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Sample records for lupus erythematosus successfully

  1. Systemic lupus erythematosus

    MedlinePlus

    Disseminated lupus erythematosus; SLE; Lupus; Lupus erythematosus; Butterfly rash - SLE; Discoid lupus ... Mouth sores. Sensitivity to sunlight. Skin rash: A "butterfly" rash in about half the people with SLE. ...

  2. Lupus erythematosus

    SciTech Connect

    Tuffanelli, D.L.

    1981-02-01

    Lupus erythematosus (LE) is a multisystem disease. Genetic predisposition, altered immunity, hormones, drugs, viruses, and ultraviolet light all may play a role in etiology. A wide range of cutaneous lesions occur, and variants such as subacute cutaneous LE, complement-deficient LE, and neonatal LE have recently been emphasized. Management of the LE patient, including appropriate diagnostic studies and therapy relevant to the dermatologist, is discussed in the review.

  3. Systemic lupus erythematosus.

    PubMed

    Samuelson, S J; Friedlander, A H; Swerdloff, M

    1980-04-01

    A case of osteomyelitis of the mandible in a patient with systemic lupus erythematosus is described. Both the disease process and the treatment modalities must be understood for correct management. PMID:6928895

  4. Hypertrophic discoid lupus erythematosus.

    PubMed

    Farley-Loftus, Rachel; Elmariah, Sarina B; Ralston, Jonathan; Kamino, Hideko; Franks, Andrew G

    2010-11-15

    Hypertrophic discoid lupus erythematosus is a distinct form of chronic cutaneous (discoid) lupus, which is characterized by hyperkeratotic plaques that typically are observed over the face, arms, and upper trunk. We present the case of a 43-year-old man with verrucous plaques that were distributed symmetrically over the face, who initially was treated with oral antibiotics and topical glucocorticoids for acne vulgaris. A biopsy specimen confirmed the diagnosis of hypertrophic discoid lupus erythematosus. The clinical and histopathologic features of this clinical variant are reviewed.

  5. Thalidomide in cutaneous lupus erythematosus.

    PubMed

    Pelle, Michelle T; Werth, Victoria P

    2003-01-01

    For nearly 50 years, thalidomide has struggled between success and controversy. After causing an epidemic of phocomelia and other birth defects during the 1960s, affecting thousands of neonates, thalidomide was used as a sedative in selective disorders including leprosy. The potent anti-inflammatory properties of thalidomide were serendipitously discovered while treating patients with erythema nodosum leprosum, and the drug is now approved by the US FDA for the treatment of this disease. Subsequently, the immunosuppressant effects of thalidomide, including the complex modulation of many cytokines, have been recognized. One promising application of thalidomide has been the treatment of cutaneous lupus erythematosus. Among the largest series reviewed, the drug has been found to ameliorate cutaneous lupus erythematosus in 90% of patients, on average. Remission is achieved in approximately 15-20% of patients with cutaneous lupus erythematosus at doses between 50-400 mg daily. Contraceptive concerns and the recognized neuropathic effects of thalidomide limit the use of the drug in patients with cutaneous lupus. Physicians who prescribe thalidomide in the US must be registered with the drug manufacturer. With appropriate control of drug access and close physician monitoring, thalidomide provides a needed therapeutic option for the treatment of refractory cases of cutaneous lupus erythematosus.

  6. Early Cutaneous Lupus Erythematosus

    PubMed Central

    Sams, Wiley M.

    1966-01-01

    Cutaneous disorders which manifest themselves on the exposed parts are more likely than are hidden lesions to cause the patient to seek professional services promptly. Usually he consults his family physician or the community dermatologist. The physician who first sees the patient is dependent upon his own resources for management and diagnosis. A background of experience, a measure of energy and an inquisitive attitude are the necessary ingredients for successful management. The difficulties involved in differentiating early lupus erythematosus and polymorphic light eruptions cannot be invariably resolved even with the most complete review. The course of the disorder and the response to environmental factors supply important clues. Investigative work, especially in the field of immunology, offers hope for the solution of some of our problems. PMID:5909872

  7. Genetics Home Reference: systemic lupus erythematosus

    MedlinePlus

    ... Genetics Home Health Conditions systemic lupus erythematosus systemic lupus erythematosus Enable Javascript to view the expand/collapse ... Download PDF Open All Close All Description Systemic lupus erythematosus (SLE) is a chronic disease that causes ...

