

    Potential long-term side effects of treatment for juvenile idiopathic arthritis are concerning. This has necessitated accurate tools, such as MRI, to monitor treatment response and allow for personalized therapy. To examine the extent to which timing of post-contrast MR images influences the scoring of inflammatory change in the wrist in children with juvenile idiopathic arthritis. We studied two sets of post-contrast 3-D gradient echo MRI series of the wrist in 34 children with juvenile idiopathic arthritis. These images were obtained immediately after administration of intravenous contrast material and again after approximately 10 min. The dataset was drawn from a prospective multicenter project conducted 2006-2010. We assessed five wrist locations for synovial enhancement, effusion and overall inflammation. Examinations were scored by one radiologist in two sessions - the first was based on the early post-contrast images, and the later session, for which the previous findings were masked, was based on the later post-contrast images. Fifty-two of the 170 locations (30.6%) received a higher synovial enhancement score based on the late post-contrast images as compared to the early images. Sixty of the 170 (35%) locations received a higher total inflammation score. The mean scores of synovial enhancement and total inflammation were significantly higher when based on the late post-contrast images as compared to the early post-contrast images. An MRI-based scoring system for the presence and degree of synovitis should be based on a standardized MR-protocol with a fixed interval between intravenous contrast injection and post-contrast images.

  13. 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.

  14. Brace treatment in juvenile idiopathic scoliosis: a prospective study in accordance with the SRS criteria for bracing studies - SOSORT award 2013 winner.

    PubMed

    Aulisa, Angelo G; Guzzanti, Vincenzo; Marzetti, Emanuele; Giordano, Marco; Falciglia, Francesco; Aulisa, Lorenzo

    2014-01-01

    The Juvenile idiopathic scoliosis by age of onset, severity and evolutivity is source of great doubts concerning the purpose and use of conservative treatment. The different clinical experiences leave unsolved the question that arises in applying a conservative treatment when the patients are effectively forward a long growing period, in scoliosis characterized by inevitable evolutivity. The purpose of the present prospective study was to determine the effectiveness of conservative treatment in Juvenile idiopathic scoliosis. From 1238 patients treated for idiopathic scoliosis between 1995 and 2012 fulfill the inclusion criteria 163 patients treated with PASB, Lyon brace and Milwaukee. Of these, 113 patients had a definite outcome, 27 have abandoned treatment e 23 are still in treatment. The minimum follow-up was 24 months. Radiographs were used to estimate the curve magnitude (CM) and the torsion of the apical vertebra (TA) at 5 time points: beginning (t1), 6 months after the beginning (t2), intermediate time between t1 and t4 (t3), end of weaning (t4), 2-years minimum follow-up (t5). Three outcomes were distinguished in agreement with SRS criteria: correction, stabilization and progression. The results from our study showed that of the 113 patients with a definite outcome CM mean value was 29.6 ± 7.5 SD at t1 and 16.9 ± 11.1 SD at t5. TA was 13.5 ± 5.4 SD at t1 and 8.5 ± 5.6 at t5. The variations between CM t5-t1 and TA t5-t1 were statistically significantly different. Curve correction was accomplished in 88 patients (77.8%), stabilization was obtained in 18 patients (15.9%). 7 patients (6.19%) have a progression and 4 of these were recommended for surgery. Of 26 patients who abandoned the treatment, at the time of abandonment (12.5 age) have achieved curve correction in 19 cases (70.0%), stabilization in 5 cases (19%) and progression in 3 cases (11%). Of these patients, reviewed at the end of growing, four have been operated on. Our study

  15. The iPeer2Peer Program: a pilot randomized controlled trial in adolescents with Juvenile Idiopathic Arthritis.

    PubMed

    Stinson, Jennifer; Ahola Kohut, Sara; Forgeron, Paula; Amaria, Khush; Bell, Mary; Kaufman, Miriam; Luca, Nadia; Luca, Stephanie; Harris, Lauren; Victor, Charles; Spiegel, Lynn

    2016-09-02

    Adolescents with Juvenile Idiopathic Arthritis (JIA) are at risk for physical, emotional, social and role challenges that negatively impact quality of life. Peer mentoring has been shown to improve positive health behaviours in adolescents with chronic disease while simultaneously providing social support. The objectives of this paper are to examine the feasibility and acceptability of an online peer mentoring program (iPeer2Peer Program) for adolescents with JIA. The iPeer2Peer program was examined using a waitlist pilot randomized control trial (RCT). Participants were randomly allocated to the intervention or wait-list control group via a secure, web-based randomization service. Health care providers and investigators were blinded to participant group allocation. Trained peer mentors (16-25 years; successfully managing their JIA) were matched to participants (12-18 years; diagnosed with JIA) randomized to the intervention group to provide peer support and education for effective self-management of JIA. Participant-mentor pairings connected ten times over 8 weeks using Skype video calls. Primary outcomes focused on implementation (i.e. measures of feasibility and acceptability). Secondary outcomes focused on effectiveness (i.e. measures of self-management, self-efficacy, pain, social support and quality of life). Thirty adolescents (mean age 14.3 ± 1.7 years, 97 % female) completed the RCT (intervention n = 16, control n = 14). One third (32 %) of adolescents approached agreed to participate, completed baseline measures and were randomized. Half of pairings completed ten calls within 8 weeks. Average call length was twice the required amount with call lengths of 44.72 ± 15.76 min. Participants reported satisfaction with the program and all reported that they would recommend it to their peers. Participants' mean engagement level with the program was 8.53/10 (range = 7-10). Participants who completed the iPeer2Peer Program demonstrated

  16. Health-related quality of life in children with juvenile idiopathic arthritis - child's and parent's point of view.

    PubMed

    Mańczak, Małgorzata; Rutkowska-Sak, Lidia; Raciborski, Filip

    2016-01-01

    To assess the quality of life (QoL) of children suffering from juvenile idiopathic arthritis (JIA) in Poland, to compare QoL of children with JIA and healthy children, and to compare children's and parents' assessments of QoL. The KIDSCREEN-52 questionnaire (children's and parents' version) was used to assess the quality of life. The QoL in JIA patients and healthy peers from European and Polish reference groups was compared by the t-test. The Bland-Altman method was used to evaluate child and parent assessment agreement. Eighty-nine questionnaires were obtained from children (median age: 14 years; 62% female; JIA history longer than 1 year) and 84 questionnaires from parents. The QoL of JIA patients was lower than in healthy peers from the European reference group in terms of physical well-being (p < 0.001), psychological well-being (p = 0.011), autonomy (p < 0.001) and social support and peers (p < 0.001). The QoL of JIA patients compared with the QoL of children from the Polish reference group was lower only in terms of physical well-being (p < 0.001), whereas it was higher in terms of moods and emotions (p = 0.023), parent relations and home life (p = 0.005) and financial resources (p < 0.001). In most terms the assessment performed by the parent was lower than the child's. The most significant differences were observed for physical well-being (p < 0.001), psychological well-being (p = 0.016), and self-perception (p = 0.013). The present study is the first assessment of QoL of JIA children in Poland. In our study the quality of life in JIA children was lower than in healthy peers. Discrepancies between the assessment of the child's QoL performed by the child and the parent were found. Both assessments should be taken into account in clinical practice as well as in research studies.

  17. Evaluation of anti-citrullinated type II collagen and anti-citrullinated vimentin antibodies in patients with juvenile idiopathic arthritis

    PubMed Central

    2013-01-01

    Background To determine the prevalence and significance of anti-citrullinated vimentin and anti-citrullinated type II collagen antibodies and elucidate their role in the disease process of juvenile idiopathic arthritis (JIA). Methods Sera were obtained from 95 patients with various subtypes of JIA, 19 systemic lupus erythematosus (SLE) patients, and 10 healthy children. Antibodies were measured in the sera against citrullinated and native type II collagen and vimentin (vim1-16 and vim 59-74) by enzyme-linked immunosorbent assay. Samples were compared to anti-cyclic citrullinated peptide (anti-CCP) antibody and rheumatoid factor (RF) isotypes, and our previously measured anti-citrullinated fibrinogen and α-enolase antibodies on the same patient population, in addition to erythrocyte sedimentation rate and C-reactive protein. The relationship between the anti-citrullinated antibody profile and disease activity and joint damage were also investigated. Results Twenty-three JIA patients (24%) demonstrated reactivity to anti-citrullinated type II collagen. Ten JIA patients (10.5%) demonstrated reactivity to anti-citrullinated vimentin 1–16 antibodies and 7 (7.4%) to anti-citrullinated vimentin 59–74 antibodies. One IgM RF-positive polyarticular patient was positive for all 5 of the citrullinated autoantibodies tested. Thirty-seven different subsets of patients were identified based on their anti-citrullinated autoantibody and RF isotype profile. No significant associations were noted with anti-citrullinated type II collagen and anti-citrullinated vimentin antibodies with joint damage or disease activity. Anti-citrullinated vimentin 59–74 antibodies demonstrated the highest overall specificity at 89.7%, with anti-citrullinated vimentin 1–16 and anti-citrullinated type II collagen antibodies at 86.2%. Conclusion This study demonstrates that antibodies to multiple citrullinated epitopes are present in the sera of patients with various subtypes of JIA. It also

  18. 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

  19. The comparisons between thermography and ultrasonography with physical examination for wrist joint assessment in juvenile idiopathic arthritis.

    PubMed

    Lerkvaleekul, Butsabong; Jaovisidha, Suphaneewan; Sungkarat, Witaya; Chitrapazt, Niyata; Fuangfa, Praman; Ruangchaijatuporn, Thumanoon; Vilaiyuk, Soamarat

    2017-03-01

    This study aimed to assess infrared thermography (IRT) and ultrasonography (US) for detecting wrist arthritis in juvenile idiopathic arthritis (JIA) patients. Although IRT could help us detecting joint inflammation, IRT studies in JIA patients with wrist arthritis are still limited. Currently, no validated US criteria exist for detecting arthritis, and the most useful parameters between Gray-scale ultrasound (GSUS) or Power Doppler ultrasound (PDUS) remain unclear. Therefore, this study focused on detecting wrist arthritis in varying degrees using IRT and US compared with physical examination. Of 46 JIA patients, 16 had previous wrist arthritis but currently inactive, 30 still had wrist arthritis, and the median ages (IQR) were 7.7 (4.3) and 10.2 (4.8) years respectively. Fifteen healthy participants were included, with a median age (IQR) of 9.2 (2.0) years. Using IRT, mean temperature (Tmean) and maximum temperature (Tmax) at skin surface in the region of interest (ROI) in the arthritis group were higher than in the inactive group and the healthy controls with p < 0.05. When patients with arthritis were subgroup analyzed by disease severity based on physical examination, the moderate to severe arthritis had Tmean and Tmax higher than the mild arthritis group with statistical significance. The Heat Distribution Index (HDI), two standard deviations of all pixel temperature values in the ROI, in the moderate to severe arthritis group was higher than in the healthy controls (p = 0.027). The receiver operating characteristic analysis in arthritis detection revealed diagnostic sensitivity of 85.7% and 71.4% and specificity of 80.0% and 93.3% at a cut-off points of Tmean ≥ 31.0 C and Tmax ≥ 32.3 C respectively. For US, GSUS and PDUS are useful in detecting arthritis, providing high sensitivity (83.3%) and specificity (81.3%). Our study demonstrated that both IRT and US were applicable tools for detecting wrist arthritis.

  20. 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

  1. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... vein that are done regularly at the hospital. Physical Therapy An appropriate physical therapy program is essential to the management of any type of arthritis. A physical therapist will explain the importance of certain activities ...

  2. Rare causes of osteoporosis

    PubMed Central

    Marcucci, Gemma; Brandi, Maria Luisa

    2015-01-01

    Summary Osteoporosis is a metabolic bone disease characterized by loss of bone mass and strength, resulting in increased risk of fractures. It is classically divided into primary (post-menopausal or senile), secondary and idiopathic forms. There are many rare diseases, that cause directly or indirectly osteoporosis. The identification and classification of most of these rare causes of osteoporosis is crucial for the specialists in endocrinology and not, in order to prevent this bone complication and to provide for an early therapy. Several pathogenic mechanisms are involved, including various aspects of bone metabolism such as: decreased bone formation, increased bone resorption, altered calcium, phosphorus and/or vitamin D homeostasis, and abnormal collagen synthesis. In this review, less common forms of primary and secondary osteoporosis are described, specifying, if applicable: genetic causes, epidemiology, clinical features, and pathogenic mechanisms causing osteoporosis. A greater awareness of all rare causes of osteoporosis could reduce the number of cases classified as idiopathic osteoporosis and allow the introduction of appropriate and timely treatments. PMID:26604941

  3. Determination of anti-cyclic citrullinated peptide antibodies in the sera of patients with juvenile idiopathic arthritis.

    PubMed

    Low, Jason M; Chauhan, Anil K; Kietz, Daniel A; Daud, Umar; Pepmueller, Peri H; Moore, Terry L

    2004-09-01

    Anti-cyclic citrullinated peptide (anti-CCP) antibodies have been found in sera of 76% of patients with rheumatoid arthritis (RA), mainly in rheumatoid factor (RF) positive patients, with a specificity of 96%. We evaluated the presence of anti-CCP antibodies in patients with juvenile idiopathic arthritis (JIA) and assessed the possibility of synthetic citrullinated peptides as antigenic determinants in JIA. The presence of anti-CCP antibodies was determined using 3 synthetic citrullinated peptide variants and 2 commercial kits (Inova Diagnostics and Axis-Shield Diagnostics) optimized for detecting JIA-specific antibodies in serum by an ELISA based assay. We evaluated 66 patients with JIA (16 RF positive polyarthritis, 18 RF negative polyarthritis, 19 oligoarthritis, and 13 systemic arthritis). We also tested 9 adult RA patients, 34 patients with systemic lupus erythematosus (SLE), and 25 healthy persons as controls. Significant concentrations of anti-CCP antibodies were detected in the majority of RF positive JIA patients with polyarthritis. Using the 2 synthetic linear peptides, 12/16 (75%) were positive; 9/12 (75%) were positive with the Inova kit and 9/10 (90%) were positive with the Axis-Shield kit. However, utilizing the synthetic linear peptides, significant concentrations of anti-CCP antibodies were detected in 51/66 (77%) JIA patients, including 15/18 (83%) RF negative polyarthritis, 16/19 (84%) oligoarthritis, and 8/13 (62%) systemic arthritis patients. No healthy control showed elevated antibody levels. In contrast, 4/9 (44%) patients with adult RA and 2/6 (33%) with SLE had elevated anti-CCP levels. The synthetic cyclic variant cfc-1-cyc yielded significant anti-CCP levels for 13/14 (93%) patients with RF negative polyarthritis, 6/10 (60%) with oligoarthritis, and 3/7 (43%) with systemic arthritis, and 8/9 (88%) RF positive patients. No healthy control had increased anti-CCP levels. However, 4/9 (44%) adult RA and 9/34 (26%) SLE patients were found to

  4. Evidence of fibrinogen as a target of citrullination in IgM rheumatoid factor-positive polyarticular juvenile idiopathic arthritis

    PubMed Central

    2011-01-01

    Background Several studies have noted the significance of measuring anti-cyclic citrullinated peptide (CCP) antibodies in juvenile idiopathic arthritis (JIA) as an important indicator for destructive disease, as is the case in rheumatoid arthritis (RA). While the role of anti-CCP antibodies in RA and JIA has become better understood, the identity of the target proteins of this modification has remained elusive. In this study, we evaluated serum from patients with various subtypes of JIA to investigate the presence of anti-deiminated (citrullinated) fibrinogen and anti-citrullinated α-enolase antibodies, and their association with RF and anti-CCP antibody isotypes. Methods Sera were obtained from 96 JIA patients, 19 systemic lupus erythematosus (SLE) patients, and 10 healthy children. All sera were measured for antibodies against citrullinated and native fibrinogen and α-enolase by an enzyme linked immunosorbent assay (ELISA). In addition, all sera were assayed for anti-CCP antibody isotypes and rheumatoid factor (RF) isotypes by ELISA. The relationship between anti-citrullinated fibrinogen and anti-α-enolase antibodies and disease activity and joint damage were also investigated. All results were correlated with clinical and laboratory parameters using Spearman's rho correlation coefficient. Multiple logistic regression analysis was utilized to identify which variables were associated with joint erosions and diagnosis of JIA. Results Thirty-one JIA patients (32%) demonstrated reactivity to citrullinated fibrinogen and 9 (9%) to citrullinated α-enolase. Reactivity to citrullinated fibrinogen and α-enolase was predominantly found in IgM RF-positive polyarthritis patients. Fourteen JIA patients reacted with native α-enolase and a higher percentage of SLE patients reacted with citrullinated α-enolase when compared to JIA patients. Anti-citrullinated fibrinogen antibodies correlated with the presence of IgG anti-CCP antibodies and IgA and IgM RF. The presence of

  5. In favour of the definition "adolescents with idiopathic scoliosis": juvenile and adolescent idiopathic scoliosis braced after ten years of age, do not show different end results. SOSORT award winner 2014

    PubMed Central

    2014-01-01

    Background The most important factor discriminating juvenile (JIS) from adolescent idiopathic scoliosis (AIS) is the risk of deformity progression. Brace treatment can change natural history, even when risk of progression is high. The aim of this study was to compare the end of growth results of JIS subjects, treated after 10 years of age, with final results of AIS. Methods Design: prospective observational controlled cohort study nested in a prospective database. Setting: outpatient tertiary referral clinic specialized in conservative treatment of spinal deformities. Inclusion criteria: idiopathic scoliosis; European Risser 0–2; 25 degrees to 45 degrees Cobb; start treatment age: 10 years or more, never treated before. Exclusion criteria: secondary scoliosis, neurological etiology, prior treatment for scoliosis (brace or surgery). Groups: 27 patients met the inclusion criteria for the AJIS, (Juvenile Idiopathic Scoliosis treated in adolescence), demonstrated by an x-ray before 10 year of age, and treatment start after 10 years of age. AIS group included 45 adolescents with a diagnostic x-ray made after the threshold of age 10 years. Results at the end of growth were analysed; the threshold of 5 Cobb degree to define worsened, improved and stabilized curves was considered. Statistics: Mean and SD were used for descriptive statistics of clinical and radiographic changes. Relative Risk of failure (RR), Chi-square and T-test of all data was calculated to find differences among the two groups. 95% Confidence Interval (CI) , and of radiographic changes have been calculated. Results We did not find any Cobb angle significant differences among groups at baseline and at the end of treatment. The only difference was in the number of patients progressed above 45 degrees, found in the JIS group. The RR of progression of AJIS was, 1.35 (IC95% 0.57-3.17) versus AIS, and it wasn't statistically significant in the AJIS group, in respect to AIS group (p = 0.5338). Conclusion

  6. DRESS-syndrome on sulfasalazine and naproxen treatment for juvenile idiopathic arthritis and reactivation of human herpevirus 6 in an 11-year-old Caucasian boy.

    PubMed

    Piñana, E; Lei, S H; Merino, R; Melgosa, M; De La Vega, R; Gonzales-Obeso, E; Ramírez, E; Borobia, A; Carcas, A

    2010-06-01

    DRESS-syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) is a severe drug-induced hypersensitivity syndrome characterized by diffuse maculopapular rash, lymphadenopathy, multivisceral involvement, eosinophilia and atypical lymphocytes with a mortality rate of 10-40% (Seminars in Cutaneous Medicine and Surgery, 1, 250). It is described in adults treated with aromatic antiepileptics and less frequently with sulphonamides, and non-steroidal anti-inflammatory drugs (Clinics in Dermatology, 23, 171; Pediatrics, 108, 485). We report on an 11-year-old Caucasian boy hospitalized with a skin eruption, lymphadenopathy, acute hepatitis, renal tubular involvement, haematological abnormalities and human-herpevirus-6 reactivation, treated with sulfasalazine and naproxen for juvenile idiopathic arthritis (JIA). This is the first report in children with rheumatic disease and highlights the possibility of sulfasalazine and naproxen-induced-DRESS-syndrome in children with JIA.

  7. Functional changes following distraction osteogenesis treatment of asymmetric mandibular growth deviation in unilateral juvenile idiopathic arthritis: a prospective study with long-term follow-up.

    PubMed

    Nørholt, S E; Pedersen, T K; Herlin, T

    2013-03-01

    In juvenile idiopathic arthritis (JIA), temporomandibular joint involvement is a frequent complication leading to deficient mandibular growth. Occurring unilaterally this will give rise to mandibular and maxillary asymmetry that will affect the soft tissue and the muscles and result in complex dentofacial anomaly. In the case of severe dentofacial malformation, orthognathic surgery is the only treatment option. Vertical osseodistraction of the mandibular ramus has been suggested as a means of rectifying the mandibular growth deviation and soft-tissue problems. Whether such treatment introduces dysfunctional side effects of the temporomandibular joint and muscles has been debated and concern has been raised that treatment impairs the patient's mouth opening capacity and mandibular movement. The present study prospectively evaluated 23 patients with JIA and mandibular asymmetry caused by unilateral temporomandibular joint arthritis. The authors found a clinical effect on the asymmetry with only minor subjective complaints and limited objective changes in functional parameters.

  8. Selecting magnetic resonance imaging (MRI) outcome measures for juvenile idiopathic arthritis (JIA) clinical trials: first report of the MRI in JIA special interest group.

    PubMed

    Hemke, Robert; Doria, Andrea S; Tzaribachev, Nikolay; Maas, Mario; van der Heijde, Désirée M F M; van Rossum, Marion A J

    2014-02-01

    Recent advances in magnetic resonance imaging (MRI) techniques have substantially improved the evaluation of joint pathologies in juvenile idiopathic arthritis (JIA). Because of the current availability of highly effective antirheumatic therapies and the unique and useful features of MRI, there is a growing need for an accurate and reproducible MRI assessment scoring system for JIA, such as the rheumatoid arthritis MRI Scoring (RAMRIS) for patients with rheumatoid arthritis (RA). To effectively evaluate the efficacy of treatment in clinical research trials, we need to develop and validate scoring methods to accurately measure joint outcomes, standardize imaging protocols for data acquisition and interpretation, and create imaging atlases to differentiate physiologic and pathologic joint findings in childhood and adolescence. Such a standardized, validated, JIA-MRI scoring method could be used as an outcome measure in clinical trials.

  9. 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

    2016-10-27

    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.

  10. 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.

  11. IGF-1 Receptor Expression on Circulating Osteoblast Progenitor Cells Predicts Tissue-Based Bone Formation Rate and Response to Teriparatide in Premenopausal Women With Idiopathic Osteoporosis.

    PubMed

    Cohen, Adi; Kousteni, Stavroula; Bisikirska, Brygida; Shah, Jayesh G; Manavalan, J Sanil; Recker, Robert R; Lappe, Joan; Dempster, David W; Zhou, Hua; McMahon, Donald J; Bucovsky, Mariana; Kamanda-Kosseh, Mafo; Stubby, Julie; Shane, Elizabeth

    2017-06-01

    We have previously reported that premenopausal women with idiopathic osteoporosis (IOP) have profound microarchitectural deficiencies and heterogeneous bone remodeling. Those with the lowest bone formation rate have higher baseline serum insulin-like growth factor-1 (IGF-1) levels and less robust response to teriparatide. Because IGF-1 stimulates bone formation and is critical for teriparatide action on osteoblasts, these findings suggest a state of IGF-1 resistance in some IOP women. To further investigate the hypothesis that osteoblast and IGF-1-related mechanisms mediate differential responsiveness to teriparatide in IOP, we studied circulating osteoblast progenitor (COP) cells and their IGF-1 receptor (IGF-1R) expression. In premenopausal women with IOP, peripheral blood mononuclear cells (PBMCs) were obtained at baseline (n = 25) and over 24 months of teriparatide treatment (n = 11). Flow cytometry was used to identify and quantify COPs (non-hematopoetic lineage cells expressing osteocalcin and RUNX2) and to quantify IGF-1R expression levels. At baseline, both the percent of PBMCs that were COPs (%COP) and COP cell-surface IGF-1R expression correlated directly with several histomorphometric indices of bone formation in tetracycline-labeled transiliac biopsies. In treated subjects, both %COP and IGF-1R expression increased promptly after teriparatide, returning toward baseline by 18 months. Although neither baseline %COP nor increase in %COP after 3 months predicted the bone mineral density (BMD) response to teriparatide, the percent increase in IGF-1R expression on COPs at 3 months correlated directly with the BMD response to teriparatide. Additionally, lower IGF-1R expression after teriparatide was associated with higher body fat, suggesting links between teriparatide resistance, body composition, and the GH/IGF-1 axis. In conclusion, these assays may be useful to characterize bone remodeling noninvasively and may serve to predict early response to

  12. A case-control study of fractures in men with idiopathic osteoporosis: fractures are associated with older age and low cortical bone density.

    PubMed

    Ostertag, Agnès; Collet, Corinne; Chappard, Christine; Fernandez, Sylvie; Vicaut, Eric; Cohen-Solal, Martine; de Vernejoul, Marie-Christine

    2013-01-01

    To determine biochemical, radiological and micro-architectural bone factors related to fragility fractures in idiopathic male osteoporosis (IMO) patients. IMO is a rare disorder characterized by low areal bone mineral density (aBMD) (Z-score<-2) occurring in men after excluding secondary causes of low BMD. We conducted a case-control study in 31 patients with fragility fracture (IMO F+) that had occurred after the age of 40 years and 37 without fracture (IMO F-). We first compared IMO group to 40 age-matched disease-free men. We measured aBMD and bone micro-architectural indices at distal radius and tibia sites with a HR-pQCT scan (XtremeCT) using standard and extended cortical analysis. Urine and blood samples were collected in order to determine the levels of bone-turnover markers and the potential determinant of bone fragility. Models of analysis of covariance, including age, height and weight as adjustment factors, were used to compare the groups. Compared to their controls, IMO patients showed marked disturbance of their micro-architectural parameters at tibia and radius affecting both trabecular and cortical parameters. IMO F+ subjects were significantly older than IMO F- subjects (58 ± 8 vs. 53 ± 9 yrs, p=0.01). BMD Z-score at the total-hip was significantly lower in IMO F+ (-1.3 ± 0.5 vs. -0.9 ± 0.8 g/cm(2), p=0.01). After adjustment, trabecular micro-architectural parameters, biochemical markers and hormonal parameters were not different in the 2 groups. At distal tibia, cortical v-BMD was significantly lower in IMO F+ patients (799 ± 73 vs. 858 ± 60 mg/cm(3), p=0.03), while cortical thickness was not different. Our results show that patients with IMO display a marked disturbance of trabecular and cortical bone micro-architecture, and that age and low cortical density are determinants of the fracture occurrence. Copyright © 2012 Elsevier Inc. All rights reserved.

  13. 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 Central

    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; Santos, Maria José; Melo-Gomes, José A; Fonseca, João Eurico

    2016-01-01

    Objectives 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. Methods 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. Results 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. Conclusions 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

  14. Osteoporosis Overview

    MedlinePlus

    ... supported by your browser. Home Osteoporosis Osteoporosis Basics Osteoporosis Overview Publication available in: PDF (23 KB) Related ... products. NIH Pub. No. 15-AR-8004 NIH Osteoporosis and Related Bone Diseases ~ National Resource Center 2 ...

  15. 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

  16. Evolving spectrum of LRBA deficiency-associated chronic arthritis: is there a causative role in juvenile idiopathic arthritis?

    PubMed

    Al-Mayouf, Sulaiman M; Naji, Hamzah; Alismail, Khalid; Alazami, Anas M; Sheikh, Farrukh; Conca, Walter; Al-Mousa, Hamoud

    2017-01-01

    Lipopolysaccharide-responsive, beige-like anchor protein (LRBA) deficiency causes common variable immunodeficiency (CVID) disorders and autoimmunity. LRBA deficiency has become a clinically variable syndrome with a wide spectrum of clinical manifestations. We report a patient with LRBA deficiency associated chronic non-erosive arthritis. This report highlights the spectrum of arthritis in such patients and the potential causative role of LRBA gene in juvenile arthritis.

  17. Pain in School: Patterns of Pain-Related School Impairment among Adolescents with Primary Pain Conditions, Juvenile Idiopathic Arthritis Pain, and Pain-Free Peers

    PubMed Central

    Agoston, Anna Monica; Gray, Laura S.; Logan, Deirdre E.

    2016-01-01

    Children with chronic pain frequently experience impairment in the school setting, but we do not yet understand how unique these struggles are to children with primary pain conditions compared to peers with disease-related pain or those without chronic pain symptoms. The objective of this study is to examine school functioning, defined as school attendance rates, overall quality of life in the school setting, and school nurse visits among adolescents with primary pain conditions, those with juvenile idiopathic arthritis (JIA)-related pain, and healthy peers. Two hundred and sixty adolescents participated in the study, including 129 with primary pain conditions, 61 with JIA, and 70 healthy comparison adolescents. They completed self- and parent-reported measures of school function. Findings show that as a group, youth with primary pain conditions reported more school absences, lower quality of life in the school setting, and more frequent school nurse visits compared to both adolescents with JIA-related pain and healthy peers. We conclude that compared to those who experience pain specific to a disease process, adolescents with primary pain conditions may face unique challenges in the school setting and may require more support to help them succeed in school in spite of pain. PMID:27916882

  18. Disruption of vascular endothelial homeostasis in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The dynamic roles of angiopoietin-1 and -2.

    PubMed

    Tasaki, Yuko; Shimizu, Masaki; Inoue, Natsumi; Mizuta, Mao; Nakagishi, Yasuo; Wada, Taizo; Yachie, Akihiro

    2016-04-01

    To assess the role of angiopoietin (Ang)-1 and Ang-2 and to investigate the clinical significance of serum levels of them in systemic juvenile idiopathic arthritis (s-JIA)-associated macrophage activation syndrome (MAS), we determined these levels in 51 patients with s-JIA, 11 patients with polyarticular JIA (poly-JIA), 12 patients with virus associated hemophagocytic syndrome (VAHS), 12 patients with Kawasaki disease (KD), and 15 age-matched healthy controls (HC). The results were compared with clinical features of MAS. During the MAS phase, serum Ang-1 levels were significantly decreased compared with those during the active and inactive phases. Serum Ang-2/1 ratio were significantly elevated during the MAS phase, compared with those during the active and inactive phases. There was a rapid increase in the Ang-2/1 ratio at the onset of MAS. Serum Ang-1 and the Ang-2/1 ratio significantly correlated with measures of disease activity, including AST and LDH. Ang-2/1 dysregulation was also observed in patients with VAHS, whereas not observed in most cases of KD. The homeostasis of vascular endothelial function by Ang-1 and Ang-2 is disrupted in MAS. Serum Ang-1 levels and the Ang-2/1 ratio might represent promising indicators of disease activity for MAS.

  19. CD3(+)CD56(+) natural killer T cell activity in children with different forms of juvenile idiopathic arthritis and the influence of etanercept treatment on polyarticular subgroup.

    PubMed

    Zhou, Juan; Ding, Yuan; Zhang, Yu; Feng, Ye; Tang, Xuemei; Zhao, Xiaodong

    2017-03-01

    Juvenile idiopathic arthritis (JIA) has three major onset types with widely varying clinical features. We assessed the natural killer T (NKT) cell function in patients with different JIA subtypes, and found systemic patients exhibited lower NKT cell counts, perforin and granzyme B expression, while the pauciarticular and polyarticular patients displayed higher perforin and granzyme B expression as compared with the controls. The synovial fluid had more NKT cells with higher levels of perforin, granzyme B, and tumour necrosis factor (TNF)-α than peripheral cells. The polyarticular patients that responded to etanercept had lower NKT cell counts, intracellular perforin, granzyme B and the mean fluorescence intensity of TNF-α than the patients that did not respond. Treatment with etanercept reduced the granzyme B and perforin, interferon (IFN)-γ and TNF-α expression in NKT cells in the responsive group. Therefore, a higher NKT cell function may indicate a decreased response to etanercept in polyarticular patients. Copyright © 2016. Published by Elsevier Inc.

  20. IGF-I and IGF-binding proteins in articular exudates of children with post-traumatic knee damage and juvenile idiopathic arthritis.

    PubMed

    Guszczyn, Tomasz; Rzeczycka, Justyna; Popko, Janusz

    2009-01-01

    IGF-I stimulates multiple functions of connective tissue cells and its activity is modulated by IGF-binding proteins (BPs). Some metalloproteinases are expected to modify IGF-I activity by digestion of IGF-BPs. It was decided to evaluate the concentration of IGF-I, IGF-BPs and the activity of gelatinases A and B in knee exudates of children with post-traumatic damage (PTD) and children with juvenile idiopathic arthritis (JIA) in comparison with those in the sera of the same patients. ELISA (for IGF-I assay), polyacrylamine gel electrophoresis following Western immunoblotting (for IGF-I and IGF-BPs expression), and zymography (for gelatinase detection) were used. The knee exudates, especially those taken from patients with JIA, contained large amounts of IGF-I. The exudates of PTD and JIA patients contained some forms of IGF-BP-1 of molecular weight lower than those occurring in serum. Low expression BP-3 and high activity of gelatinase B were detected in the JIA exudates. The high gelatinase activities in exudates imply joint tissue damage. The cellular response to damage of this kind is an increase in IGF-I production, which stimulates repair processes. High proteolytic activities of gelatinase B in JIA patients may lower the amount of BP-3, possibly causing a relative decrease of IGF-I concentration and impairing the reparation processes stimulated by IGF-I. Copyright 2009 S. Karger AG, Basel.

  1. A novel reactive epitope-based antigen targeted by serum autoantibodies in oligoarticular and polyarticular juvenile idiopathic arthritis and development of an electrochemical biosensor.

    PubMed

    Araujo, Galber R; Fujimura, Patricia T; Vaz, Emília R; Silva, Tamiris A; Rodovalho, Vinícius R; Britto-Madurro, Ana Graci; Madurro, João M; Fonseca, João E; Silva, Carlos H M; Santos, Paula S; Mourão, Ana F; Canhão, Helena; Goulart, Luiz R; Gonçalves, João; Ueira-Vieira, Carlos

    2016-05-01

    Currently, there are no specific markers for juvenile idiopathic arthritis (JIA) diagnosis, which is based on clinical symptoms and some blood tests for diseases' exclusion. Aiming to select new epitope-based antigens (mimotopes) that could recognize circulating autoantibodies in most JIA forms, we screened a phage displayed random peptide library against IgG antibodies purified from serum of JIA patients. ELISA assay was carried out to confirm immunoreactivity of selected peptides against sera IgG antibodies from JIA patients, healthy children and patients with other autoimmune diseases. The mimotope PRF+1 fused to phage particles was able to efficiently discriminate JIA patients from controls, and for this reason was chosen to be chemically synthesized for validation in a larger sample size. The synthetic peptide was immobilized onto bioelectrodes' surface for antibody detection by electrochemical analyses through differential pulse voltammetry. The PRF+1 synthetic peptide has efficiently discriminated JIA patients from control groups (p<0.0001) with a very good accuracy (AUC>0.84; sensitivity=61%; specificity=91%). The electrochemical platform proved to be fast, low cost and effective in detecting anti-PRF+1 antibodies from JIA patients compared to healthy controls (p=0.0049). Our study describes a novel and promising epitope-based biomarker for JIA diagnosis that can become a useful tool for screening tests, which was successfully incorporated onto an electrochemical biosensor and could be promptly used in field diagnostics.

  2. A CLEC16A variant confers risk for juvenile idiopathic arthritis and anti-cyclic citrullinated peptide antibody negative rheumatoid arthritis.

    PubMed

    Skinningsrud, Beate; Lie, Benedicte A; Husebye, Eystein S; Kvien, Tore K; Førre, Øystein; Flatø, Berit; Stormyr, Alice; Joner, Geir; Njølstad, Pål R; Egeland, Thore; Undlien, Dag E

    2010-08-01

    Variants in CLEC16A have conferred susceptibility to autoimmune diseases in genome-wide association studies. The present work aimed to investigate the locus' involvements in juvenile idiopathic arthritis (JIA) and further explore the association with rheumatoid arthritis (RA), type 1 diabetes (T1D) and Addison's disease (AD) in the Norwegian population. Three single nucleotide polymorphisms (SNPs) were genotyped in patients with RA (n=809), JIA (n=509), T1D (n=1211) and AD (n=414) and in healthy controls (n=2149). All diseases were associated with CLEC16A, but with different SNPs. The intron 22 SNP, rs6498169, was associated with RA (p=0.006) and JIA (p=0.016) and the intron 19 SNPs, rs12708716/rs12917716, with T1D (p=1x10-5) and AD (p=2x10-4). The RA association was confined to the anti-cyclic citrullinated peptide antibody (anti-CCP) negative subgroup (p=2x10-4). This is the first report of a CLEC16A association with JIA and a split of the RA association according to anti-CCP status. Different causative variants underlie the rheumatic versus the organ specific diseases.

  3. Struggle and adjustment to an insecure everyday life and an unpredictable life course. Living with juvenile idiopathic arthritis from childhood to adult life - an interview study.

    PubMed

    Ostlie, Ingrid Landgraff; Johansson, Inger; Möller, Anders

    2009-01-01

    . To obtain a deeper understanding of the meaning of living with juvenile idiopathic arthritis (JIA) through childhood and adolescence into adult life as described by young adults with JIA themselves. Based on a qualitative study design, 15 young adults were interviewed individually. The data were analysed using a qualitative content analysis approach. Living with JIA involves struggle and adjustment to an insecure everyday life and an unpredictable life course. The informants' experiences emerged as dichotomies on a continuum describing the dynamics in life experiences individually and over time. The categories include bodily experiences of limitations or freedom, being acknowledged or set aside in interpersonal relationships, and intrapersonal experiences of insecurity or confidence. The findings indicate a change to greater acceptance and adjustment to the disease over time. The impact of JIA on life in a time of transition from childhood to adult life involves complex challenges on coping strategies and adjustment processes. Understanding this complexity is urgent for health professionals to contribute to both normal developmental task achievements and overall well-being for young people with JIA. Further investigations should focus on coping and adjustment processes when facing disease fluctuations and unpredictability in a life-span perspective.

  4. 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.

  5. 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.

  6. Changes in fecal microbiota and metabolomics in a child with juvenile idiopathic arthritis (JIA) responding to two treatment periods with exclusive enteral nutrition (EEN).

    PubMed

    Berntson, Lillemor; Agback, Peter; Dicksved, Johan

    2016-06-01

    The microbiome and immune system of the digestive tract are highly important in both health and disease. Exclusive enteral nutrition (EEN) is a common anti-inflammatory treatment in children with Crohn's disease in the European countries, and the mechanism is most likely linked to changes in the intestinal microbiome. In the present study, EEN was given in two treatment periods several months apart to a patient with very severe, disabling juvenile idiopathic arthritis (JIA), with a remarkable clinical response as the result. The aim of the present study was to study how the EEN treatment influenced the microbiome and metabolome of this patient. Fecal samples from before, during, and between treatments with EEN were studied. The microbiome was analyzed by sequencing of 16S rRNA amplicons using Illumina MiSeq, and the metabolome was analyzed using nuclear magnetic resonance. The microbiome changed markedly from treatment with EEN, with a strong reduction of the Bacteroidetes phylum. Metabolic profiles showed clear differences before, during, and between treatment with EEN, where butyrate, propionate, and acetate followed a cyclic pattern with the lowest levels at the end of each treatment period. This patient with JIA showed remarkable clinical improvement after EEN treatment, and we found corresponding changes in both the fecal microbiome and the metabolome. Further studies are needed to explore the pathophysiological role of the intestinal canal in children with JIA.

  7. Pain in School: Patterns of Pain-Related School Impairment among Adolescents with Primary Pain Conditions, Juvenile Idiopathic Arthritis Pain, and Pain-Free Peers.

    PubMed

    Agoston, Anna Monica; Gray, Laura S; Logan, Deirdre E

    2016-11-30

    Children with chronic pain frequently experience impairment in the school setting, but we do not yet understand how unique these struggles are to children with primary pain conditions compared to peers with disease-related pain or those without chronic pain symptoms. The objective of this study is to examine school functioning, defined as school attendance rates, overall quality of life in the school setting, and school nurse visits among adolescents with primary pain conditions, those with juvenile idiopathic arthritis (JIA)-related pain, and healthy peers. Two hundred and sixty adolescents participated in the study, including 129 with primary pain conditions, 61 with JIA, and 70 healthy comparison adolescents. They completed self- and parent-reported measures of school function. Findings show that as a group, youth with primary pain conditions reported more school absences, lower quality of life in the school setting, and more frequent school nurse visits compared to both adolescents with JIA-related pain and healthy peers. We conclude that compared to those who experience pain specific to a disease process, adolescents with primary pain conditions may face unique challenges in the school setting and may require more support to help them succeed in school in spite of pain.

  8. 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

  9. Clinical Orofacial Examination in Juvenile Idiopathic Arthritis: International Consensus-based Recommendations for Monitoring Patients in Clinical Practice and Research Studies.

    PubMed

    Stoustrup, Peter; Twilt, Marinka; Spiegel, Lynn; Kristensen, Kasper Dahl; Koos, Bernd; Pedersen, Thomas Klit; Küseler, Annelise; Cron, Randy Q; Abramowicz, Shelly; Verna, Carlalberta; Peltomäki, Timo; Alstergren, Per; Petty, Ross; Ringold, Sarah; Nørholt, Sven Erik; Saurenmann, Rotraud K; Herlin, Troels

    2017-03-01

    To develop international consensus-based recommendations for the orofacial examination of patients with juvenile idiopathic arthritis (JIA), for use in clinical practice and research. Using a sequential phased approach, a multidisciplinary task force developed and evaluated a set of recommendations for the orofacial examination of patients with JIA. Phase 1: A Delphi survey was conducted among 40 expert physicians and dentists with the aim of identifying and ranking the importance of items for inclusion. Phase 2: The task force developed consensus about the domains and items to be included in the recommendations. Phase 3: A systematic literature review was performed to assess the evidence supporting the consensus-based recommendations. Phase 4: An independent group of orofacial and JIA experts were invited to assess the content validity of the task force's recommendations. Five recommendations were developed to assess the following 5 domains: medical history, orofacial symptoms, muscle and temporomandibular joint function, orofacial function, and dentofacial growth. After application of data search criteria, 56 articles were included in the systematic review. The level of evidence for the 5 recommendations was derived primarily from descriptive studies, such as cross-sectional and case-control studies. Five recommendations are proposed for the orofacial examination of patients with JIA to improve the clinical practice and aid standardized data collection for future studies. The task force has formulated a future research program based on the proposed recommendations.

  10. Male osteoporosis: A review

    PubMed Central

    Herrera, Antonio; Lobo-Escolar, Antonio; Mateo, Jesús; Gil, Jorge; Ibarz, Elena; Gracia, Luis

    2012-01-01

    Osteoporosis in men is a heterogeneous disease that has received little attention. However, one third of worldwide hip fractures occur in the male population. This problem is more prevalent in people over 70 years of age. The etiology can be idiopathic or secondary to hypogonadism, vitamin D deficiency and inadequate calcium intake, hormonal treatments for prostate cancer, use of toxic and every disease or drug use that alters bone metabolism. Risk factors such as a previous history of fragility fracture should be assessed for the diagnosis. However, risk factors in men are very heterogeneous. There are significant differences in the pharmacological treatment of osteoporosis between men and women fundamentally due to the level of evidence in published trials supporting each treatment. New treatments will offer new therapeutic prospects. The goal of this work is a revision of the present status knowledge about male osteoporosis. PMID:23362466

  11. Low rate of surgery in juvenile idiopathic scoliosis treated with a complete and tailored conservative approach: end-growth results from a retrospective cohort

    PubMed Central

    2014-01-01

    Background context The main distinctive aspect of Juvenile Idiopathic Scoliosis (JIS) with respect to Adolescent Idiopathic Scoliosis (AIS) is the high risk of severe deformity and surgery. Approximately 70% of curves in patients with JIS progress and ultimately require surgery. There are presently very few studies with long-term follow-up of JIS and even fewer looking specifically at bracing Purpose To verify the effectiveness of a complete conservative treatment, including bracing and exercises, for JIS. Study design/setting Retrospective cohort observational study nested in a clinical prospective database of consecutive outpatients. Patient Sample Inclusion criteria: JIS, no previous treatment, all consecutive radiographies available from treatment start to end of growth (Risser sign 3). We found 30 patients, 27 females, 10 JIS type 1; mean age at first diagnosis was 7.8 +/-1.5 and mean treatment lasted 5.8 years. Cobb degrees 24.4+/-10 degrees, with 7 cases >30 degrees, and 2 > 45degrees. Outcome Measures Physiological measures. Radiographic and clinical data. Methods Treatment (exercises alone, or elastic-rigid-highly rigid braces plus exercises) was tailored and continuously changed according to Cobb degrees, individual preferences, anthropometric characteristics, pubertal spurt, remaining growth, rotation, hump, lumbar curve take-off, and imbalance. The SOSORT Guidelines for patients’ management have been followed. Funding and Conflict of Interest: no. Results 33.3% (95% Confidence Interval 16.4-50.2%) of patients worsened over the years. At the end of growth, 6.6% (0–15.5%) had surgical deformities (>45degrees). We observed a good correction in the first years of treatment until pubertal growth spurt, when progression was usually noted and treatment changed increasing corrective forces (hours or rigidity of bracing). 23 cases were followed up until they had two consecutive radiographies showing Risser sign 5 and showed stability. Conclusions

  12. Effects of the live attenuated measles-mumps-rubella booster vaccination on disease activity in patients with juvenile idiopathic arthritis: a randomized trial.

    PubMed

    Heijstek, Marloes W; Kamphuis, Sylvia; Armbrust, Wineke; Swart, Joost; Gorter, Simone; de Vries, Lara D; Smits, Gaby P; van Gageldonk, Pieter G; Berbers, Guy A M; Wulffraat, Nico M

    2013-06-19

    The immunogenicity and the effects of live attenuated measles-mumps-rubella (MMR) vaccination on disease activity in patients with juvenile idiopathic arthritis (JIA) are matters of concern, especially in patients treated with immunocompromising therapies. To assess whether MMR booster vaccination affects disease activity and to describe MMR booster immunogenicity in patients with JIA. Randomized, multicenter, open-label clinical equivalence trial including 137 patients with JIA aged 4 to 9 years who were recruited from 5 academic hospitals in The Netherlands between May 2008 and July 2011. Patients were randomly assigned to receive MMR booster vaccination (n=68) or no vaccination (control group; n=69). Among patients taking biologics, these treatments were discontinued at 5 times their half-lives prior to vaccination. Disease activity as measured by the Juvenile Arthritis Disease Activity Score (JADAS-27), ranging from 0 (no activity) to 57 (high activity). Disease activity in the year following randomization was compared between revaccinated patients and controls using a linear mixed model. A difference in JADAS-27 of 2.0 was the equivalence margin. Primary immunogenicity outcomes were seroprotection rates and MMR-specific antibody concentrations at 3 and 12 months. Of 137 randomized patients, 131 were analyzed in the modified intention-to-treat analysis, including 60 using methotrexate and 15 using biologics. Disease activity during complete follow-up did not differ between 63 revaccinated patients (JADAS-27, 2.8; 95% CI, 2.1-3.5) and 68 controls (JADAS-27, 2.4; 95% CI, 1.7-3.1), with a difference of 0.4 (95% CI, -0.5 to 1.2), within the equivalence margin of 2.0. At 12 months, seroprotection rates were higher in revaccinated patients vs controls (measles, 100% vs 92% [95% CI, 84%-99%]; mumps, 97% [95% CI, 95%-100%] vs 81% [95% CI, 72%-93%]; and rubella, 100% vs 94% [95% CI, 86%-100%], respectively), as were antibody concentrations against measles (1.63 vs 0

  13. Linear growth in children suffering from juvenile idiopathic arthritis requiring steroid therapy: natural history and effects of growth hormone treatment on linear growth.

    PubMed

    Simon, D; Lucidarme, N; Prieur, A M; Ruiz, J C; Czernichow, P

    2001-01-01

    We assessed linear growth and final height retrospectively in a group of 24 patients suffering from juvenile idiopathic arthritis (JIA) during childhood who had received steroid therapy. In these patients, there was a significant loss of height of more than 2 standard deviations during the first years of the disease, which correlated positively with the duration of prednisone therapy. After remission of the disease and discontinuation of prednisone treatment, 70% of the patients achieved catch-up growth, although 30% showed a persistent loss of height. Their mean final height was strongly correlated with their mean height at the end of steroid therapy and was significantly different between the group of patients with catch-up growth and the group without catch-up growth. This pattern of growth observed in patients with JIA should help us to define strategies of growth hormone (GH) treatment in these patients in order to improve their final height. We have previously reported the beneficial effects on growth and body composition of 1 year of GH treatment in a group of 14 growth-retarded patients suffering from JIA who received glucocorticoid therapy. These patients (n = 13) were treated again with GH at the same dosage (0.46 mg/kg/week [0.07 mg/kg/day]) for another 3-year period. GH treatment markedly increased growth velocity in these patients, but had a minor effect on height SDS, suggesting that these children will remain short when adults. Starting GH therapy in these patients earlier after the onset of the disease may prevent growth deterioration and metabolic complications induced by chronic inflammation and long-term steroid therapy.

  14. Mental health and adjustment to juvenile idiopathic arthritis: Level of agreement between parent and adolescent reports according to Strengths and Difficulties Questionnaire and Adolescent Outcomes Questionnaire

    PubMed Central

    Adamczyk, Katarzyna A.; Niedziela, Marek; Głowacki, Maciej; Głowacki, Jakub

    2017-01-01

    The aims of this study were threefold. Firstly, to analyze the psychometric properties of the Polish-language Pediatric Outcomes Data Collection Instrument (PODCI) questionnaire in the self-report Adolescent Outcomes Questionnaire (adolescents, 11–18 years of age) and in the parent-report Adolescent Outcomes Questionnaire (completed by a parent or guardian of an adolescent aged 11–18 years). Secondly, to determine the level of agreement between parents and adolescents in rating dysfunction in juvenile idiopathic arthritis (JIA) and thirdly, to examine associations between psychological adjustments of patients to JIA and disease as well as their socio-demographic characteristics. The study sample consisted of 52 participants. 26 adolescents between the ages of 11 and 18 years with a diagnosis of JIA and 26 parents were considered for inclusion. Disease course was classified as pauciarticular (n = 12, 46.2%) and polyarticular (n = 14, 53.8%). Participants completed the PODCI (self- and parent- report) twice and the Strengths and Difficulties Questionnaire-25 (SDQ-25). Considering the distribution of results regarding PODCI normative scores, 73.1% of parents and 69.2% of patients scored below 50 on the Global Functioning Scale; that is lower than the average for the general healthy population. Regarding the parent report, the total score of the SDQ-25 equaled 11.86 (SD 2.66), whereas the patient report equaled 11.23 (SD 2.78). The study groups do not differ significantly in regards to either the PODCI or the SDQ-25 results. Parents and adolescents with JIA appear to hold very similar perceptions of patients' health. Greater differences emerge as disease severity and age of patients increase. Excellent internal consistency, intrarater and test-retest reliability of the Global Functioning Scale have been confirmed in the Polish version of the PODCI, the questionnaire may therefore aid identification of patients reporting significant problems in this group. PMID

  15. 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.

  16. Validation of the Brazilian version of the pediatric outcomes data collection instrument: a cross-sectional evaluation in children and adolescents with juvenile idiopathic arthritis

    PubMed Central

    2013-01-01

    Background There is a lack of health-related quality of life (HRQOL) questionnaires to evaluate pediatric musculoskeletal diseases in Brazil. The Pediatric Outcome Data Collection Instrument (PODCI) is widely used elsewhere for pediatric patients with musculoskeletal disorders, but it has not been fully validated in Brazil. Validation of the PODCI in the Brazilian Portuguese language is important to improve the assessment of pediatric patients with musculoskeletal diseases and to compare Brazilian study results with results from the international literature. This study aimed to analyze the test–re-test reliability and the convergent validity indicators for the quality of life scores obtained by application of the PODCI to children and adolescents with juvenile idiopathic arthritis (JIA). Methods The PODCI underwent translation, transcultural adaptation, and field testing. Fifty-seven children and adolescents with JIA were administered the PODCI questionnaire. The Child Health Questionnaire - Parent Form 28 (CHQ PF-28) was used as the gold standard. Pain scales were employed, clinical examinations were performed, and laboratory inflammatory activity tests were conducted. Results The three versions of the PODCI exhibited good internal consistency (Cronbach’s alpha coefficient >0.70), good reproducibility (p < 0.05), and good correlation compared with the gold standard (CHQ), as shown by a Spearman coefficient (Rho) >0.40 (p < 0.05). Conclusions The PODCI was validated in patients with JIA in Brazil. This questionnaire was found to be valid, precise, and reliable. It can be successfully applied in research conducted by healthcare professionals who work with children and adolescents with musculoskeletal system disorders. PMID:24171906

  17. Prospective surveillance study of acute respiratory infections, influenza-like illness and seasonal influenza vaccine in a cohort of juvenile idiopathic arthritis patients

    PubMed Central

    2013-01-01

    Background Acute respiratory infections (ARI) are frequent in children and complications can occur in patients with chronic diseases. We evaluated the frequency and impact of ARI and influenza-like illness (ILI) episodes on disease activity, and the immunogenicity and safety of influenza vaccine in a cohort of juvenile idiopathic arthritis (JIA) patients. Methods Surveillance of respiratory viruses was conducted in JIA patients during ARI season (March to August) in two consecutive years: 2007 (61 patients) and 2008 (63 patients). Patients with ARI or ILI had respiratory samples collected for virus detection by real time PCR. In 2008, 44 patients were immunized with influenza vaccine. JIA activity index (ACRPed30) was assessed during both surveillance periods. Influenza hemagglutination inhibition antibody titers were measured before and 30-40 days after vaccination. Results During the study period 105 ARI episodes were reported and 26.6% of them were ILI. Of 33 samples collected, 60% were positive for at least one virus. Influenza and rhinovirus were the most frequently detected, in 30% of the samples. Of the 50 JIA flares observed, 20% were temporally associated to ARI. Influenza seroprotection rates were higher than 70% (91-100%) for all strains, and seroconversion rates exceeded 40% (74-93%). In general, response to influenza vaccine was not influenced by therapy or disease activity, but patients using anti-TNF alpha drugs presented lower seroconversion to H1N1 strain. No significant differences were found in ACRPed30 after vaccination and no patient reported ILI for 6 months after vaccination. Conclusion ARI episodes are relatively frequent in JIA patients and may have a role triggering JIA flares. Trivalent split influenza vaccine seems to be immunogenic and safe in JIA patients. PMID:23510667

  18. 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

  19. 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

    2017-08-15

    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.

  20. Serum levels of osteoprotegerin and receptor activator of nuclear factor -κB ligand in children with early juvenile idiopathic arthritis: a 2-year prospective controlled study

    PubMed Central

    2010-01-01

    Background The clinical relevance of observations of serum levels of osteoprotegerin (OPG) and receptor activator of nuclear factor -κB ligand (RANKL) in juvenile idiopathic arthritis (JIA) is not clear. To elucidate the potential role of OPG and RANKL in JIA we determined serum levels of OPG and RANKL in patients with early JIA compared to healthy children, and prospectively explored changes in relation to radiographic score, bone and lean mass, severity of the disease, and treatment. Methods Ninety children with early oligoarticular or polyarticular JIA (ages 6-18 years; mean disease duration 19.4 months) and 90 healthy children individually matched for age, sex, race, and county of residence, were examined at baseline and 2-year follow-up. OPG and RANKL were quantified by enzyme-immunoassay. Data were analyzed with the use of t-tests, ANOVA, and multiple regression analyses. Results Serum OPG was significantly lower in patients than controls at baseline, and there was a trend towards higher RANKL and a lower OPG/RANKL ratio. Patients with polyarthritis had significantly higher increments in RANKL from baseline to follow-up, compared to patients with oligoarthritis. RANKL was a significant negative predictor for increments in total body lean mass. Patients who were receiving corticosteroids (CS) or disease-modifying antirheumatic drugs (DMARDs) at follow-up had higher OPG/RANKL ratio compared with patients who did not receive this medication. Conclusions The data supports that levels of OPG are lower in patients with JIA compared to healthy children, and higher levels of RANKL is associated with more serious disease. RANKL was a significant negative predictor of lean mass in patients with JIA. The OPG/RANKL ratio was higher in patients on DMARDs or CS treatment. PMID:21134287

  1. Long‐Term Safety, Efficacy, and Quality of Life in Patients With Juvenile Idiopathic Arthritis Treated With Intravenous Abatacept for Up to Seven Years

    PubMed Central

    Ruperto, Nicolino; Mouy, Richard; Paz, Eliana; Rubio‐Pérez, Nadina; Silva, Clovis A.; Abud‐Mendoza, Carlos; Burgos‐Vargas, Ruben; Gerloni, Valeria; Melo‐Gomes, Jose A.; Saad‐Magalhaes, Claudia; Chavez‐Corrales, J.; Huemer, Christian; Kivitz, Alan; Blanco, Francisco J.; Foeldvari, Ivan; Hofer, Michael; Huppertz, Hans‐Iko; Job Deslandre, Chantal; Minden, Kirsten; Punaro, Marilynn; Block, Alan J.; Giannini, Edward H.; Martini, Alberto

    2015-01-01

    Objective The efficacy and safety of abatacept in patients with juvenile idiopathic arthritis (JIA) who experienced an inadequate response to disease‐modifying antirheumatic drugs were previously established in a phase III study that included a 4‐month open‐label lead‐in period, a 6‐month double‐blind withdrawal period, and a long‐term extension (LTE) phase. The aim of this study was to present the safety, efficacy, and patient‐reported outcomes of abatacept treatment (10 mg/kg every 4 weeks) during the LTE phase, for up to 7 years of followup. Methods Patients enrolled in the phase III trial could enter the open‐label LTE phase if they had not achieved a response to treatment at month 4 or if they had received abatacept or placebo during the double‐blind period. Results One hundred fifty‐three (80.5%) of 190 patients entered the LTE phase, and 69 patients (36.3%) completed it. The overall incidence rate (events per 100 patient‐years) of adverse events decreased during the LTE phase (433.61 events during the short‐term phase [combined lead‐in and double‐blind periods] versus 132.39 events during the LTE phase). Similar results were observed for serious adverse events (6.82 versus 5.60), serious infections (1.13 versus 1.72), malignancies (1.12 versus 0), and autoimmune events (2.26 versus 1.18). American College of Rheumatology (ACR) Pediatric 30 (Pedi 30) responses, Pedi 70 responses, and clinically inactive disease status were maintained throughout the LTE phase in patients who continued to receive therapy. Improvements in the Child Health Questionnaire physical and psychosocial summary scores were maintained over time. Conclusion Long‐term abatacept treatment for up to 7 years was associated with consistent safety, sustained efficacy, and quality‐of‐life benefits in patients with JIA. PMID:26097215

  2. 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.

  3. Immunogenicity and safety of the bivalent HPV vaccine in female patients with juvenile idiopathic arthritis: a prospective controlled observational cohort study.

    PubMed

    Heijstek, Marloes W; Scherpenisse, Mirte; Groot, Noortje; Tacke, Carline; Schepp, Rutger M; Buisman, Anne-Marie; Berbers, Guy A M; van der Klis, Fiona R M; Wulffraat, Nico M

    2014-08-01

    To compare the immunogenicity and safety of the bivalent human papillomavirus (HPV)16/18 vaccine between female patients with juvenile idiopathic arthritis (JIA) and healthy female adolescents. 68 patients and 55 healthy girls aged 12-18 years were included in a prospective controlled observational cohort and were vaccinated at 0, 1 and 6 months. Primary outcomes were immunogenicity expressed as seropositivity rate after three vaccine doses at 7 and 12 months and HPV-specific geometric mean antibody concentrations. Secondary outcomes were HPV16/18-specific memory B cell responses in a subset of participants and safety, defined as adverse events and the effect of vaccination on JIA disease activity. All participants were seropositive for HPV16 and HPV18 at 7 months. One patient turned seronegative at 12 months for HPV16/18. No significant differences were found between patients and controls in HPV-specific antibody concentrations; however, antibody concentrations were consistently lower in patients. No effect of methotrexate on HPV16 antibodies (p=0.79) or HPV18 antibodies (p=0.37) was detected. All patients on anti-TNFα treatment were seropositive after vaccination. The kinetics of HPV16/18 memory B cell responses was comparable between patients and controls, but the magnitude of B cell responses at 7 and 12 months appeared lower in patients. No relevant differences in adverse events were found. HPV vaccination did not aggravate JIA disease. The bivalent HPV16/18 vaccine is immunogenic and well tolerated in JIA patients. However, HPV-specific antibodies and B cell responses tended to be lower in patients compared with healthy controls. NCT00815282. 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.

  4. Abnormal expression of the genes involved in cytokine networks and mitochondrial function in systemic juvenile idiopathic arthritis identified by DNA microarray analysis.

    PubMed

    Ishikawa, S; Mima, T; Aoki, C; Yoshio-Hoshino, N; Adachi, Y; Imagawa, T; Mori, M; Tomiita, M; Iwata, N; Murata, T; Miyoshi, M; Takei, S; Aihara, Y; Yokota, S; Matsubara, K; Nishimoto, N

    2009-02-01

    Systemic juvenile idiopathic arthritis (sJIA) is a rheumatic disease in childhood characterised by systemic symptoms and a relatively poor prognosis. Peripheral leukocytes are thought to play a pathological role in sJIA although the exact cause of the disease is still obscure. In this study, we aimed to clarify cellular functional abnormalities in sJIA. We analysed the gene expression profile in peripheral leukocytes from 51 patients with sJIA, 6 patients with polyarticular type JIA (polyJIA) and 8 healthy children utilising DNA microarrays. Gene ontology analysis and network analysis were performed on the genes differentially expressed in sJIA to clarify the cellular functional abnormalities. A total of 3491 genes were differentially expressed in patients with sJIA compared to healthy individuals. They were functionally categorised mainly into a defence response group and a metabolism group according to gene ontology, suggesting the possible abnormalities in these functions. In the defence response group, molecules predominantly constituting interferon (IFN)gamma and tumour necrosis factor (TNF) network cascades were upregulated. In the metabolism group, oxidative phosphorylation-related genes were downregulated, suggesting a mitochondrial disorder. Expression of mitochondrial DNA-encoded genes including cytochrome c oxidase subunit 1(MT-CO1) and MT-CO2 were suppressed in patients with sJIA but not in patients with polyJIA or healthy children. However, nuclear DNA-encoded cytochrome c oxidases were intact. Our findings suggest that sJIA is not only an immunological disease but also a metabolic disease involving mitochondria disorder.

  5. Adalimumab: long-term safety in 23 458 patients from global clinical trials in rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, psoriatic arthritis, psoriasis and Crohn's disease

    PubMed Central

    Burmester, Gerd R; Panaccione, Remo; Gordon, Kenneth B; McIlraith, Melissa J; Lacerda, Ana P M

    2013-01-01

    Background As long-term treatment with antitumour necrosis factor (TNF) drugs becomes accepted practice, the risk assessment requires an understanding of anti-TNF long-term safety. Registry safety data in rheumatoid arthritis (RA) are available, but these patients may not be monitored as closely as patients in a clinical trial. Cross-indication safety reviews of available anti-TNF agents are limited. Objective To analyse the long-term safety of adalimumab treatment. Methods This analysis included 23 458 patients exposed to adalimumab in 71 global clinical trials in RA, juvenile idiopathic arthritis, ankylosing spondylitis (AS), psoriatic arthritis, psoriasis (Ps) and Crohn's disease (CD). Events per 100 patient-years were calculated using events reported after the first dose through 70 days after the last dose. Standardised incidence rates for malignancies were calculated using a National Cancer Institute database. Standardised death rates were calculated using WHO data. Results The most frequently reported serious adverse events across indications were infections with greatest incidence in RA and CD trials. Overall malignancy rates for adalimumab-treated patients were as expected for the general population; the incidence of lymphoma was increased in patients with RA, but within the range expected in RA without anti-TNF therapy; non-melanoma skin cancer incidence was raised in RA, Ps and CD. In all indications, death rates were lower than, or equivalent to, those expected in the general population. Conclusions Analysis of adverse events of interest through nearly 12 years of adalimumab exposure in clinical trials across indications demonstrated individual differences in rates by disease populations, no new safety signals and a safety profile consistent with known information about the anti-TNF class. PMID:22562972

  6. Abnormal kappa:lambda light chain ratio in circulating immune complexes as a marker for B cell activity in juvenile idiopathic arthritis.

    PubMed

    Low, J M; Chauhan, A K; Moore, T L

    2007-01-01

    Patients with juvenile idiopathic arthritis (JIA) have been shown to have elevated levels of circulating immune complexes (CICs) which correlated with disease activity. Our aim was to assess B cell activity by measuring the amount of and the kappa:lambda chain immunoglobulin light (L) chain ratio in CICs from JIA patients and to determine potential evidence for either an antigen-driven response or B-cell receptor editing. We used an enzyme-linked immunosorbent assay to measure kappa and lambda chains present in the CICs from the sera of patients with JIA. Statistical analysis was performed using Pearson's correlation, one-way ANOVA and Bonferroni post hoc analysis. Sera from 44 JIA patients were examined for the concentration of L chains in CICs. Healthy controls had a kappa:lambda chain ratio of 1.2:1, whereas this ratio was reversed among JIA subgroups with RF-positive polyarthritis (1:1.2), RF-negative polyarthritis (1:1.3), oligoarthritis (1:2.3) and systemic-onset arthritis (1:2.5). In addition, overall lambda chain selection was not significantly associated with a particular immunoglobulin heavy (H) chain and occurred with all immunoglobulin isotypes. We showed preferential selection of lambda chains contributing to the formation of potentially pathogenic CICs from JIA patients, of all onset types compared to healthy controls, in an H chain-independent manner. The reversal of kappa:lambda chain ratio within the JIA CICs and association with all immunoglobulin isotypes demonstrated the potential for L chain editing. Furthermore, we conclude that a reversal of the normal kappa:lambda chain ratio in JIA CICs may be used as a marker for increased B-cell activity.

  7. 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

  8. Significance of complement components C1q and C4 bound to circulating immune complexes in juvenile idiopathic arthritis: support for classical complement pathway activation.

    PubMed

    Gilliam, Brooke E; Reed, Melinda R; Chauhan, Anil K; Dehlendorf, Amanda B; Moore, Terry L

    2011-01-01

    Immune complexes (ICs) from sera of juvenile idiopathic arthritis (JIA) patients show increased complement opsonisation; however, a definitive role for involvement of the classical or alternative pathway is not entirely clear. To delineate the role of these pathways, we measured activated complement products bound to circulating IC (CICs) in the sera of JIA patients. Sera from 100 JIA patients and 22 healthy children were collected. C1q, C4, C3, C3d, and membrane attack complex (MAC) bound to CICs were measured by enzyme-linked immunosorbent assay. Data was compared to IgM rheumatoid factor (RF), IgG anti-cyclic citrullinated peptide (CCP) antibodies, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) levels. Mean levels of C1q, C4, and MAC bound to CICs were significantly elevated in JIA patients compared to healthy children. C1q correlated significantly with C4 and MAC bound to CICs and C4 and MAC also demonstrated significant correlation. No significant differences were noted in complement components bound to CICs when evaluating IgM RF, anti-CCP antibody, and CRP positivity. A significant correlation was noted between MAC bound to CICs and ESR. C1q and MAC bound to CICs mean levels were significantly higher in patients with an elevated ESR compared to those with a normal ESR level. JIA patients have elevated levels of complement components bound to CICs, particularly from the classical pathway. Moreover, classical pathway components were associated with ESR, a marker of disease activity. MAC bound to CICs also correlated significantly with ESR, further supporting the notion of complement-mediated tissue injury that is triggered by IC-mediated classical pathway activation.

  9. Growing up and moving on. A multicentre UK audit of the transfer of adolescents with juvenile idiopathic arthritis from paediatric to adult centred care

    PubMed Central

    Robertson, L P; McDonagh, J E; Southwood, T R; Shaw, K L

    2006-01-01

    Objective To assess the provisions made for the transfer of adolescents with juvenile idiopathic arthritis to adult rheumatology clinics in the UK and the impact of a transitional care programme. Methods An audit of the documentation of the provisions made for transfer in 10 centres participating in a controlled trial of transitional care. Each centre conducted a retrospective case note audit of the recent patients transferred to adult care before and 12–24 months after the start of the trial. Demographic details, age when transition was first discussed, age at transfer, transitional issues, multidisciplinary team involvement, adolescent self advocacy, and readiness were documented. Results There were improvements at follow up in documentation of transitional issues, disease specific educational needs, adolescent readiness, and parental needs with the exception of dental care, dietary calcium, and home exercise programmes. The age at which the concept of an independent clinic visit was introduced was lower (mean (SD): 16.8 (1.06) v 15.8 (1.46) years, p = 0.01) but there were no other changes in age related transitional milestones. Significantly more participants had preparatory visits to the adult clinic, had a transition plan, and had joint injections while awake at follow up. Conclusions The improvement in documentation suggests that involvement in the research project increased awareness of transitional issues. The difficulty of changing policy into practice was highlighted, with room for improvement, particularly at the paediatric/adult interface. The reasons for this are likely to be multiple, including resources and lack of specific training. PMID:15994281

  10. Frequency of joint involvement in juvenile idiopathic arthritis during a 5-year follow-up of newly diagnosed patients: implications for MR imaging as outcome measure.

    PubMed

    Hemke, Robert; Nusman, Charlotte M; van der Heijde, Désirée M F M; Doria, Andrea S; Kuijpers, Taco W; Maas, Mario; van Rossum, Marion A J

    2015-02-01

    To assess the sequence and type of active joints in a cohort of newly diagnosed juvenile idiopathic arthritis (JIA) patients with full access to current treatment at first visit and during a follow-up period of 5-years, in order to identify an index joint/group of joints for magnetic resonance imaging in JIA. Patient charts of all consecutive newly diagnosed JIA patients with a follow-up duration of at least 5 years were analyzed. Patients were derived from two tertiary pediatric rheumatology centers. Patient characteristics and data concerning the presence of joints with arthritis and the use of medication were recorded. Findings from 95 JIA patients [39 (41 %) oligoarticular and 56 (59 %) polyarticular] were analyzed. At first visit, distribution of active joints among patients was as follows: knee (n = 70, 74 %), ankle (n = 55, 58 %), elbow (n = 23, 24 %), wrist (n = 23, 24 %), metacarpophalangeal (MCP) (n = 20, 21 %), proximal interphalangeal (PIP) (n = 13, 14 %), hip (n = 6, 6 %), shoulder (n = 5, 5 %), and distal interphalangeal (DIP) (n = 4, 4 %) joints. After a follow-up period of 5 years, the cumulative percentage of patients with specific joint involvement changed into: knee (n = 88, 93 %), ankle (n = 79, 83 %), elbow (n = 43, 45 %), wrist (n = 38, 40 %), MCP (n = 36, 38 %), PIP (n = 29, 31 %), shoulder (n = 20, 21 %), hip (n = 17, 19 %), and DIP (n = 9, 10 %) joints. Despite changes in treatment strategies over the years, the knee remains the most commonly involved joint at onset and during follow-up in JIA, followed by the ankle, elbow, and wrist. For the evaluation of outcome with MRI, the knee appears the most appropriate joint in JIA.

  11. Development of a Vision Related Quality of Life Instrument for Children 8–18 Years of Age for use in Juvenile Idiopathic Arthritis-associated Uveitis

    PubMed Central

    Angeles-Han, Sheila T; Griffin, Kenneth W; Harrison, Melanie J; Lehman, Thomas JA; Leong, Traci; Robb, Rachel Reeves; Shainberg, Marla; Ponder, Lori; Lenhart, Phoebe; Hutchinson, Amy; Srivastava, Sunil K; Prahalad, Sampath; Lambert, Scott R; Drews-Botsch, Carolyn

    2011-01-01

    Objective To determine the validity and reliability of a novel questionnaire to measure vision related quality of life (VRQOL) in children 8–18 years old for use in juvenile idiopathic arthritis-associated uveitis (JIA-U) –Effects of Youngsters’ Eyesight on Quality of Life (EYE-Q). Methods Several steps validated the EYE-Q. We interviewed experts and children on how vision affects a child’s activities. We developed new items and selected relevant items from existing instruments. We administered initial versions of the EYE-Q to normal-sighted children and those with JIA-U. For this study, children with various (or no) ocular conditions were recruited from a clinical population. Visual acuity (VA) and contrast sensitivity were performed, and the EYE-Q and Pediatric Quality of Life Inventory (PedsQL) were administered. The EYE-Q was repeated 10 days later. Patients, parents and physicians rated vision severity. Results Of 120 patients, 48% were female, 46.7% had no visual impairment (VI), and 52% had bilateral eye involvement. Mean age was 11.3 years. There were significant differences in the measures based on VA (p<0.001). Children with more severe VA and bilateral eye involvement had worse EYE-Q scores (p<0.001). There were significant associations between the EYE-Q and PedsQL (r = 0.375), repeat EYE-Q (r = 0.864), and clinical measures of ocular disease (r = −0.620). Conclusions Our study provides evidence of the validity and reliability of the EYE-Q in the measurement of VRQOL. The EYE-Q may complement clinical measures of VI and overall QOL and become an important tool in the assessment of QOL in JIA-U. PMID:21678564

  12. Current Status of Efforts on Standardizing Magnetic Resonance Imaging of Juvenile Idiopathic Arthritis: Report from the OMERACT MRI in JIA Working Group and Health-e-Child.

    PubMed

    Nusman, Charlotte M; Ording Muller, Lil-Sofie; Hemke, Robert; Doria, Andrea S; Avenarius, Derk; Tzaribachev, Nikolay; Malattia, Clara; van Rossum, Marion A J; Maas, Mario; Rosendahl, Karen

    2016-01-01

    To report on the progress of an ongoing research collaboration on magnetic resonance imaging (MRI) in juvenile idiopathic arthritis (JIA) and describe the proceedings of a meeting, held prior to Outcome Measures in Rheumatology (OMERACT) 12, bringing together the OMERACT MRI in JIA working group and the Health-e-Child radiology group. The goal of the meeting was to establish agreement on scoring definitions, locations, and scales for the assessment of MRI of patients with JIA for both large and small joints. The collaborative work process included premeeting surveys, presentations, group discussions, consensus on scoring methods, pilot scoring, conjoint review, and discussion of a future research agenda. The meeting resulted in preliminary statements on the MR imaging protocol of the JIA knee and wrist and determination of the starting point for development of MRI scoring systems based on previous studies. It was also considered important to be descriptive rather than explanatory in the assessment of MRI in JIA (e.g., "thickening" instead of "hypertrophy"). Further, the group agreed that well-designed calibration sessions were warranted before any future scoring exercises were conducted. The combined efforts of the OMERACT MRI in JIA working group and Health-e-Child included the assessment of currently available material in the literature and determination of the basis from which to start the development of MRI scoring systems for both the knee and wrist. The future research agenda for the knee and wrist will include establishment of MRI scoring systems, an atlas of MR imaging in healthy children, and MRI protocol requisites.

  13. Individual Trabeculae Segmentation (ITS)–Based Morphological Analysis of High-Resolution Peripheral Quantitative Computed Tomography Images Detects Abnormal Trabecular Plate and Rod Microarchitecture in Premenopausal Women With Idiopathic Osteoporosis

    PubMed Central

    Liu, X Sherry; Cohen, Adi; Shane, Elizabeth; Stein, Emily; Rogers, Halley; Kokolus, Shannon L; Yin, Perry T; McMahon, Donald J; Lappe, Joan M; Recker, Robert R; Guo, X Edward

    2010-01-01

    Idiopathic osteoporosis (IOP) in premenopausal women is a poorly understood entity in which otherwise healthy women have low-trauma fracture or very low bone mineral density (BMD). In this study, we applied individual trabeculae segmentation (ITS)–based morphological analysis to high-resolution peripheral quantitative computed tomography (HR-pQCT) images of the distal radius and distal tibia to gain greater insight into skeletal microarchitecture in premenopausal women with IOP. HR-pQCT scans were performed for 26 normal control individuals and 31 women with IOP. A cubic subvolume was extracted from the trabecular bone compartment and subjected to ITS-based analysis. Three Young's moduli and three shear moduli were calculated by micro–finite element (µFE) analysis. ITS-based morphological analysis of HR-pQCT images detected significantly decreased trabecular plate and rod bone volume fraction and number, decreased axial bone volume fraction in the longitudinal axis, increased rod length, and decreased rod-to-rod, plate-to-rod, and plate-to-plate junction densities at the distal radius and distal tibia in women with IOP. However, trabecular plate and rod thickness did not differ. A more rod-like trabecular microstructure was found in the distal radius, but not in the distal tibia. Most ITS measurements contributed significantly to the elastic moduli of trabecular bone independent of bone volume fraction (BV/TV). At a fixed BV/TV, plate-like trabeculae contributed positively to the mechanical properties of trabecular bone. The results suggest that ITS-based morphological analysis of HR-pQCT images is a sensitive and promising clinical tool for the investigation of trabecular bone microstructure in human studies of osteoporosis. © 2010 American Society for Bone and Mineral Research. PMID:20200967

  14. Is hydrotherapy cost-effective? A randomised controlled trial of combined hydrotherapy programmes compared with physiotherapy land techniques in children with juvenile idiopathic arthritis.

    PubMed

    Epps, H; Ginnelly, L; Utley, M; Southwood, T; Gallivan, S; Sculpher, M; Woo, P

    2005-10-01

    To compare the effects of combined hydrotherapy and land-based physiotherapy (combined) with land-based physiotherapy only (land) on cost, health-related quality of life (HRQoL) and outcome of disease in children with juvenile idiopathic arthritis (JIA). Also to determine the cost-effectiveness of combined hydrotherapy and land-based physiotherapy in JIA. A multicentre randomised controlled, partially blinded trial was designed with 100 patients in a control arm receiving land-based physiotherapy only (land group) and 100 patients in an intervention arm receiving a combination of hydrotherapy and land-based physiotherapy (combined group). Three tertiary centres in the UK. Patients aged 4-19 years diagnosed more than 3 months with idiopathic arthritides, onset before their 16th birthday, stable on medication with at least one active joint. Patients in the combined and land groups received 16 1-hour treatment sessions over 2 weeks followed by local physiotherapy attendances for 2 months. Disease improvement defined as a decrease of > or =30% in any three of six core set variables without there being a 30% increase in more than one of the remaining three variables was used as the primary outcome measure and assessed at 2 months following completion of intervention. Health services resource use (in- and outpatient care, GP visits, drugs, interventions, and investigations) and productivity costs (parents' time away from paid work) were collected at 6 months follow-up. HRQoL was measured at baseline and 2 and 6 months following intervention using the EQ-5D, and quality-adjusted life-years (QALYs) were calculated. Secondary outcome measures at 2 and 6 months included cardiovascular fitness, pain, isometric muscle strength and patient satisfaction. Seventy-eight patients were recruited into the trial and received treatment. Two months after intervention 47% patients in the combined group and 61% patients in the land group had improved disease with 11 and 5% with worsened

  15. Health related quality of life and parental perceptions of child vulnerability among parents of a child with juvenile idiopathic arthritis: results from a web-based survey

    PubMed Central

    2014-01-01

    Background A chronic illness, such as Juvenile Idiopathic Arthritis (JIA), has an impact on the whole family, especially on parents caring for the ill child. Therefore the aim of this study is to evaluate parental Health Related Quality of Life (HRQOL) and parental perceptions of child vulnerability (PPCV) and associated variables in parents of a child with JIA. Methods Parents of all JIA patients (0–18 years) in Amsterdam, the Netherlands, were eligible. HRQOL was measured using the TNO-AZL Questionnaire (TAAQOL) and PPCV using the Child Vulnerability Scale (CVS). The HRQOL of parents of a child with JIA was compared to a norm population, and differences between parents of a child with JIA and active arthritis versus parents of a child with JIA without active arthritis were analyzed (ANOVA). For PPCV, parents of a child with JIA were compared to a norm population, including healthy and chronically ill children (Chi2, Mann-Whitney U test). Variables associated with PPCV were identified by logistic regression analyses. Results 155 parents (87.5% mothers) completed online questionnaires. JIA parents showed worse HRQOL than parents of healthy children on one out of twelve domains: fine motor HRQOL (p < .001). Parents of children with active arthritis showed worse HRQOL regarding daily activities (p < .05), cognitive functioning (p < .01) and depressive emotions (p < .05) compared to parents of children without active arthritis. Parents of children with JIA perceived their child as more vulnerable than parents of a healthy child (p < .001) and parents of a chronically ill child (p < .001). Parents of children with active arthritis reported higher levels of PPCV (p < .05) than parents of children without active arthritis. A higher degree of functional disability (p < .01) and shorter disease duration (p < .05) were associated with higher levels of PPCV. Conclusion The HRQOL of JIA parents was comparable to the HRQOL of parents of a

  16. Subcutaneous golimumab for children with active polyarticular-course juvenile idiopathic arthritis: results of a multicentre, double-blind, randomised-withdrawal trial.

    PubMed

    Brunner, Hermine I; Ruperto, Nicolino; Tzaribachev, Nikolay; Horneff, Gerd; Chasnyk, Vyacheslav G; Panaviene, Violeta; Abud-Mendoza, Carlos; Reiff, Andreas; Alexeeva, Ekaterina; Rubio-Pérez, Nadina; Keltsev, Vladimir; Kingsbury, Daniel J; Del Rocio Maldonado Velázquez, Maria; Nikishina, Irina; Silverman, Earl D; Joos, Rik; Smolewska, Elzbieta; Bandeira, Márcia; Minden, Kirsten; van Royen-Kerkhof, Annet; Emminger, Wolfgang; Foeldvari, Ivan; Lauwerys, Bernard R; Sztajnbok, Flavio; Gilmer, Keith E; Xu, Zhenhua; Leu, Jocelyn H; Kim, Lilianne; Lamberth, Sarah L; Loza, Matthew J; Lovell, Daniel J; Martini, Alberto

    2017-05-15

    This report aims to determine the safety, pharmacokinetics (PK) and efficacy of subcutaneous golimumab in active polyarticular-course juvenile idiopathic arthritis (polyJIA). In this three-part randomised double-blinded placebo-controlled withdrawal trial, all patients received open-label golimumab (30 mg/m(2) of body surface area; maximum: 50 mg/dose) every 4 weeks together with weekly methotrexate during Part 1 (weeks 0-16). Patients with at least 30% improvement per American College of Rheumatology Criteria for JIA (JIA ACR30) in Part 1 entered the double-blinded Part 2 (weeks 16-48) after 1:1 randomisation to continue golimumab or start placebo. In Part 3, golimumab was continued or could be restarted as in Part 1. The primary outcome was JIA flares in Part 2; secondary outcomes included JIA ACR50/70/90 responses, clinical remission, PK and safety. Among 173 patients with polyJIA enrolled, 89.0% (154/173) had a JIA ACR30 response and 79.2%/65.9%/36.4% demonstrated JIA ACR50/70/90 responses in Part 1. At week 48, the primary endpoint was not met as treatment groups had comparable JIA flare rates (golimumab vs placebo: 32/78=41% vs 36/76=47%; p=0.41), and rates of clinical remission were comparable (golimumab vs placebo: 10/78=12.8% vs 9/76=11.8%). Adverse event and serious adverse event rates were similar in the treatment groups during Part 2. Injection site reactions occurred with <1% of all injections. PK analysis confirmed adequate golimumab dosing for polyJIA. Although the primary endpoint was not met, golimumab resulted in rapid, clinically meaningful, improvement in children with active polyJIA. Golimumab was well tolerated, and no unexpected safety events occurred. NCT01230827; Results. © 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.

  17. Efficacy and safety of tocilizumab in patients with polyarticular-course juvenile idiopathic arthritis: results from a phase 3, randomised, double-blind withdrawal trial.

    PubMed

    Brunner, Hermine I; Ruperto, Nicolino; Zuber, Zbigniew; Keane, Caroline; Harari, Olivier; Kenwright, Andrew; Lu, Peng; Cuttica, Ruben; Keltsev, Vladimir; Xavier, Ricardo M; Calvo, Inmaculada; Nikishina, Irina; Rubio-Pérez, Nadina; Alexeeva, Ekaterina; Chasnyk, Vyacheslav; Horneff, Gerd; Opoka-Winiarska, Violetta; Quartier, Pierre; Silva, Clovis A; Silverman, Earl; Spindler, Alberto; Baildam, Eileen; Gámir, M Luz; Martin, Alan; Rietschel, Christoph; Siri, Daniel; Smolewska, Elzbieta; Lovell, Daniel; Martini, Alberto; De Benedetti, Fabrizio

    2015-06-01

    To evaluate the interleukin-6 receptor inhibitor tocilizumab for the treatment of patients with polyarticular-course juvenile idiopathic arthritis (pcJIA). This three-part, randomised, placebo-controlled, double-blind withdrawal study (NCT00988221) included patients who had active pcJIA for ≥6 months and inadequate responses to methotrexate. During part 1, patients received open-label tocilizumab every 4 weeks (8 or 10 mg/kg for body weight (BW) <30 kg; 8 mg/kg for BW ≥30 kg). At week 16, patients with ≥JIA-American College of Rheumatology (ACR) 30 improvement entered the 24-week, double-blind part 2 after randomisation 1:1 to placebo or tocilizumab (stratified by methotrexate and steroid background therapy) for evaluation of the primary end point: JIA flare, compared with week 16. Patients flaring or completing part 2 received open-label tocilizumab. In part 1, 188 patients received tocilizumab (<30 kg: 10 mg/kg (n=35) or 8 mg/kg (n=34); ≥30 kg: n=119). In part 2, 163 patients received tocilizumab (n=82) or placebo (n=81). JIA flare occurred in 48.1% of patients on placebo versus 25.6% continuing tocilizumab (difference in means adjusted for stratification: -0.21; 95% CI -0.35 to -0.08; p=0.0024). At the end of part 2, 64.6% and 45.1% of patients receiving tocilizumab had JIA-ACR70 and JIA-ACR90 responses, respectively. Rates/100 patient-years (PY) of adverse events (AEs) and serious AEs (SAEs) were 480 and 12.5, respectively; infections were the most common SAE (4.9/100 PY). Tocilizumab treatment results in significant improvement, maintained over time, of pcJIA signs and symptoms and has a safety profile consistent with that for adults with rheumatoid arthritis. NCT00988221. 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.

  18. Efficacy and safety of tocilizumab in patients with polyarticular-course juvenile idiopathic arthritis: results from a phase 3, randomised, double-blind withdrawal trial

    PubMed Central

    Brunner, Hermine I; Ruperto, Nicolino; Zuber, Zbigniew; Keane, Caroline; Harari, Olivier; Kenwright, Andrew; Lu, Peng; Cuttica, Ruben; Keltsev, Vladimir; Xavier, Ricardo M; Calvo, Inmaculada; Nikishina, Irina; Rubio-Pérez, Nadina; Alexeeva, Ekaterina; Chasnyk, Vyacheslav; Horneff, Gerd; Opoka-Winiarska, Violetta; Quartier, Pierre; Silva, Clovis A; Silverman, Earl; Spindler, Alberto; Baildam, Eileen; Gámir, M Luz; Martin, Alan; Rietschel, Christoph; Siri, Daniel; Smolewska, Elzbieta; Lovell, Daniel; Martini, Alberto; De Benedetti, Fabrizio

    2015-01-01

    Objective To evaluate the interleukin-6 receptor inhibitor tocilizumab for the treatment of patients with polyarticular-course juvenile idiopathic arthritis (pcJIA). Methods This three-part, randomised, placebo-controlled, double-blind withdrawal study (NCT00988221) included patients who had active pcJIA for ≥6 months and inadequate responses to methotrexate. During part 1, patients received open-label tocilizumab every 4 weeks (8 or 10 mg/kg for body weight (BW) <30 kg; 8 mg/kg for BW ≥30 kg). At week 16, patients with ≥JIA-American College of Rheumatology (ACR) 30 improvement entered the 24-week, double-blind part 2 after randomisation 1:1 to placebo or tocilizumab (stratified by methotrexate and steroid background therapy) for evaluation of the primary end point: JIA flare, compared with week 16. Patients flaring or completing part 2 received open-label tocilizumab. Results In part 1, 188 patients received tocilizumab (<30 kg: 10 mg/kg (n=35) or 8 mg/kg (n=34); ≥30 kg: n=119). In part 2, 163 patients received tocilizumab (n=82) or placebo (n=81). JIA flare occurred in 48.1% of patients on placebo versus 25.6% continuing tocilizumab (difference in means adjusted for stratification: −0.21; 95% CI −0.35 to −0.08; p=0.0024). At the end of part 2, 64.6% and 45.1% of patients receiving tocilizumab had JIA-ACR70 and JIA-ACR90 responses, respectively. Rates/100 patient-years (PY) of adverse events (AEs) and serious AEs (SAEs) were 480 and 12.5, respectively; infections were the most common SAE (4.9/100 PY). Conclusions Tocilizumab treatment results in significant improvement, maintained over time, of pcJIA signs and symptoms and has a safety profile consistent with that for adults with rheumatoid arthritis. Trial registration number: NCT00988221. PMID:24834925

  19. Development and preliminary validation of the 'Mind the Gap' scale to assess satisfaction with transitional health care among adolescents with juvenile idiopathic arthritis.

    PubMed

    Shaw, K L; Southwood, T R; McDonagh, J E

    2007-07-01

    To develop a scale to assess satisfaction with transitional health care among adolescents with a chronic illness and their parents. The 'Mind the Gap' scale was developed using evidence from a previous needs assessment, in three stages: (1) definition of the construct; (2) design of the scale items, response options and instructions; (3) full administration of the scale, item analysis and dimensionality analysis. The scale was administered to 308 adolescents with juvenile idiopathic arthritis (JIA) and 303 parents/guardians, prior to and 12 months after the implementation of an evaluation of a structured and co-ordinated programme of transitional care. The patient population involved adolescents with JIA and their parents recruited from 10 major UK rheumatology centres. A total of 301 (97.7%) adolescents and 286 (95.0%) parents chose to complete the questionnaire, with median item completion rates of 100.0% (0-100%) for both adolescents and parents thus confirming feasibility. Face and content validity were confirmed. Factor analyses revealed a three-factor structure which explained 49.5% and 56.1% of the variation in adolescent and parent scores respectively. The internal consistency of each subscale ('management of environment', 'provider characteristics' and 'process issues') was indicated by Cronbach's alphas of 0.71, 0.89 and 0.89 for adolescents, respectively, and 0.83, 0.91 and 0.92 for parents respectively. Cronbach's alphas for the entire scales were 0.91 and 0.94 for the adolescent and parent forms respectively. These preliminary results report the potential of the 'Mind the Gap' scale in evaluating transitional care for adolescents with JIA. In view of the generic nature of transitional care reflected in the scale, this scale has wider potential for use with adolescents with other chronic illness in view of the generic nature of transition. This development is particularly timely in the context of transitional care developments in the UK and further

  20. Intra-articular corticosteroid injections to the temporomandibular joints are safe and appear to be effective therapy in children with juvenile idiopathic arthritis.

    PubMed

    Stoll, Matthew L; Good, Jennifer; Sharpe, Tyler; Beukelman, Timothy; Young, Daniel; Waite, Peter D; Cron, Randy Q

    2012-08-01

    The purpose of this study was to evaluate the safety and efficacy of intra-articular corticosteroid injections (IACIs) of the temporomandibular joint (TMJ) in children with juvenile idiopathic arthritis (JIA) when administered by an oral and maxillofacial surgeon without imaging guidance. This was a retrospective study of children with JIA, seen at a single center, who were selected based on having received IACIs of the TMJ. All subjects received the intervention, which consisted of referral to a single oral and maxillofacial surgeon for TMJ IACI with 5 to 10 mg triamcinolone hexacetonide, under general anesthesia. Primary outcomes assessed in all subjects were the safety of the procedure and efficacy as determined by the change in maximal incisal opening (MIO). In addition, a subset of 31 subjects underwent repeat magnetic resonance imaging of the TMJ, permitting analysis of the change in the acute and chronic findings of arthritis in those patients. Sixty-three patients (68% female) received 137 IACIs. The mean age for diagnosis of JIA was 8.5 years, and the mean age at presentation for TMJ injections was 10 years. The injections were well tolerated: only 1 patient developed the steroid complication of hypopigmentation, and none developed degeneration or ankylosis. In terms of efficacy, the mean MIO increased from 40.8 ± 0.93 to 43.5 ± 0.90 mm (P = .001); in addition, changing the unit of analysis to individual joints, in patients who underwent repeat magnetic resonance imaging examination, 51% of TMJs showed magnetic resonance imaging evidence of improvement of arthritic changes, of whom 18% had complete resolution of TMJ arthritis. The results indicate that IACI of the TMJ can be safely performed by experienced oral and maxillofacial surgeons without a requirement for computed tomographic guidance. In addition, these results show that IACI may be effective in the management of TMJ arthritis, although further studies are required. Copyright © 2012 American

  1. Can Seeding in the Clinic Reach a Wide Audience? A Proof of Concept Study on Spreading a Health Message About Juvenile Idiopathic Arthritis Using a Shareable Online Video

    PubMed Central

    Fay, Michaela; Rapley, Tim; Foster, Helen; Pain, Clare

    2016-01-01

    Background Shareable online video offers the potential for spreading a health message across online and real world social networks. Seeding a message in a clinical setting may be advantageous. Objective To investigate the potential of an online video to spread a health message about juvenile idiopathic arthritis (JIA) when delivered or seeded in a clinical setting and investigate factors that influence sharing behavior. Methods Multimethod proof of concept study. Concepts for two different styles of video were developed using focus groups and interviews and reviewed by an online market research panel. We compared dissemination of the two videos from two specialist pediatric rheumatology clinics in NHS Hospitals. Participants were 15 patients, family members, and clinical staff with knowledge of JIA at concept stage; 300 market research panel members in development stage; and 38 patients and their parents or guardians in the seeding stage. Newly diagnosed patients with JIA and/or parents or guardians were invited to view and share an online video with a health message about JIA across real-life and electronic social networks. Main outcome measures were viewing statistics, sharing behavior and patterns, and participant feedback. Results Of 38 patients and/or their parents or guardians given links, 26 visited the video webpage and shared the link, 2 visited and did not share, and 10 did not visit. Most links were viewed and shared within a few days. A total of 3314 pageviews were recorded with a mean of 89.6 pageviews per link (range 0-1245). Links were accessed from 26 countries, with most viewers in the United Kingdom (82.5%). Mothers were the most active group of sharers. Conclusions Distribution of a video link in a clinical setting may be an effective way to spread a health message. Parents or guardians of children with JIA are more likely to share a link than young people. Dissemination depends on a small number of active sharers, the content of the video, and

  2. Pathophysiology of juvenile idiopathic arthritis induced pes planovalgus in static and walking condition: a functional view using 3D gait analysis.

    PubMed

    Merker, Josephine; Hartmann, Matthias; Kreuzpointner, Florian; Schwirtz, Ansgar; Haas, Johannes-Peter

    2015-06-10

    Patients suffering from juvenile idiopathic arthritis (JIA) frequently have affected ankle joints, which can lead to foot deformities such as pes planovalgus (JIA-PPV). Usually, JIA-PPV is diagnosed by examining the foot in non-weightbearing or in weightbearing, static condition. However, functional limitations typically appear during dynamic use in daily activities such as walking. The aim of this study was to quantify the pathophysiology of JIA-PPV in both static and dynamic condition, i.e. in upright standing and during the stance phase of walking using three-dimensional (3d) gait analysis. Eleven JIA patients (age = 12y) with at least one affected ankle joint and fixed pes planovalgus (≥5°) were compared to healthy controls (CG) (n = 14, age = 11y). Kinematic and kinetic data were obtained in barefoot standing and walking condition (1.1-1.3 m/s) with an 8-camera 3d motion analysis system including two force-plates and one pressure distribution plate. All participants were prepared using reflecting markers according to the Oxford Foot and Plug-in-Gait Model. Results were compared using the Mann-Whitney-U-test and Wilcoxon signed-rank test (p < 0.05). In comparison to CG, JIA-PPV had an excessive hindfoot/tibia eversion (p < 0.001) and a forefoot/hindfoot supination (p < 0.001) in both static and walking condition. JIA-PPV showed a greater hindfoot/tibia eversion during walking (midstance) compared to standing (p = 0.021) in contrast to CG. The arch index, measured by plantar pressure distribution, indicates a reduced arch height in JIA-PPV (p = 0.007). Patients had a lower maximum dorsiflexion of hindfoot/tibia (p = 0.001) and a lower plantarflexion of forefoot/hindfoot (p = 0.028), both when standing and walking. The kinetic results showed lower maximum ankle dorsiflexion moments (p < 0.037) as well as generated ankle power (p = 0.086) in JIA-PPV. The pathophysiology of JIA-PPV during walking indicated that

  3. 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.

  4. Health-related quality of life in children with juvenile idiopathic arthritis – child’s and parent’s point of view

    PubMed Central

    Rutkowska-Sak, Lidia; Raciborski, Filip

    2016-01-01

    Objectives To assess the quality of life (QoL) of children suffering from juvenile idiopathic arthritis (JIA) in Poland, to compare QoL of children with JIA and healthy children, and to compare children’s and parents’ assessments of QoL. Material and methods The KIDSCREEN-52 questionnaire (children’s and parents’ version) was used to assess the quality of life. The QoL in JIA patients and healthy peers from European and Polish reference groups was compared by the t-test. The Bland-Altman method was used to evaluate child and parent assessment agreement. Results Eighty-nine questionnaires were obtained from children (median age: 14 years; 62% female; JIA history longer than 1 year) and 84 questionnaires from parents. The QoL of JIA patients was lower than in healthy peers from the European reference group in terms of physical well-being (p < 0.001), psychological well-being (p = 0.011), autonomy (p < 0.001) and social support and peers (p < 0.001). The QoL of JIA patients compared with the QoL of children from the Polish reference group was lower only in terms of physical well-being (p < 0.001), whereas it was higher in terms of moods and emotions (p = 0.023), parent relations and home life (p = 0.005) and financial resources (p < 0.001). In most terms the assessment performed by the parent was lower than the child’s. The most significant differences were observed for physical well-being (p < 0.001), psychological well-being (p = 0.016), and self-perception (p = 0.013). Conclusions The present study is the first assessment of QoL of JIA children in Poland. In our study the quality of life in JIA children was lower than in healthy peers. Discrepancies between the assessment of the child’s QoL performed by the child and the parent were found. Both assessments should be taken into account in clinical practice as well as in research studies. PMID:27994269

  5. A Systematic Critical Appraisal of Clinical Practice Guidelines in Juvenile Idiopathic Arthritis Using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) Instrument

    PubMed Central

    Smith, Christine A. M.; Toupin-April, Karine; Jutai, Jeffrey W.; Duffy, Ciarán M.; Rahman, Prinon; Cavallo, Sabrina; Brosseau, Lucie

    2015-01-01

    Objectives The objectives of this review are to: 1) appraise the methodological quality of clinical practice guidelines (CPGs) in juvenile idiopathic arthritis (JIA) providing pharmacological and/or non-pharmacological intervention recommendations, and 2) summarize the recommendations provided by the included CPGs and compare them where possible. Methods A systematic search was performed. Three trained appraisers independently evaluated the methodological quality of the CPGs using a validated and reliable instrument, the Appraisal of Guidelines in Research and Evaluation II. Six domains were considered: 1) score and purpose; 2) stakeholder involvement; 3) rigor of development; 4) clarity of presentation; 5) applicability; and 6) editorial independence. The domains consist of a total of 23 items each scored on a 7-point scale. High quality CPGs were identified if they had a domain score above 60% in rigor of development, and two other domains. Results Of the three included CPGs, the Royal Australian College of General Practitioners (RACGP) and American College of Rheumatology (ACR) CPGs were considered to be of high quality, but the German Society for Pediatric Rheumatology was of lower quality. Domains one to four had high domain scores across the guidelines (mean (standard deviation)): 72.76 (13.80); 66.67 (9.81); 64.67 (7.77); and 87.00 (9.64), respectively. Lower scores were obtained for applicability (14.00 (5.57)) and editorial independence (43.44 (7.02)). Recommendations varied across CPGs due to differences in context, target audience (general practitioners, rheumatologists, and other multidisciplinary healthcare professionals) and patients’ disease presentations. Despite this variability, progression of pharmacological treatment did not conflict between CPGs. Recommendations for non-pharmacological interventions were vague and the interventions considered varied between CPGs. Conclusions Overall, recommendations were based on a paucity of evidence and

  6. Analysis of intracellular methotrexate polyglutamates in patients with juvenile idiopathic arthritis: effect of route of administration on variability in intracellular methotrexate polyglutamate concentrations.

    PubMed

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

    2010-06-01

    Intracellular methotrexate (MTX) polyglutamates (MTXGlu) have been shown to be potentially useful biomarkers of clinical response in adult patients with rheumatoid arthritis. The present study was undertaken to measure intracellular MTXGlu concentrations in a cohort of patients with juvenile idiopathic arthritis (JIA) to determine the predictors of MTXGlu variability in these patients. Blood samples were obtained from patients with JIA who were being treated with a stable dose of MTX for >or=3 months. Clinical data were collected by chart review. Concentrations of MTXGlu(1-7) in red blood cell lysates were quantitated using an innovative ion-pairing chromatography procedure, with detection by mass spectrometry. Patients with JIA from a single center (n = 99; mean +/- SD age 117.8 +/- 56.5 months, 69 female) were included in the analysis. The mean +/- SD dose of MTX was 0.51 +/- 0.25 mg/kg per week, with a median treatment duration of 18 months (interquartile range 3-156 months). MTX was administered subcutaneously in 66 patients (67%). Fifty-six patients (57%) had active arthritis at the time of the clinic visit. Total intracellular MTXGlu (MTXGlu(TOT)) concentrations varied 40-fold, with a mean +/- SD total concentration of 85.8 +/- 48.4 nmoles/liter. Concentrations of each MTXGlu subtype (MTXGlu(1-7)) were measured individually and as a percentage of MTXGlu(TOT) in each patient. MTXGlu(3) was the most prominent subtype identified, comprising 42% of MTXGlu(TOT), and the interindividual variability in the concentration of MTXGlu(3) was the most highly correlated with that of MTXGlu(TOT) (r = 0.96). The route of MTX administration was significantly associated with MTXGlu(1-5) subtypes; higher concentrations of MTXGlu(1 + 2) were observed in patients receiving oral doses of MTX, whereas higher concentrations of MTXGlu(3-5) were observed in patients receiving subcutaneous doses of MTX (P < 0.0001). In this cohort of patients with JIA, the MTXGlu(TOT) concentration

  7. Socioeconomic costs and health-related quality of life in juvenile idiopathic arthritis: a cost-of-illness study in the United Kingdom.

    PubMed

    Angelis, Aris; Kanavos, Panos; López-Bastida, Julio; Linertová, Renata; Serrano-Aguilar, Pedro

    2016-08-02

    Juvenile idiopathic arthritis (JIA) refers to a number of rare chronic inflammatory diseases. Although JIA imposes a significant societal burden, limited data are available on the cost of JIA. The study's objective is to quantify the socioeconomic burden of JIA patients in the United Kingdom (UK), along with their health-related quality of life (HRQoL). A bottom-up, cross-sectional, cost-of-illness analysis of 23 patients was carried out. To collect data on demographic characteristics, health resource utilization, informal care, productivity losses and HRQoL, questionnaires were administered to and completed by patients or their caregivers. The EuroQol five dimensions (EQ-5D) instrument was used to measure HRQoL. This study found that the average annual cost for a JIA patient was €31,546, with direct health care costs equalling €14,509 (46.0 % of total costs), direct non-health care costs amounting to €8,323 (26.4 %) and productivity losses being €8,715 (27.6 %). This was calculated using unit costs for 2012. The largest expenditures on average were accounted for by early retirement (27.0 %), followed by informal care (24.1 %), medications (21.1 %), outpatient and primary health care visits (13.2 %) and diagnostic tests (7.9 %). Important differences existed between JIA patients in need of caregiver assistance and those with no need (€39,469 vs. €25,452 respectively). Among adult JIA patients, mean EQ-5D index scores and visual analogue scale (VAS) scores were found to be 0.26 and 49.00 respectively; the same scores among caregivers were 0.66 and 67.14 respectively. JIA poses a significant cost burden on the UK society. Over half of the total average costs (54 %) are related to non-health care and productivity losses. HRQoL of JIA patients is considerably worse than the UK general population.

  8. Effects of acute exercise on circulating endothelial and progenitor cells in children and adolescents with juvenile idiopathic arthritis and healthy controls: a pilot study.

    PubMed

    Obeid, Joyce; Nguyen, Thanh; Cellucci, Tania; Larché, Maggie J; Timmons, Brian W

    2015-10-12

    Youth with juvenile idiopathic arthritis (JIA) may be at risk of poor cardiovascular health. Circulating endothelial progenitor cells (EPCs) and circulating endothelial cells (CECs) are markers of cardiovascular repair and damage, respectively, and respond to exercise. The objectives of this study were to compare resting levels of EPCs and CECs in JIA and controls, and to assess the effects of distinct types of exercise on EPCs and CECs in JIA and controls. Seven youth with JIA and six controls completed 3 visits. First, aerobic fitness was assessed. Participants then performed either moderate intensity, continuous exercise (MICE) or high intensity, intermittent exercise (HIIE) on separate days. Blood samples were collected at the beginning (REST), mid-point (MID) and end of exercise (POST) for determination of EPCs (CD31(+)CD34(bright)CD45(dim)CD133(+)) and CECs (CD31(bright)CD34(+)CD45(-)CD133(-)) by flow cytometry. Between group differences in EPCs and CECs were examined using two-way ANOVA, followed by Tukey's HSD post hoc, where appropriate. Statistical significance set at p ≤ 0.05. Both EPCs and CECs were similar between groups at REST (p = 0.18-0.94). During MICE, EPCs remained unchanged in JIA (p = 0.95) but increased significantly at POST in controls (REST: 0.91 ± 0.55 × 10(6) cells/L vs. POST: 1.53 ± 0.36 × 10(6) cells/L, p = 0.04). Compared with controls, lower levels of EPCs were observed in JIA at MID (0.48 ± 0.50 × 10(6) cells/L vs. 1.10 ± 0.39 × 10(6) cells/L, p = 0.01) and POST (0.38 ± 0.34 × 10(6) cells/L vs. 1.53 ± 0.36 × 10(6) cells/L, p < 0.001) during MICE. No changes were detected in CECs with MICE in JIA and controls (p = 0.69). Neither EPCs nor CECs were modified with HIIE (p = 0.28-0.69). Youth with JIA demonstrated a blunted EPC response to MICE when compared with controls. Future work should examine factors that may increase or normalize EPC

  9. Muscle strength, physical fitness and well-being in children and adolescents with juvenile idiopathic arthritis and the effect of an exercise programme: a randomized controlled trial

    PubMed Central

    2013-01-01

    Background Decreased muscle strength, fitness and well-being are common in children and adolescents with juvenile idiopathic arthritis (JIA) compared to healthy peers. Biological drugs have improved health in children with JIA, but despite this pain is still a major symptom and bone health is reported as decreased in the group. The improvement made by the biological drugs makes it possible to more demanding exercises. To jump is an exercise that can improve bone heath, fitness and muscle strength. The aim of the study was to see if an exercise programme with jumps had an effect on muscle strength, physical fitness and well-being and how it was tolerated. Methods Muscle strength and well-being were studied before and after a 12-week exercise programme in 54 children and adolescents with JIA, 9–21 years old. The participants were randomized into an exercise and a control group. Muscle strength, fitness and well-being were documented before and after the training period and at follow-up after 6 months. Physical activity in leisure time was documented in diaries. The fitness/exercise programme was performed at home three times a week and included rope skipping and muscle strength training exercises. Assessment included measurement of muscle strength with a handheld device, and with Grip-it, step-test for fitness with documentation of heart rate and pain perception and two questionnaires (CHAQ, CHQ) on well-being. Results There were no differences between exercise and control group regarding muscle strength, grip strength, fitness or well-being at base line. Muscle weakness was present in hip extensors, hip abductors and handgrip. For the exercise group muscle strength in hip and knee extensors increased after the 12-week exercise programme and was maintained in knee extensors at follow-up. There was no change in fitness tested with the individually adapted step-test. The CHQ questionnaire showed that pain was common in the exercise group and in the control group

  10. Feasibility of a Website and a Hospital-Based Online Portal for Young Adults With Juvenile Idiopathic Arthritis: Views and Experiences of Patients

    PubMed Central

    Scholtus, Lieske W; Drossaert, Constance HC; van Os-Medendorp, Harmieke; Prakken, Berent; Kruize, Aike A; Bijlsma, Johannes JW

    2015-01-01

    Background To improve knowledge and to encourage active involvement of young adults with juvenile idiopathic arthritis (JIA), an informative website with written and video information and an online portal with access to the personal medical record, self-monitoring, and e-consult functionalities were developed. Before implementing these applications in daily practice, it is important to gain insight into their feasibility in terms of ease of use, perceived usefulness and intention to use. Objective The aim of this study was to evaluate and to examine the feasibility of the website and the online portal for young adults with JIA. Methods A qualitative, feasibility study was conducted among the first users: 13 young adults with JIA. After provided access to the website and online portal, patients were interviewed on perceived usefulness, ease of use, and intention to (re)use the applications. Results Participants in the study considered the website and online portal as useful and easy-to-use. New medical information and feedback would motivate them to revisit the applications again. On the website, videos showing other young adults, telling how they handle their condition, were found as the most useful. On the portal, access to their medical records was most appreciated: it made the young JIA patients feel in control and it helped them monitor symptoms and disease activity. e-consults were thought to facilitate communication with physicians. Conclusions The young adults considered both the website and the online portal as feasible, but they also had valuable suggestions to improve accessibility and use. Based on these findings, a news and event section was added on the website and a direct link was made to a discussion board and social media. To provide and support health information, the website is actively used in daily care. Considering the online portal, the use of self-monitoring tools and e-consult can be stimulated if there is direct linkage to treatment and

  11. TLR4 rs41426344 increases susceptibility of rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) in a central south Chinese Han population.

    PubMed

    Wang, Yan; Chen, Lianghui; Li, Fang; Bao, Meihua; Zeng, Jie; Xiang, Ju; Luo, Huaiqing; Li, Jianming; Tang, Liang

    2017-02-21

    The aim of the study was to determine whether polymorphisms in toll-like receptor 4 (TLR4) confer susceptibility to rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) in a central south Chinese Han population. Genotyping for six well studied polymorphisms (rs4986790, rs4986791, rs10759932, rs41426344, rs11536889 and rs7873784) in TLR4 gene were conducted in 1074 unrelated patients with RA and 1692 healthy control subjects, as well as in 217 unrelated patients with JIA and 378 healthy control subjects using direct sequencing technique. Comparisons between cases and controls in alleles, genotypes and haplotypes were carried out using Fisher's exact test. Significant genetic associations were detected between the 3'UTR rs41426344C and RA (p < 0.001, p adj < 0.001, OR = 2.24) and JIA (p < 0.001, p adj < 0.001, OR = 2.05). In addition, rs4986790G was found to be significantly associated with the susceptibility for RA (p = 0.005, p adj = 0.03, OR = 3.43), but not for JIA (p = 0.06, p adj = 0.36, OR = 2.65). Furthermore, significant increasing in the distributions of haplotypes H4 and H10 in RA (H4: p = 0.001, OR = 1.13; H10: p = 0.001, OR = 1.15) and JIA (H4: p = 0.04, OR = 2.06; H10: p = 0.02, OR = 2.47) were also found. Moreover, the frequency of rs41426344C significantly increased in RF-positive and anti-CCP positive subjects both in RA (RF(+): p <0.0001, OR = 2.33; anti-CCP(+): p =0.008, OR = 2.79) and JIA (RF(+): p =0.02, OR = 2.91; anti-CCP(+): p = 0.02, OR = 2.78). Our study suggested that rs41426344 and rs4986790 of TLR4 might contribute to RA, and rs41426344 might contribute to JIA pathogenesis in central south Chinese Han population.

  12. Health related quality of life and parental perceptions of child vulnerability among parents of a child with juvenile idiopathic arthritis: results from a web-based survey.

    PubMed

    Haverman, Lotte; van Oers, Hedy A; Maurice-Stam, Heleen; Kuijpers, Taco W; Grootenhuis, Martha A; van Rossum, Marion Aj

    2014-01-01

    A chronic illness, such as Juvenile Idiopathic Arthritis (JIA), has an impact on the whole family, especially on parents caring for the ill child. Therefore the aim of this study is to evaluate parental Health Related Quality of Life (HRQOL) and parental perceptions of child vulnerability (PPCV) and associated variables in parents of a child with JIA. Parents of all JIA patients (0-18 years) in Amsterdam, the Netherlands, were eligible. HRQOL was measured using the TNO-AZL Questionnaire (TAAQOL) and PPCV using the Child Vulnerability Scale (CVS). The HRQOL of parents of a child with JIA was compared to a norm population, and differences between parents of a child with JIA and active arthritis versus parents of a child with JIA without active arthritis were analyzed (ANOVA). For PPCV, parents of a child with JIA were compared to a norm population, including healthy and chronically ill children (Chi(2), Mann-Whitney U test). Variables associated with PPCV were identified by logistic regression analyses. 155 parents (87.5% mothers) completed online questionnaires. JIA parents showed worse HRQOL than parents of healthy children on one out of twelve domains: fine motor HRQOL (p < .001). Parents of children with active arthritis showed worse HRQOL regarding daily activities (p < .05), cognitive functioning (p < .01) and depressive emotions (p < .05) compared to parents of children without active arthritis. Parents of children with JIA perceived their child as more vulnerable than parents of a healthy child (p < .001) and parents of a chronically ill child (p < .001). Parents of children with active arthritis reported higher levels of PPCV (p < .05) than parents of children without active arthritis. A higher degree of functional disability (p < .01) and shorter disease duration (p < .05) were associated with higher levels of PPCV. The HRQOL of JIA parents was comparable to the HRQOL of parents of a healthy child. JIA parents of a child

  13. 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 ...

  14. 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 ...

  15. Transient osteoporosis of the hip.

    PubMed

    Mirza, Rabeea; Ishaq, Saliha; Amjad, Hira

    2012-02-01

    Transient Osteoporosis of Hip (TOH) is an uncommon disorder of idiopathic nature, particularly in the Asian population. It has been described to mostly occur in middle aged men and women in their third trimester of pregnancy. A distinctive hallmark of this condition is that it is self limiting and resolves in a few months. The patient presents to the physician with pain on movement and impaired mobility of the affected joint, developing without any history of trauma. MRI is the main diagnostic tool. We report herein a case of a forty five year old male, who developed transient osteoporosis of the hip, and was managed conservatively.

  16. 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

  17. Developing and Evaluating JIApp: Acceptability and Usability of a Smartphone App System to Improve Self-Management in Young People With Juvenile Idiopathic Arthritis

    PubMed Central

    Beste, Dominik; Chaplin, Hema; Varakliotis, Socrates; Suffield, Linda; Josephs, Francesca; Sen, Debajit; Wedderburn, Lucy R; Ioannou, Yiannakis; Hailes, Stephen; Eleftheriou, Despina

    2017-01-01

    Background Flare-ups in juvenile idiopathic arthritis (JIA) are characterized by joint pain and swelling and often accompanied with fatigue, negative emotions, and reduced participation in activities. To minimize the impact of JIA on the physical and psychosocial development and well-being of young people (YP), it is essential to regularly monitor disease activity and side effects, as well as to support self-management such as adherence to treatment plans and engagement in general health-promoting behaviors. Smartphone technology has the potential to engage YP with their health care through convenient self-monitoring and easy access to information. In addition, having a more accurate summary of self-reported fluctuations in symptoms, behaviors, and psychosocial problems can help both YP and health care professionals (HCPs) better understand the patient’s condition, identify barriers to self-management, and assess treatment effectiveness and additional health care needs. No comprehensive smartphone app has yet been developed in collaboration with YP with JIA, their parents, and HCPs involved in their care. Objectives The objective of this study was to design, develop, and evaluate the acceptability and usability of JIApp, a self-management smartphone app system for YP with JIA and HCPs. Methods We used a qualitative, user-centered design approach involving YP, parents, and HCPs from the rheumatology team. The study was conducted in three phases: (1) phase I focused on developing consensus on the features, content, and design of the app; (2) phase II was used for further refining and evaluating the app prototype; and (3) phase III focused on usability testing of the app. The interview transcripts were analyzed using qualitative content analysis. Results A total of 29 YP (aged 10-23, median age 17) with JIA, 7 parents, and 21 HCPs were interviewed. Major themes identified as the ones that helped inform app development in phase I were: (1) remote monitoring of

  18. Developing and Evaluating JIApp: Acceptability and Usability of a Smartphone App System to Improve Self-Management in Young People With Juvenile Idiopathic Arthritis.

    PubMed

    Cai, Ran A; Beste, Dominik; Chaplin, Hema; Varakliotis, Socrates; Suffield, Linda; Josephs, Francesca; Sen, Debajit; Wedderburn, Lucy R; Ioannou, Yiannakis; Hailes, Stephen; Eleftheriou, Despina

    2017-08-15

    Flare-ups in juvenile idiopathic arthritis (JIA) are characterized by joint pain and swelling and often accompanied with fatigue, negative emotions, and reduced participation in activities. To minimize the impact of JIA on the physical and psychosocial development and well-being of young people (YP), it is essential to regularly monitor disease activity and side effects, as well as to support self-management such as adherence to treatment plans and engagement in general health-promoting behaviors. Smartphone technology has the potential to engage YP with their health care through convenient self-monitoring and easy access to information. In addition, having a more accurate summary of self-reported fluctuations in symptoms, behaviors, and psychosocial problems can help both YP and health care professionals (HCPs) better understand the patient's condition, identify barriers to self-management, and assess treatment effectiveness and additional health care needs. No comprehensive smartphone app has yet been developed in collaboration with YP with JIA, their parents, and HCPs involved in their care. The objective of this study was to design, develop, and evaluate the acceptability and usability of JIApp, a self-management smartphone app system for YP with JIA and HCPs. We used a qualitative, user-centered design approach involving YP, parents, and HCPs from the rheumatology team. The study was conducted in three phases: (1) phase I focused on developing consensus on the features, content, and design of the app; (2) phase II was used for further refining and evaluating the app prototype; and (3) phase III focused on usability testing of the app. The interview transcripts were analyzed using qualitative content analysis. A total of 29 YP (aged 10-23, median age 17) with JIA, 7 parents, and 21 HCPs were interviewed. Major themes identified as the ones that helped inform app development in phase I were: (1) remote monitoring of symptoms, well-being, and activities; (2

  19. 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.

  20. Treating osteoporosis

    PubMed Central

    Gupta, Akhil; March, Lyn

    2016-01-01

    summary Osteoporotic fractures are common resulting in increased morbidity and mortality. Exercise can help prevent osteoporosis. It can also benefit patients with osteoporosis, but the exercises must be tailored to the patient. Most Australians should be able to obtain adequate calcium in their diet and vitamin D from the sun. Supplements may be needed in some patients and they are recommended for use with other drugs for osteoporosis. Bisphosphonates, and in some patients denosumab, are first-line drugs for osteoporosis. Raloxifene and strontium ranelate can be considered in patients who cannot take bisphosphonates or denosumab. Teriparatide is reserved for patients with severe osteoporosis and the use of strontium ranelate is declining because of cardiovascular safety concerns. PMID:27340321

  1. Secondary osteoporosis

    PubMed Central

    Sheu, Angela; Diamond, Terry

    2016-01-01

    SUMMARY Secondary osteoporosis is less common than primary osteoporosis. It may be suspected in patients who present with a fragility fracture despite having no risk factors for osteoporosis. In addition, secondary osteoporosis should be considered if the bone density Z-score is –2.5 or less. Consider the fracture site and presence of other clinical clues to guide investigations for an underlying cause. The tests to use are those that are indicated for the suspected cause. Baseline investigations include tests for bone and mineral metabolism (calcium, phosphate, alkaline phosphatase, 25-hydroxyvitamin D, parathyroid hormone), liver and kidney function, full blood count and thyroid-stimulating hormone. More detailed testing may be required in patients with severe osteoporosis. PMID:27346916

  2. Glucocorticoid-associated osteoporosis in chronic inflammatory diseases: epidemiology, mechanisms, diagnosis, and treatment.

    PubMed

    von Scheven, Emily; Corbin, Kathleen Jo; Stagi, Stefano; Stefano, Stagi; Cimaz, Rolando

    2014-09-01

    Children with chronic illnesses such as Juvenile Idiopathic Arthritis and Crohn's disease, particularly when taking glucocorticoids, are at significant risk for bone fragility. Furthermore, when childhood illness interferes with achieving normal peak bone mass, life-long fracture risk is increased. Osteopenia and osteoporosis, which is increasingly recognized in pediatric chronic disease, likely results from numerous disease- and treatment-related factors, including glucocorticoid exposure. Diagnosing osteoporosis in childhood is complicated by the limitations of current noninvasive techniques such as DXA, which despite its limitations remains the gold standard. The risk:benefit ratio of treatment is confounded by the potential for spontaneous restitution of bone mass deficits and reshaping of previously fractured vertebral bodies. Bisphosphonates have been used to treat secondary osteoporosis in children, but limited experience and potential long-term toxicity warrant caution in routine use. This article reviews the factors that influence loss of normal bone strength and evidence for effective treatments, in particular in patients with gastrointestinal and rheumatologic disorders who are receiving chronic glucocorticoid therapy.

  3. A systematic review of the effectiveness of strategies for reducing fracture risk in children with juvenile idiopathic arthritis with additional data on long-term risk of fracture and cost of disease management.

    PubMed

    Thornton, J; Ashcroft, D; O'Neill, T; Elliott, R; Adams, J; Roberts, C; Rooney, M; Symmons, D

    2008-03-01

    To review outcome measures and treatment costs in children with juvenile idiopathic arthritis (JIA) and low bone mineral density (BMD) and/or fragility fractures. To review evidence for effectiveness and safety of bisphosphonates and calcium and/or vitamin D in these children. To assess long-term bone health in adults with JIA. Major databases were searched up to July 2005 for effectiveness studies and up to January 2005 for costs. A structured search strategy was conducted. For the evaluation of long-term bone health, outcome data were derived from two cohorts of adult patients with JIA. As there were few published cost data, an ongoing UK longitudinal study (CAPS) provided background data on the cost of managing JIA. Sixteen studies (78 children with JIA) were included. At baseline, the children had BMD below the expected values for age- and sex-matched children; treatment with bisphosphonates increased BMD with mean percentage increases in spine BMD varying from 4.5 to 19.1%. None of the studies with control groups compared results between the intervention and control groups, they only compared each group with its own baseline. Overall, studies were heterogeneous in design, of variable quality and with no consistency in methods of assessing and reporting outcomes. Hence, data could not be combined or an effect size calculated. A further 43 papers were included in the safety review; side-effects were generally transient. Two studies assessed treatment with calcium and/or vitamin D; BMD was increased from 0.75 to 0.830 g/cm2 after 6 months and BMD Z-score from -2.8 to -2.3 after 6 months and -2.4 after 1 year. There are relatively few long-term studies on the occurrence of low BMD and fragility fractures in children with JIA, with most studies only following children for 1 or 2 years. However, the long- and short-term data indicate that children with JIA have a lower BMD and more fractures than children without JIA. There are very few data on long-term bone health

  4. Idiopathic hypersomnia

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/000803.htm Idiopathic hypersomnia To use the sharing features on this page, please enable JavaScript. Idiopathic hypersomnia is a sleep disorder in which a person ...

  5. [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

  6. Transient osteoporosis.

    PubMed

    Korompilias, Anastasios V; Karantanas, Apostolos H; Lykissas, Marios G; Beris, Alexandros E

    2008-08-01

    Transient osteoporosis is characterized primarily by bone marrow edema. The disease most commonly affects the hip, knee, and ankle in middle-aged men. Its cause remains unknown. The hallmark that separates transient osteoporosis from other conditions presenting with a bone marrow edema pattern is its self-limited nature. Laboratory tests usually do not contribute to the diagnosis. Plain radiographs may reveal regional osseous demineralization. Magnetic resonance imaging is used primarily for early diagnosis and monitoring disease progression. Early differentiation from more aggressive conditions with long-term sequelae is essential to avoid unnecessary treatment. Clinical entities such as transient osteoporosis of the hip and regional migratory osteoporosis are spontaneously resolving conditions. However, early differential diagnosis and surgical treatment are crucial for the patient with osteonecrosis of the hip or knee.

  7. MALE OSTEOPOROSIS

    PubMed Central

    Oliveira, Lindomar Guimarães; Guimarães, Mara Lucia Rassi

    2015-01-01

    ABSTRACT Population aging is a reality that is being faced worldwide, and Brazil is no different. Osteoporosis was considered to be a postmenopausal women's disease for many years. Men have many development and hormonal factors that differentiate their skeletal maturation, which affects the incidence of osteoporosis and fractures. An up-to-date review of the specific literature within the Medline system is presented. PMID:27022584

  8. What Is Osteoporosis?

    MedlinePlus

    ... your browser. Home Osteoporosis Osteoporosis Basics 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 ...

  9. Osteoporosis guidelines.

    PubMed

    Barlow, D H

    2007-10-01

    The Position Statement from the International Menopause Society (IMS) in 2004 recommends the use of hormone therapy for the 'avoidance of bone-wasting and fractures'. It also states that 'prevention, not treatment, is the most feasible goal'. In updating the Statement, this paper considers the relevance of Osteoporosis Guidelines. Relevant documents will be of two broad types. These may be consensus statements/position statements that summarize the 'state of the art' for practitioners, based on the work of expert groups, or they may be formal Guidelines generated through formal 'evidence-based' methodology. The former approach is generally used by Societies and can be generated through relatively efficient consensus processes. The latter approach will normally involve extensive work and cost, necessarily becomes very detailed, involving systematic review and technology appraisal and can lead to highly specific recommendations on intervention thresholds. For the revision of the general IMS Position Statement, the specific IMS Paper on Postmenopausal Osteoporosis (2005) must be a key reference document. This provides a description of the international consensus on the management of osteoporosis up to late 2004 and which remains relevant today. Additionally, other consensus statements and systematic guidelines need to be considered. Across these documents providing guidance, the substantial influence of the International Osteoporosis Foundation/National Osteoporosis Foundation Position Paper, defining a 'New approach to the development of assessment guidelines for osteoporosis', can be seen. This flagged the importance of a shift from guidance, tying the diagnostic threshold to the intervention threshold, and instead advised linking the intervention threshold to estimated fracture risk probability. This moves the intervention decision away from a simple bone density threshold to a more complex, but more realistic, threshold estimate, taking into account a range of

  10. 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

    Berntson, Lillemor; Nordal, Ellen; Fasth, Anders; Aalto, Kristiina; Herlin, Troels; Nielsen, Susan; Rygg, Marite; Zak, Marek; Rönnelid, Johan

    2014-01-01

    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). 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. 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. 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.

  11. Postmenopausal osteoporosis.

    PubMed

    Eastell, Richard; O'Neill, Terence W; Hofbauer, Lorenz C; Langdahl, Bente; Reid, Ian R; Gold, Deborah T; Cummings, Steven R

    2016-09-29

    Osteoporosis is a metabolic bone disorder that is characterized by low bone mass and micro-architectural deterioration of bone tissue. Fractures of the proximal femur, the vertebrae and the distal radius are the most frequent osteoporotic fractures, although most fractures in the elderly are probably at least partly related to bone fragility. The incidence of fractures varies greatly by country, but on average up to 50% of women >50 years of age are at risk of fractures. Fractures severely affect the quality of life of an individual and are becoming a major public health problem owing to the ageing population. Postmenopausal osteoporosis, resulting from oestrogen deficiency, is the most common type of osteoporosis. Oestrogen deficiency results in an increase in bone turnover owing to effects on all types of bone cells. The imbalance in bone formation and resorption has effects on trabecular bone (loss of connectivity) and cortical bone (cortical thinning and porosity). Osteoporosis is diagnosed using bone density measurements of the lumbar spine and proximal femur. Preventive strategies to improve bone health include diet, exercise and abstaining from smoking. Fractures may be prevented by reducing falls in high-risk populations. Several drugs are licensed to reduce fracture risk by slowing down bone resorption (such as bisphosphonates and denosumab) or by stimulating bone formation (such as teriparatide). Improved understanding of the cellular basis for osteoporosis has resulted in new drugs targeted to key pathways, which are under development.

  12. 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.

  13. Leucine-rich α2-glycoprotein as the acute-phase reactant to detect systemic juvenile idiopathic arthritis disease activity during anti-interleukin-6 blockade therapy: A case series.

    PubMed

    Shimizu, Masaki; Nakagishi, Yasuo; Inoue, Natsumi; Mizuta, Mao; Yachie, Akihiro

    2017-09-01

    To assess the role of leucine-rich α2-glycoprotein (LRG) as a biomarker for monitoring systemic juvenile idiopathic arthritis (s-JIA) disease activity during interleukin (IL)-6 blockade treatment. We serially measured serum LRG levels in four s-JIA patients treated with the anti-IL-6 receptor antibody tocilizumab and determined the correlation between clinical symptoms and other inflammatory biomarkers and proinflammatory cytokines, including IL-18, IL-6, neopterin, and tumor necrosis factor-α receptor type I and II. The serum levels of LRG and proinflammatory cytokines were determined using enzyme-linked immunosorbent assay. Serum LRG levels increased concomitantly with s-JIA disease flare-up and macrophage activation syndrome development. Furthermore, even in the clinically inactive phase, serum LRG levels were well above normal values. There were no correlations between serum LRG levels and indicators of s-JIA disease activity other than aspartate aminotransferase. There were significant positive correlations between serum LRG levels and proinflammatory cytokines. Serum LRG levels might be a unique and potential biomarker of s-JIA disease activity during IL-6 blockade treatment.

  14. 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

    SciTech Connect

    Whitehouse, W.P.; Rees, M.; Curtis, D.; Sundqvist, A.; Parker, K.; Chung, E.; Baralle, D.; Gardiner, R.M.

    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 recessive 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.

  15. Premenopausal Osteoporosis.

    PubMed

    Cohen, Adi

    2017-03-01

    Most premenopausal women with low trauma fracture(s) or low bone mineral density have a secondary cause of osteoporosis or bone loss. Where possible, treatment of the underlying cause should be the focus of management. Premenopausal women with an ongoing cause of bone loss and those who have had, or continue to have, low trauma fractures may require pharmacologic intervention. Clinical trials provide evidence of benefits of bisphosphonates and teriparatide for bone mineral density in several types of premenopausal osteoporosis, but studies are small and do not provide evidence regarding fracture risk reduction.

  16. [Osteoporosis treatment].

    PubMed

    Uebelhart, B; Rizzoli, R

    2006-01-04

    As for any chronic disease, adherence to osteoporosis treatment is low. Folates and vitamin B12 decrease hip fracture risk in elderly Japanese with stroke. Raloxifene (Evista) decreases the incidence of positive estrogen receptor breast cancer and could prevent cardiovascular events in patients at high risk. Strontium ranelate (Protélos) prevents hip fracture in elderly women. The action of alendronate (Fosamax) on bone mineral density and markers of bone remodelling is of higher amplitude than that of risedronate (Actonel). Once monthly ibandronate (Bonviva) increases bone mineral density in post menopausal women with osteoporosis. Excessive suppression of bone remodelling and osteonecrosis of the yaws could be related to bisphosphonate intake.

  17. Superior oblique tendon (Brown's) syndrome as the presenting finding in childhood onset HLA-B27-related enthesitis and juvenile idiopathic oligoarticular arthritis.

    PubMed

    Pham, C; Utz, V; Marcotty, A; Zeft, A; Rychwalski, P

    2014-11-06

    We report two patients who presented with Brown's syndrome. The first is a 7-year-old boy who at the time of his diagnosis was also found to have enthesitis and HLA-B27 positivity. The second patient was diagnosed with bilateral Brown's syndrome at 13 months of age. At age 7 she developed a persistent oligoarticular arthritis and unilateral anterior iritis consistent with the oligoarticular Juvenile Idiopatic Arthritis (JIA) phenotype. These cases highlight ophthalmologic findings and diagnostic considerations with respect to Brown's syndrome and associated childhood onset rheumatologic disease.

  18. 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 ...

  19. Idiopathic anaphylaxis.

    PubMed

    Greenberger, Paul A

    2007-05-01

    Idiopathic anaphylaxis is a prednisone-responsive condition without external cause, but it can coexist with food-, medication-, or exercise-induced anaphylaxis. Mast cell activation may occur at night or after foods that have been eaten with impunity many times previously. Idiopathic anaphylaxis can be classified into frequent (if there are six or more episodes per year or two episodes in the last 2 months) or infrequent (if episodes occur less often). Idiopathic anaphylaxis-generalized consists of urticaria or angioedema associated with severe respiratory distress, syncope or hypotension, and gastrointestinal symptoms. Idiopathic anaphylaxis-angioedema consists of massive tongue enlargement or severe pharyngeal or laryngeal swelling with urticaria or peripheral angioedema. The differential diagnosis of idiopathic anaphylaxis is reviewed, and treatment approaches are presented.

  20. The importance of trunk perception during brace treatment in moderate juvenile idiopathic scoliosis: What is the impact on self-image?

    PubMed

    Paolucci, Teresa; Piccinini, Giulia; Iosa, Marco; Piermattei, Cristina; De Angelis, Simona; Zangrando, Federico; Saraceni, Vincenzo Maria

    2017-01-01

    The perception of body image and the deformity of the trunk in patients with adolescent idiopathic scoliosis (AIS) are a silver lining that has yet to be discussed in the relevant literature during brace rehabilitation treatment. To determine whether and how the use of the brace changes perception of the trunk in patients with AIS by the drawing test. We observed 32 subjects with AIS from our Rehabilitation outpatient clinic and divided them into the brace treatment (BG-16 subjects) and the non-brace treatment (CG-16 subjects). Trunk perception and quality of life were evaluated using the Trunk Appearance Perception Scale and Scoliosis Research Society-22 questionnaire, and the perception of one's back was measured by the drawing test. Pain was lower in BG versus CG (p= 0.095). Satisfaction with the treatment was higher in BG than in CG (p= 0.002). Self-image did not differ significantly between the groups in terms of TAPS. Drawings of the most severe cases of scoliosis were made by the group without the brace. The use of the brace corrects the function of the trunk and has a positive influence on its perception.

  1. Idiopathic hypersomnia.

    PubMed

    Billiard, M; Merle, C; Carlander, B; Ondze, B; Alvarez, D; Besset, A

    1998-04-01

    Identification of idiopathic hypersomnia dates back 20 years only. It typically consists of prolonged nocturnal sleep, great difficulty waking up in the morning or at the end of a nap, and constant or recurrent excessive daytime sleepiness. Complete and incomplete forms are encountered. Twenty-three subjects fulfilling ICSD criteria are reported with clinical, polysomnographic and immunogenetic data. Considering differential diagnosis is an important step in the diagnosis of idiopathic hypersomnia. Idiopathic hypersomnia is much less frequent than narcolepsy. A strong genetic component is suggested by the high proportion of familial cases. No association with HLA has been evidenced to date.

  2. 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

  3. Osteoporosis and Hispanic Women

    MedlinePlus

    ... not supported by your browser. Home Osteoporosis Women Osteoporosis and Hispanic Women Publication available in: PDF (54 KB) Related Resources Women Fitness: Overtraining Risks Pregnancy, Nursing and Bone Health Osteoporosis and African American ...

  4. Male osteoporosis.

    PubMed

    Giusti, A; Bianchi, G

    2014-07-28

    As a result of population ageing worldwide, osteoporotic fractures are becoming a serious problem in the western world. Osteoporotic fractures are associated with a significant burden in terms of morbidity, mortality, and economic costs. Although less frequent than in women, male osteoporosis is also a relatively common problem. Since bone loss and fragility fractures in men have been recognized as a serious medical condition, over the last two decades several studies have investigated a number of aspects related to the pathogenesis, diagnosis and assessment, prevention and treatment of male osteoporosis. A better understanding of factors underlying increased bone fragility in men has led to the definition of appropriate screening and diagnostic strategies, and the development of treatments that have shown to improve bone mineral density and, in some cases, reduce fracture risk in men as well as in women. This review will summarize recent findings on male osteoporosis with a particular focus on risk factors and causes of bone loss, and available therapeutic options.

  5. Corticosteroid osteoporosis.

    PubMed

    Sambrook, P; Lane, N E

    2001-07-01

    Corticosteroids are widely used and effective agents for the control of many inflammatory diseases, but corticosteroid osteoporosis is a common problem associated with their long term high dose use. Prevention of corticosteroid osteoporosis is preferable to treatment of established corticosteroid bone loss. Several large double-blind controlled clinical trials in patients with corticosteroid osteoporosis have recently been published that provide new insights into its treatment. Based upon available evidence, the rank order of choice for prophylaxis would be a bisphosphonate followed by a vitamin D metabolite or an oestrogen type medication. Calcium alone appears to be unable to prevent rapid bone loss in patients starting corticosteroids, especially with prednisolone doses at 10 mg a day or greater. If an active vitamin D metabolite is used, calcium supplementation should be avoided unless dietary calcium intake is low. Hormone replacement therapy should be considered if hypogonadism is present. Since vertebral fracture is a common and important complication of high dose corticosteroid therapy, these findings suggest that rapid bone loss and hence fractures, can be prevented by prophylactic treatment. Although the follow-up data is limited, it is likely that such therapy needs to be continued beyond 12 months whilst patients continue significant doses of corticosteroid therapy.

  6. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

    PubMed

    Ravelli, Angelo; Minoia, Francesca; Davì, Sergio; Horne, AnnaCarin; Bovis, Francesca; Pistorio, Angela; Aricò, Maurizio; Avcin, Tadej; Behrens, Edward M; De Benedetti, Fabrizio; Filipovic, Lisa; Grom, Alexei A; Henter, Jan-Inge; Ilowite, Norman T; Jordan, Michael B; Khubchandani, Raju; Kitoh, Toshiyuki; Lehmberg, Kai; Lovell, Daniel J; Miettunen, Paivi; Nichols, Kim E; Ozen, Seza; Pachlopnik Schmid, Jana; Ramanan, Athimalaipet V; Russo, Ricardo; Schneider, Rayfel; Sterba, Gary; Uziel, Yosef; Wallace, Carol; Wouters, Carine; Wulffraat, Nico; Demirkaya, Erkan; Brunner, Hermine I; Martini, Alberto; Ruperto, Nicolino; Cron, Randy Q

    2016-03-01

    To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ=0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies. Published by the BMJ Publishing Group Limited. For permission to use (where not

  7. APL-2, an altered peptide ligand derived from heat-shock protein 60, induces interleukin-10 in peripheral blood mononuclear cell derived from juvenile idiopathic arthritis patients and downregulates the inflammatory response in collagen-induced arthritis model.

    PubMed

    Lorenzo, Norailys; Cantera, Dolores; Barberá, Ariana; Alonso, Amaris; Chall, Elsy; Franco, Lourdes; Ancizar, Julio; Nuñez, Yanetsy; Altruda, Fiorella; Silengo, Lorenzo; Padrón, Gabriel; Del Carmen Dominguez, Maria

    2015-02-01

    Juvenile idiopathic arthritis (JIA) is a heterogeneous group of diseases characterized by autoimmune arthritis of unknown cause with onset before age of 16 years. Methotrexate provides clinical benefits in JIA. For children who do not respond to methotrexate, treatment with anti-tumor necrosis factor (TNF)-α is an option. However, some patients do not respond or are intolerant to anti-TNF therapy. Induction of peripheral tolerance has long been considered a promising approach to the treatment of chronic autoimmune diseases. We aimed to evaluate the potentialities of two altered peptide ligands (APLs) derived from human heat-shock protein 60, an autoantigen involved in the pathogenesis of autoimmune arthritis, in JIA patients. Interferon (IFN)-γ, TNF-α and interleukin (IL)-10 levels were determined in ex vivo assays using peripheral blood mononuclear cells (PBMC) from these patients. Wild-type peptide and one of these APLs increased IFN-γ and TNF-α levels. Unlike, the other APLs (called APL2) increased the IL-10 level without affecting IFN-γ and TNF-α levels. On the other hand, APL2 induces a marked activation of T cells since it transforms cell cycle phase's distribution of CD4+ T cells from these patients. In addition, we evaluated the therapeutic effect of APL2 in collagen-induced arthritis model. Therapy with APL2 reduced arthritis scores and histological lesions in mice. This effect was associated to a decrease in TNF-α and IL-17 levels. These results indicate a therapeutic potentiality of APL2 for JIA.

  8. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

    PubMed

    Ravelli, Angelo; Minoia, Francesca; Davì, Sergio; Horne, AnnaCarin; Bovis, Francesca; Pistorio, Angela; Aricò, Maurizio; Avcin, Tadej; Behrens, Edward M; De Benedetti, Fabrizio; Filipovic, Lisa; Grom, Alexei A; Henter, Jan-Inge; Ilowite, Norman T; Jordan, Michael B; Khubchandani, Raju; Kitoh, Toshiyuki; Lehmberg, Kai; Lovell, Daniel J; Miettunen, Paivi; Nichols, Kim E; Ozen, Seza; Pachlopnik Schmid, Jana; Ramanan, Athimalaipet V; Russo, Ricardo; Schneider, Rayfel; Sterba, Gary; Uziel, Yosef; Wallace, Carol; Wouters, Carine; Wulffraat, Nico; Demirkaya, Erkan; Brunner, Hermine I; Martini, Alberto; Ruperto, Nicolino; Cron, Randy Q

    2016-03-01

    To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ = 0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies. © 2015, American College of Rheumatology.

  9. Establishing clinical meaning and defining important differences for Patient-Reported Outcomes Measurement Information System (PROMIS(®)) measures in juvenile idiopathic arthritis using standard setting with patients, parents, and providers.

    PubMed

    Morgan, Esi M; Mara, Constance A; Huang, Bin; Barnett, Kimberly; Carle, Adam C; Farrell, Jennifer E; Cook, Karon F

    2017-03-01

    Patient-Reported Outcomes Measurement Information System (PROMIS) measures are used increasingly in clinical care. However, for juvenile idiopathic arthritis (JIA), scores lack a framework for interpretation of clinical severity, and minimally important differences (MID) have not been established, which are necessary to evaluate the importance of change. We identified clinical severity thresholds for pediatric PROMIS measures of mobility, upper extremity function (UE), fatigue, and pain interference working with adolescents with JIA, parents of JIA patients, and clinicians, using a standard setting methodology modified from educational testing. Item parameters were used to develop clinical vignettes across a range of symptom severity. Vignettes were ordered by severity, and panelists identified adjacent vignettes considered to represent upper and lower boundaries separating category cut-points (i.e., from none/mild problems to moderate/severe). To define MIDs, panelists reviewed a full score report for the vignettes and indicated which items would need to change and by how much to represent "just enough improvement to make a difference." For fatigue and UE, cut-points among panels were within 0.5 SD of each other. For mobility and pain interference, cut-scores among panels were more divergent, with parents setting the lowest cut-scores for increasing severity. The size of MIDs varied by stakeholders (parents estimated largest, followed by patients, then clinicians). MIDs also varied by severity classification of the symptom. We estimated clinically relevant severity cut-points and MIDs for PROMIS measures for JIA from the perspectives of multiple stakeholders and found notable differences in perspectives.

  10. The most important needs and preferences of patients for support from health care professionals: A reflective practice on (transitional) care for young adults with Juvenile Idiopathic Arthritis.

    PubMed

    Ammerlaan, Judy W; van Os-Medendorp, Harmieke; de Boer-Nijhof, Nienke C; Prakken, Berent; Bijlsma, Johannes W J; Kruize, Aike A

    2017-03-12

    In this manuscript, presented as a Reflective Practice, the learning experiences and reflections of a healthcare team on redeveloping the transitional care for young adults with a juvenile rheumatic disease are described. In this process of redeveloping care, the healthcare team experienced that small step, driven by patient stories and involvement of patients in all phases from development to evaluation, led to meaningful results. The eHealth interventions, developed to support the transition and to increase self-management were found to be feasible and evaluated positively by the young adult group. But the healthcare team also experienced that the focus on the patient alone, is not enough to implement self-management interventions and sustain patient centered care in daily practice. How healthcare professionals personally think and feel about patient centered care is essential and needs to be discussed in daily care.It determines the way of being present with attention and commitment in daily health care. It affects the hands, head and heart. A daily reflection on shared answers of the patient and the health care professional to the question 'what is the most important to you?'may help to implement patient centered care in health practice.

  11. Idiopathic scoliosis.

    PubMed

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit.

  12. Distraction magnitude and frequency affects the outcome in juvenile idiopathic patients with growth rods: finite element study using a representative scoliotic spine model.

    PubMed

    Agarwal, Aakash; Zakeri, Amanda; Agarwal, Anand K; Jayaswal, Arvind; Goel, Vijay K

    2015-08-01

    Growth rods are used to limit the progression of scoliosis without restraining the opportunity for the spine to grow. However, major complications like rod breakage, screw loosening, and altered sagittal contour have been encountered. To analyse the effect of the magnitude of distraction forces on the T1-S1 growth, maximum von Mises stresses on the rods, sagittal contours, and load at the pedicle screw-bone interface and quantify the maximum stresses on the rod for a period of 24 months using different frequencies of distraction in a representative scoliotic spine model. A representative finite element model of a juvenile scoliotic spine was used to study the effect of magnitude and frequency of distraction on growth rods. A representative scoliotic model was developed and instrumented using proximal foundation, distal foundation, and rods. Part 1: simulation steps comprised 6 months of growth under various distraction forces to analyze effects of distraction force on the biomechanics of the spine and instrument. Part 2: simulation steps comprised 24 months of growth under various intervals of distraction to analyze effects of distraction interval on the propensity of rod fracture. Part 1: an optimal distraction force exists for which the growth is sustained with minimum stress on the rod, lower loads at screw-bone interface, and unaltered sagittal contours. Part 2: the stresses on the rods were highest for 12-month distraction (2 distractions in 2 years) and lowest for 2-month distraction (12 distractions in 2 years). The data and trend suggest that as the distraction forces vary so do the effects on spinal growth. The results of this study also signify the importance of shorter distraction period in reducing the stresses on the rods. Published by Elsevier Inc.

  13. Idiopathic hypersomnia.

    PubMed

    Billiard, M

    1996-08-01

    Idiopathic hypersomnia is not as well delineated as narcolepsy and its history is much more recent. There are at least two forms of the disorder: (1) a polysymptomatic form, characterized by excessive daytime sleepiness, nocturnal sleep of abnormally long duration, and signs of sleep drunkenness on awakening, and (2) a monosymptomatic form that manifests only by excessive daytime sleepiness. The most widely used laboratory procedures are nocturnal polysomnographic recording following by an MSLT demonstrating a mean sleep latency of less than 10 minutes. At least in the polysymptomatic form, however, continuous polysomnography on an ad lib protocol deserves to be performed to catch the abnormally long major sleep episode and the long unrefreshing naps. Idiopathic hypersomnia is probably one of the most overdiagnosed sleep disorders. Several other disorders must be excluded before the diagnosis can be considered conclusive. Treatment of idiopathic hypersomnia relies on stimulants, which are frequently less effective and less well tolerated than in narcolepsy.

  14. Gender Disparities in Osteoporosis

    PubMed Central

    Alswat, Khaled A.

    2017-01-01

    Osteoporosis is a growing health concern worldwide and its complications are as prevalent as other common chronic disease complications such as hypertension and diabetes. In this review, we will discuss the role of gender in osteoporosis, especially related to peak bone mass and maturation, rate of annual bone loss, screening, prevalence of osteoporosis and its related fractures, mortality after osteoporosis-related fracture, fracture risk predication using different technologies and the impact of gender on osteoporosis management. PMID:28392857

  15. Glucocorticoid osteoporosis.

    PubMed

    Sambrook, Philip N

    2002-01-01

    Postmenopausal women are at greatest risk of rapid bone loss and fracture with glucocorticoids and should be actively considered for prophylactic measures. In men and premenopausal women receiving glucocorticoids, the decision to use anti-osteoporosis prophylaxis is less clear and depends upon baseline bone mineral density [BMD], anticipated dose and duration of glucocorticoids. Based upon evidence the order of choice for prophylaxis would be a bisphosphonate followed by a vitamin D metabolite or hormone replacement therapy [HRT]. Calcium alone appears unable to prevent rapid bone loss in patients starting glucocorticoids. HRT should clearly be considered if hypogonadism is present. In patients receiving chronic low dose glucocorticoids, treatment with calcium and vitamin D may be sufficient to prevent further bone loss. However since fracture risk is a function of multiple factors including the degree of reduction in BMD as well as the duration of exposure, treatment with therapy to increase BMD will reduce fracture risk even in patients receiving chronic low dose glucocorticoids.

  16. Understanding osteoporosis.

    PubMed Central

    Marcus, R.

    1991-01-01

    Considerable progress has been achieved recently in our understanding of the normal process by which bone mass is regulated. Age-related trabecular bone loss is characterized not simply by a global loss of bone but also by cortical porosity and loss of trabecular connections. Because bone strength depends on architectural as well as material properties, bone quantity alone cannot define fracture risk with precision. Traditional therapies for osteoporosis increase bone mass, and estrogen therapy, in particular, profoundly decreases fracture risk. The pharmacologic restoration of bone quantity and quality, however, remains elusive. Modern biotechnology offers the hope that progress may come about through the development of growth factors and other osteotropic compounds for clinical use. Images PMID:1877231

  17. Four dermatomyositis-specific autoantibodies-anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5-in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort.

    PubMed

    Bodoki, Levente; Nagy-Vincze, Melinda; Griger, Zoltán; Betteridge, Zoe; Szöllősi, Lászlóné; Dankó, Katalin

    2014-12-01

    Idiopathic inflammatory myopathies (IIMs) are chronic systemic autoimmune diseases characterised by symmetrical, proximal muscle weakness. Dermatomyositis represents one subset of IIMs, in which skin rashes are present in addition to muscle weakness. Myositis-specific antibodies can only be detected in myositis, and they are directed against specific proteins found in the cytoplasm or in the nucleus of cells. With this case-based article, we introduce the recently detected anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5 antibodies that form various clinical groups. These antibodies could be detected in patients with dermatomyositis. The myositis-specific autoantibodies of three hundred and thirty-seven Hungarian patients with IIM were detected. Retrospective analysis of the clinical findings has also been introduced by revision of the medical history. We had twelve patients with anti-TIF1γ positivity, four patients with anti-NXP2 positivity and four patients with anti-SAE positivity. We did not have any positive anti-MDA5 patients. The most relevant clinical findings were similar to those seen in previously published reports. Eleven of the twelve patients with anti-TIF1γ positivity had classical dermatomyositis. Three of the twelve anti-TIF1γ patients had cancer during the disease progression. This was two out of four for the anti-NXP2 subgroup and one in four for the anti-SAE subgroup. In two juvenile dermatomyositis cases, typical ulceration was seen in patients with anti-TIF1γ positivity. The frequency of pulmonary fibrosis during the disease progression was 2/12, 1/4 and 1/4 in anti-TIF1γ, anti-NXP2 and anti-SAE, respectively. Other extra-muscular manifestations, such as arthralgia, dysphagia, dysphonia and dyspnoea, were also detectable. The myositis subgroups determined by these myositis-specific autoantibodies differ from each other in their symptoms, prognosis and therapy responsiveness. Their detection is helpful for the preparation of an adequate

  18. Efficacy and safety of open-label etanercept on extended oligoarticular juvenile idiopathic arthritis, enthesitis-related arthritis and psoriatic arthritis: part 1 (week 12) of the CLIPPER study

    PubMed Central

    Horneff, Gerd; Burgos-Vargas, Ruben; Constantin, Tamas; Foeldvari, Ivan; Vojinovic, Jelena; Chasnyk, Vyacheslav G; Dehoorne, Joke; Panaviene, Violeta; Susic, Gordana; Stanevica, Valda; Kobusinska, Katarzyna; Zuber, Zbigniew; Mouy, Richard; Rumba-Rozenfelde, Ingrida; Breda, Luciana; Dolezalova, Pavla; Job-Deslandre, Chantal; Wulffraat, Nico; Alvarez, Daniel; Zang, Chuanbo; Wajdula, Joseph; Woodworth, Deborah; Vlahos, Bonnie; Martini, Alberto; Ruperto, Nicolino

    2014-01-01

    Objective To investigate the efficacy and safety of etanercept (ETN) in paediatric subjects with extended oligoarticular juvenile idiopathic arthritis (eoJIA), enthesitis-related arthritis (ERA), or psoriatic arthritis (PsA). Methods CLIPPER is an ongoing, Phase 3b, open-label, multicentre study; the 12-week (Part 1) data are reported here. Subjects with eoJIA (2–17 years), ERA (12–17 years), or PsA (12–17 years) received ETN 0.8 mg/kg once weekly (maximum 50 mg). Primary endpoint was the percentage of subjects achieving JIA American College of Rheumatology (ACR) 30 criteria at week 12; secondary outcomes included JIA ACR 50/70/90 and inactive disease. Results 122/127 (96.1%) subjects completed the study (mean age 11.7 years). JIA ACR 30 (95% CI) was achieved by 88.6% (81.6% to 93.6%) of subjects overall; 89.7% (78.8% to 96.1%) with eoJIA, 83.3% (67.2% to 93.6%) with ERA and 93.1% (77.2% to 99.2%) with PsA. For eoJIA, ERA, or PsA categories, the ORs of ETN vs the historical placebo data were 26.2, 15.1 and 40.7, respectively. Overall JIA ACR 50, 70, 90 and inactive disease were achieved by 81.1, 61.5, 29.8 and 12.1%, respectively. Treatment-emergent adverse events (AEs), infections, and serious AEs, were reported in 45 (35.4%), 58 (45.7%), and 4 (3.1%), subjects, respectively. Serious AEs were one case each of abdominal pain, bronchopneumonia, gastroenteritis and pyelocystitis. One subject reported herpes zoster and another varicella. No differences in safety were observed across the JIA categories. Conclusions ETN treatment for 12 weeks was effective and well tolerated in paediatric subjects with eoJIA, ERA and PsA, with no unexpected safety findings. PMID:23696632

  19. Rationale, design and baseline data of a mixed methods study examining the clinical impact of a brief transition programme for young people with juvenile idiopathic arthritis: the DON'T RETARD project

    PubMed Central

    Hilderson, Deborah; Westhovens, Rene; Wouters, Carine; Van der Elst, Kristien; Goossens, Eva; Moons, Philip

    2013-01-01

    Objectives To describe (1) the content of a transition programme for young people with juvenile idiopathic arthritis (JIA) designed as a brief intervention, (2) the rationale and design of a mixed-methods study evaluating the clinical impact of this transition programme and (3) to provide baseline data of the intervention group. Design An ‘embedded experimental’ design is used for the evaluation of the transition programme. A ‘one-group pretest-posttest, with a non-equivalent posttest-only comparison group design’ is used to quantitatively evaluate the impact of the transition programme, applying both longitudinal and comparative analyses. Subsequently, experiences of adolescents and their parents who participated in the experimental group will be analysed qualitatively using content analysis. Setting Participants in the intervention are recruited at a tertiary care centre in Belgium. The comparison group participants are recruited from one tertiary and three secondary care centres in Belgium. Participants The intervention group consists of 33 young people (25 females; 8 males) with a median age of 16 years. Main diagnoses are persistent or extended oligoarticular JIA (33%), polyarticular JIA (30%), enthesitis-related JIA (21%) or systemic arthritis (15%). Intervention The transition programme comprises eight key components: (1) transition coordinator; (2) providing information and education; (3) availability by telephone; (4) information about and contact with an adult care programme; (5) guidance of parents; (6) meeting with peers; (7) transfer plan; and (8) actual transfer to adult care. Primary and secondary outcomes The primary outcome is health status, as perceived by the adolescents. Secondary outcomes are health status, as perceived by the parents; medication adherence; illness-related knowledge; quality of life; fatigue; promotion of independence; support of autonomy; behavioural control and psychological control. Results At baseline, the median

  20. Anti-Interleukin-6 Receptor Tocilizumab for Severe Juvenile Idiopathic Arthritis-Associated Uveitis Refractory to Anti-Tumor Necrosis Factor Therapy: A Multicenter Study of Twenty-Five Patients.

    PubMed

    Calvo-Río, Vanesa; Santos-Gómez, Montserrat; Calvo, Inmaculada; González-Fernández, M Isabel; López-Montesinos, Berta; Mesquida, Marina; Adán, Alfredo; Hernández, María Victoria; Maíz, Olga; Atanes, Antonio; Bravo, Beatriz; Modesto, Consuelo; Díaz-Cordovés, Gisela; Palmou-Fontana, Natalia; Loricera, Javier; González-Vela, M C; Demetrio-Pablo, Rosalía; Hernández, J L; González-Gay, Miguel A; Blanco, Ricardo

    2017-03-01

    To assess the efficacy of tocilizumab (TCZ) for the treatment of juvenile idiopathic arthritis (JIA)-associated uveitis. We conducted a multicenter study of patients with JIA-associated uveitis that was refractory to conventional immunosuppressive drugs and anti-tumor necrosis factor (anti-TNF) agents. We assessed 25 patients (21 female; 47 affected eyes) with a mean ± SD age of 18.5 ± 8.3 years. Uveitis was bilateral in 22 patients. Cystoid macular edema was present in 9 patients. Ocular sequelae found at initiation of TCZ included cataracts (n = 13), glaucoma (n = 7), synechiae (n = 10), band keratopathy (n = 12), maculopathy (n = 9), and amblyopia (n = 5). Before TCZ, patients had received corticosteroids, conventional immunosuppressive drugs, and biologic agents (median 2 [range 1-5]), including adalimumab (n = 24), etanercept (n = 8), infliximab (n = 7), abatacept (n = 6), rituximab (n = 2), anakinra (n = 1), and golimumab (n = 1). Patients received 8 mg/kg TCZ intravenously every 4 weeks in most cases. TCZ yielded rapid and maintained improvement in all ocular parameters. After 6 months of therapy, 79.2% of patients showed improvement in anterior chamber cell numbers, and 88.2% showed improvement after 1 year. Central macular thickness measured by optical coherence tomography in patients with cystoid macular edema decreased from a mean ± SD of 401.7 ± 86.8 μm to 259.1 ± 39.5 μm after 6 months of TCZ (P = 0.012). The best-corrected visual acuity increased from 0.56 ± 0.35 to 0.64 ± 0.32 (P < 0.01). After a median follow-up of 12 months, visual improvement persisted, and complete remission of uveitis was observed in 19 of 25 patients. Significant reduction in the prednisone dosage was also achieved. The main adverse effects were severe autoimmune thrombocytopenia in 1 patient, pneumonia and then autoimmune anemia and thrombocytopenia in 1 patient, and viral

  1. Osteoporosis and Asian American Women

    MedlinePlus

    ... not supported by your browser. Home Osteoporosis Women Osteoporosis and Asian American Women Publication available in: PDF ( ... Are Available? Resources For Your Information What Is Osteoporosis? Osteoporosis is a condition in which the bones ...

  2. 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

  3. Core decompression for juvenile osteonecrosis.

    PubMed

    Herrera-Soto, José A; Price, Charles T

    2011-07-01

    Core decompression may be used as adjunct for treatment in some cases of Legg-Calvé-Perthes disease (LCPD). The primary application is for patients with onset at 12 years of age or older. We recommend classifying these older patients as idiopathic juvenile osteonecrosis and treating them similarly to adults with avascular necrosis. Juvenile osteonecrosis may benefit from core decompression combined with shelf acetabuloplasty during the early stages of necrosis. Younger children with LCPD may benefit from decompression by fenestration of the femoral head. Experience in adult-onset osteonecrosis and our early experience suggest that some patients may benefit from these adjunctive treatments. Copyright © 2011 Elsevier Inc. All rights reserved.

  4. 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)

  5. Osteoporosis and Your Spine

    MedlinePlus

    ... Movement › Osteoporosis and Your Spine Osteoporosis and Your Spine Your spine is made up of small bones ... called kyphosis. Kyphosis and Bone Breaks in the Spine The bones in the spine are called vertebrae. ...

  6. 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)

  7. Osteoarthritis, osteoarthrosis, and idiopathic condylar resorption.

    PubMed

    Mercuri, Louis G

    2008-05-01

    The term "osteoarthritis" has classically been defined as a low-inflammatory arthritic condition. The term "osteoarthrosis," a synonym for osteoarthritis in the medical orthopedic literature, has recently come to be identified in the dental/temporomandibular joint (TMJ) disorders literature with any noninflammatory arthritic condition that results in similar degenerative changes as in osteoarthritis. The term "idiopathic condylar resorption," also known as "progressive condylar resorption," is described as a dysfunctional remodeling of the TMJ manifested by morphologic change, decreased ramal height, progressive mandibular retrusion in the adult, or decreased mandibular growth in the juvenile. This article discusses the diagnosis and management of osteoarthritic TMJ disorders and idiopathic condylar resorption.

  8. 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 ...

  9. Osteoporosis in unstable adult scoliosis

    SciTech Connect

    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 to 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.

  10. Osteoporosis and Gastrointestinal Disease

    PubMed Central

    Weinerman, Stuart

    2010-01-01

    Gastrointestinal disease is often overlooked or simply forgotten as a cause of osteoporosis. Yet, the consequences of osteoporotic fractures can be devastating. Although the bulk of the published experience regarding osteoporosis is derived from the postmenopausal population, this review will focus on gastrointestinal disorders implicated in osteoporosis, with an emphasis on inflammatory bowel disease and celiac disease. The unique aspects of gastrointestinal diseases associated with osteoporosis include early onset of disease (and, therefore, prolonged exposure to risk factors for developing osteoporosis, particularly with inflammatory bowel disease and celiac disease), malabsorption, and maldigestion of nutrients necessary for bone health and maintenance (eg, calcium, vitamin D), as well as the impact of glucocorticoids. These factors, when added to smoking, a sedentary lifestyle, hypogonadism, and a family history of osteoporosis, accumulate into an imposing package of predictors for osteoporotic fracture. This paper will review the identification and treatment strategies for patients with gastrointestinal disorders and osteoporosis. PMID:20978554

  11. [QOL evaluation for osteoporosis].

    PubMed

    Endo, Naoto

    2012-06-01

    It is important to evaluate the Health Related QOL (HRQOL) in the treatment of the patients with osteoporosis. In 1999, the Japanese Society for Bone and Mineral Research made Japanese Osteoporosis Quality of Life Questionnaire (JOQOL) to evaluate the osteoporosis specific HRQOL of the Japanese patients. This JOQOL 1999-version was revised in 2000. JOQOL 2001 version consists of six domain 38 items, using five-point scale ranging from 0 to 4, scored from 0 to 152. Osteoporosis is a bone disorder with decreased bone strength, resulting in bone fragility and consequently fractures. The vertebral fractures cause a change in the spinal column (kyphosis) and the decline of the physical function due to the back pain. This is the decrease in QOL of the patients with osteoporosis compared to cases without osteoporosis. Therefore, assessment of QOL are recommended in the prevention and treatment for osteoporosis.

  12. Osteoporosis: Therapeutic Options.

    PubMed

    Ivanova, Stefka; Vasileva, Liliya; Ivanova, Stanislava; Peikova, Lily; Obreshkova, Danka

    2015-01-01

    The definition of osteoporosis was originally formulated at a conference of the World Health Organization (WHO) in 1993 as 'a systemic skeletal disease characterized by decreased bone mass and altered micro-architecture of bone tissue, leading to enhanced bone fragility and risk of fractures'. Osteoporosis is characterized by low bone mineral density (BMD) and loss of the structural and bio-mechanical properties that are required to maintain bone homeostasis. This review aims to address the currently available options in prevention and treatment of osteoporosis. Management of osteoporosis includes non-pharmacological treatment - diet rich of calcium and vitamin D, healthy lifestyle, proper exercise plan, and pharmacological therapy. Combination of non-pharmacological and pharmacological treatment options have to be considered for prevention of osteoporosis and minimization of the risk of fractures. Given the heterogeneity of osteoporosis syndrome and lack of significant number of comparative studies, the choice of a pharmacological agents should be individualized.

  13. Comparing Osteoporosis Drugs: The Bisphosphonates

    MedlinePlus

    Drugs to Treat Low Bone Density Comparing Osteoporosis Drugs: The Bisphosphonates What is osteoporosis (low bone density)? Osteoporosis is a condition in which the body does not build enough new bone. ...

  14. Gender specificity and osteoporosis.

    PubMed

    Bilezikian, J P

    2000-10-01

    To a certain extent, the emphasis placed on women in light of the predominance of osteoporosis in this sex is well-justified. As researchers are appreciating the potential size of the male population at risk for osteoporosis, increasing interest is becoming apparent on the part of investigators and the pharmaceutical companies that are developing therapies. As more is learned about osteoporosis in men, experts will then be in a position to better understand the similarities and differences between genders.

  15. [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.

  16. Perspectives on osteoporosis.

    PubMed

    Kaltenborn, K C

    1992-12-01

    The goal of this chapter was to provide enough information so that the following questions could be answered in a clinical context: 1. Does the patient have osteoporosis or a risk for it? Is densitometry needed? 2. Why does the patient have osteoporosis? Is the diagnosis "the tip of the iceberg" because of an occult secondary cause? 3. Is the patient receiving adequate calcium? Can the patient benefit from estrogen? 4. What clinical information or tests are required to follow a patient with osteoporosis? 5. What drugs are indicated for osteoporosis? Which are promising and which require further research?

  17. 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)

  18. 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)

  19. Diagnosis and treatment of adolescent idiopathic scoliosis.

    PubMed

    Burton, Monique S

    2013-11-01

    Scoliosis is defined as a lateral curvature of the spine greater than 10 degrees on radiography that is typically associated with trunk rotation. The three major types of scoliosis are congenital, idiopathic, and neuromuscular. Idiopathic scoliosis is divided into three subcategories based on the age of onset. Infantile idiopathic scoliosis affects patients younger than 3 years, juvenile idiopathic scoliosis appears in children between 3 and 10 years, and adolescent idiopathic scoliosis (AIS) occurs in skeletally immature patients older than 10 years. AIS is the most common form of idiopathic scoliosis. Approximately 2% to 4% of children aged 10 to 16 years have some degree of spinal curvature. Although some researchers view routine screening for AIS as controversial, well-child examinations and sports physicals are an optimal time to evaluate for AIS in the clinical setting. In 2008, the American Academy of Orthopaedic Surgeons, the Scoliosis Research Society, the Pediatric Orthopaedic Society of North America, and the American Academy of Pediatrics convened a task force to review the issues related to scoliosis screening and issued an information statement concluding that although screening has limitations, the potential benefits that patients with idiopathic scoliosis receive from early treatment can be substantial. Recommendations are now that females are screened twice, at age 10 and 12 years, and males once at age 13 or 14 years. Screening during routine well-child examinations and/or school-based evaluations will help identify patients who need ongoing monitoring. The evaluation of curvatures in conjunction with the level of skeletal maturity will help to guide the management of the curvature. Copyright 2013, SLACK Incorporated.

  20. Idiopathic cardiomegaly*

    PubMed Central

    1968-01-01

    Cardiomyopathies are certain heart diseases of unknown etiology and pathogenesis, occurring mostly in tropical and subtropical areas, where they constitute a major clinical problem and sometimes a public health problem. The need for international co-operation in the study of such forms of heart disease has long been recognized and WHO convened informal meetings of investigators on various aspects of the subject in 1964, 1965 and 1966. Out of these have arisen co-operative studies co-ordinated by WHO. In November 1967 a fourth informal meeting was held in Kingston, Jamaica, to review the following topics: the progress reports from all co-operating laboratories; the different types of cardiomyopathies; past experience with cardiac registries, and the diagnostic importance of coronary angiography. Steps were taken towards the formulation of a standard terminology, since too many confusing names are currently employed to mean “cardiomegaly of unknown origin”. A common name, “idiopathic cardiomegaly”, was therefore suggested for future use. The account presented here was prepared by Dr Z. Fejfar, Chief Medical Officer, Cardiovascular Diseases, World Health Organization, Geneva, on behalf of the other participants and is a précis of some of the information that was exchanged, some of the views that were expressed and of the suggestions that were made. PMID:4235740

  1. [Pathophysiology of immobilization osteoporosis.

    PubMed

    Menuki, Kunitaka; Sakai, Akinori

    Enhancement of bone resorption and suppression of bone formation in response to reduced mechanical stress cause rapid bone loss. pharmacotherapy for immobilization osteoporosis in motor paralysis and long-term bedrest is effective therapy. Early intervention for rapid bone loss is important for immobilization osteoporosis.

  2. Pituitary Disorders and Osteoporosis

    PubMed Central

    Jawiarczyk-Przybyłowska, Aleksandra

    2015-01-01

    Various hormonal disorders can influence bone metabolism and cause secondary osteoporosis. The consequence of this is a significant increase of fracture risk. Among pituitary disorders such effects are observed in patients with Cushing's disease, hyperprolactinemia, acromegaly, and hypopituitarism. Severe osteoporosis is the result of the coexistence of some of these disorders and hypogonadism at the same time, which is quite often. PMID:25873948

  3. Animal models for osteoporosis.

    PubMed

    Komori, Toshihisa

    2015-07-15

    The major types of osteoporosis in humans are postmenopausal osteoporosis, disuse osteoporosis, and glucocorticoid-induced osteoporosis. Animal models for postmenopausal osteoporosis are generated by ovariectomy. Bone loss occurs in estrogen deficiency due to enhanced bone resorption and impaired osteoblast function. Estrogen receptor α induces osteoclast apoptosis, but the mechanism for impaired osteoblast function remains to be clarified. Animal models for unloading are generated by tail suspension or hind limb immobilization by sciatic neurectomy, tenotomy, or using plaster cast. Unloading inhibits bone formation and enhances bone resorption, and the involvement of the sympathetic nervous system in it needs to be further investigated. The osteocyte network regulates bone mass by responding to mechanical stress. Osteoblast-specific BCL2 transgenic mice, in which the osteocyte network is completely disrupted, can be a mouse model for the evaluation of osteocyte functions. Glucocorticoid treatment inhibits bone formation and enhances bone resorption, and markedly reduces cancellous bone in humans and large animals, but not consistently in rodents.

  4. [Osteoporosis in collagen diseases].

    PubMed

    Momohara, S; Aritomi, H

    1994-09-01

    The pathogenesis of osteoporosis in patients with rheumatic diseases, especially rheumatoid arthritis (RA), is poorly understood. The duration of the disease, the severity of the inflammatory process, gender, age, steroid therapy and menopause have been suggested as risk factors for osteoporosis in patients with RA. Although these factors may contribute to the development of osteoporosis, the influence of one specific factor is difficult to evaluate. It is said that the treatment with steroids has a deleterious effect on bone turnover, but this effect has been controversial. The dose margin of prednisone that will lead to osteoporosis is not known but has been estimated to be 10 mg per day. Fractures and stress fractures in patients with RA are probably much more common. Further study concerning osteoporosis in rheumatic diseases is necessary.

  5. [Osteoporosis: a clinical perspective].

    PubMed

    Matikainen, Niina

    2016-01-01

    Osteoporosis is defined by decreased bone density and microarchitectural deterioration that predispose to fragility fractures. The WHO diagnostic criteria of osteoporosis require bone densitometry but treatment is possible on the basis of high clinical fracture risk and can be assessed by the FRAX risk algorithm. All those subject to fracture risk should be advised about proper basic treatment of osteoporosis, including exercise, prevention of falls, smoking cessation, avoidance of alcohol intake, and dietary or supplemental abundance of calcium and vitamin D. Underlying diseases must be studied after diagnosis of osteoporosis even if treatment is initiated without densitometry. When indicated, specific osteoporosis therapy includes bisphosphonates, denosumab, teriparatide, strontium ranelate or SERMs. In hypogonadism, gonadal steroids may be indicated alone or in addition to a specific treatment. Treatment effect and continuation are assessed after 2 to 5 years.

  6. Management of severe osteoporosis.

    PubMed

    Miller, Paul D

    2016-01-01

    Severe osteoporosis represents a disease of high mortality and morbidity. Recognition of what constitutes and causes severe osteoporosis and aggressive intervention with pharmacological agents with evidence to reduce fracture risk are outlined in this review. This review is a blend of evidence obtained from literature searches from PubMed and The National Library of Medicine (USA), clinical experience and the author's opinions. The review covers the recognition of what constitutes severe osteoporosis, and provides up-to-date references on this sub-set of high risk patients. Severe osteoporosis can be classified by using measurements of bone densitometry, identification of prevalent fractures, and, knowledge of what additional risk factors contribute to high fracture risk. Once recognized, the potential consequences of severe osteoporosis can be mitigated by appropriate selection of pharmacological therapies and modalities to reduce the risk for falling.

  7. Idiopathic oedema and diuretics.

    PubMed Central

    Dunnigan, M. G.; Denning, D. W.; Henry, J. A.; de Wolff, F. A.

    1987-01-01

    Diuretic abuse has been invoked as the cause of idiopathic oedema. In this study, eight patients with idiopathic oedema were studied. Symptoms and weight variation continued despite the proven absence of diuretics in seven of them as determined by urinary chromatograms. Idiopathic oedema cannot therefore be attributed to diuretic use alone. PMID:3671223

  8. OSTEOPOROSIS DIAGNOSIS AND TREATMENT

    PubMed Central

    de Souza, Márcio Passini Gonçalves

    2015-01-01

    Articles that update the state of knowledge regarding osteoporosis run the risk of quickly becoming obsolete because research and studies on osteoporosis today are arousing great interest among researchers, the pharmaceutical and medical equipment industries, governments and even WHO. All orthopedists know about osteoporosis because of its most deleterious effect: osteoporotic fracture. Osteoporosis without fractures does not arouse suspicion because this is a pathological condition with a nonspecific clinical profile. Osteoporotic fractures have an economic cost (from treatment), a social cost (from its sequelae) and a medical cost (from deaths). Many fractures could be avoided through diagnosing osteoporosis prior to the first fracture and thus many temporary and permanent disabilities could be avoided and many lives saved. Awareness of the risk factors for osteoporosis raises suspicions and bone densitometry aids in diagnosis. Treatment should be based on the physiopathology of the disease. Hence, for prevention or treatment of osteoporosis, the activity of osteoclasts should be diminished or the activity of osteoblasts should be increased, or both. Treatment that reduces the incidence of fractures by improving the bone geometry and microarchitecture would be ideal. Newly formed bone tissue needs to have good cell and matrix quality, normal mineralization, a good ratio between mineralized (mechanically resistant) and non-mineralized (flexible) bone, and no accumulated damage. The ideal treatment should have a positive remodeling rate and fast and long-lasting therapeutic effects. Such effects need to be easily detectable. They need to be safe. PMID:27022545

  9. [Genetics of osteoporosis].

    PubMed

    Cohen-Solal, M; de Vernejoul, M C

    2004-12-01

    Osteoporosis is a multifactorial disease involving genetic component and several environmental factors. Some rare diseases that are associated with osteoporosis such as Lobstein disease or the "pseudoglial osteoporosis" syndrom are monogenetic. Nevertheless common osteoporosis is a polygenic affection resulting from the interaction between the polymorphism of different genes and the environmental factors. The genetic component of osteoporosis encompasses roughly 60 to 70% of bone mineral density, whereas the effect on fracture risk seems lower because of the importance of other environmental factors as falls. Many polymorphisms of candidate genes involved in the regulation of bone mass have been correlated to bone density. It is likely that many genes participate to the regulation of bone density although the existence of a major gene is highly suspected. Moreover linkage analysis after genome-wide search in populations with severe osteoporosis has focused on some regions of interest (QTL) on the chromosomes. This will allow to localize one or more specific genes. The current genetic studies on different populations affected by osteoporosis or not will be useful in order to better predict the fracture risk in association with bone density and biochemical markers of bone turnover. Moreover, this will lead to the development of new treatments of osfeoporosis and will help to adapt the therapy for individual patients.

  10. OSTEOPOROSIS DIAGNOSIS AND TREATMENT.

    PubMed

    de Souza, Márcio Passini Gonçalves

    2010-01-01

    Articles that update the state of knowledge regarding osteoporosis run the risk of quickly becoming obsolete because research and studies on osteoporosis today are arousing great interest among researchers, the pharmaceutical and medical equipment industries, governments and even WHO. All orthopedists know about osteoporosis because of its most deleterious effect: osteoporotic fracture. Osteoporosis without fractures does not arouse suspicion because this is a pathological condition with a nonspecific clinical profile. Osteoporotic fractures have an economic cost (from treatment), a social cost (from its sequelae) and a medical cost (from deaths). Many fractures could be avoided through diagnosing osteoporosis prior to the first fracture and thus many temporary and permanent disabilities could be avoided and many lives saved. Awareness of the risk factors for osteoporosis raises suspicions and bone densitometry aids in diagnosis. Treatment should be based on the physiopathology of the disease. Hence, for prevention or treatment of osteoporosis, the activity of osteoclasts should be diminished or the activity of osteoblasts should be increased, or both. Treatment that reduces the incidence of fractures by improving the bone geometry and microarchitecture would be ideal. Newly formed bone tissue needs to have good cell and matrix quality, normal mineralization, a good ratio between mineralized (mechanically resistant) and non-mineralized (flexible) bone, and no accumulated damage. The ideal treatment should have a positive remodeling rate and fast and long-lasting therapeutic effects. Such effects need to be easily detectable. They need to be safe.

  11. POST-MENOPAUSAL OSTEOPOROSIS

    PubMed Central

    Hogg, C. Alison

    1997-01-01

    Millions of women will be prescribed hormone replacement therapy (HRT) and be told that it will prevent osteoporosis occuring, despite the fact that there is doubt about its long term usefulness. Preventive measures outlined in this article are much more preferable, but need to be directed towards the whole population, not just menopausal women. The prevention of osteoporosis is an important public health issue which needs to be addressed now, not in the next century. This article explores the issues that surround the medicalisation of post-menopausal osteoporosis. PMID:17987149

  12. Juvenile Firesetting.<