  8. Successful Treatment with Posaconazole of a Patient with Chronic Chagas Disease and Systemic Lupus Erythematosus

    PubMed Central

    Pinazo, María-Jesús; Espinosa, Gerard; Gállego, Montserrat; López-Chejade, Paulo Luis; Urbina, Julio A.; Gascón, Joaquim

    2010-01-01

    American Trypanosomiasis or Chagas disease (CD) is a neglected disease that affects Latin American people worldwide. Two old antiparasitic drugs, benznidazole and nifurtimox, are currently used for specific CD treatment with limited efficacy in chronic infections and frequent side effects. New drugs are needed for patients with chronic CD as well as for immunosuppressed patients, for whom the risk of reactivation is life-threatening. We describe a case of chronic CD and systemic lupus erythematosus (SLE) that required immunosuppression to control the autoimmune process. It was found that benznidazole induced a reduction, but not an elimination, of circulating Trypanosoma cruzi levels, whereas subsequent treatment with posaconazole led to a successful resolution of the infection, despite the maintenance of immunosuppressive therapy. PMID:20348503

  9. Cutaneous Lupus Erythematosus: Diagnosis and treatment

    PubMed Central

    Okon, Lauren G.; Werth, Victoria P.

    2013-01-01

    Cutaneous lupus erythematosus encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several subtypes, including acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Chronic cutaneous lupus erythematosus includes discoid lupus erythematosus, lupus erythematosus profundus, chilblain cutaneous lupus, and lupus tumidus. Diagnosis of these diseases requires proper classification of the subtype, through a combination of physical exam, laboratory studies, histology, antibody serology, and occasionally direct immunofluorescence, while ensuring to exclude systemic disease. Treatment of cutaneous lupus consists of patient education on proper sun protection along with appropriate topical and systemic agents. Systemic agents are indicated in cases of widespread, scarring, or treatment-refractory disease. In this review, we discuss issues in classification and diagnosis of the various subtypes of CLE, as well as provide an update on therapeutic management. PMID:24238695

  10. Treatment of Cutaneous Lupus Erythematosus

    PubMed Central

    Kim, Grace K.; Del Rosso, James Q.

    2013-01-01

    The treatment of cutaneous lupus erythematosus is centered upon formulating a regimen of topical and systemic therapies designed to reduce disease activity and minimize cosmetic damage. Sun avoidance and sunscreen are important preventative measures proven to minimize cutaneous lupus erythematosus exacerbations. Limited disease is typically managed with topical corticosteroids or calcineurin inhibitors. Antimalarial therapy is the gold standard of systemic therapy. Many other treatments have been studied in patients with recalcitrant cutaneous lupus erythematosus, and their use must be evaluated based on individual risk-benefit concerns. R-salbutamol and pulsed dye laser therapy have proven to be effective topical alternatives. Additional systemic agents include retinoids, immunosuppressants, immunomodulators, biologics, and other experimental therapies with novel modes of action. According to the Oxford Centre for Evidence-based Medicine criteria for evaluating the strength of evidence supporting an individual treatment measure, no therapy for cutaneous lupus erythematosus has achieved Level 1 status. This demonstrates the need for randomized, controlled trials and systematic reviews of all cutaneous lupus erythematosus interventions in order to meet increasing standards and demand for evidence-based practice. PMID:23320123

  11. Systemic lupus erythematosus.

    PubMed

    Kaul, Arvind; Gordon, Caroline; Crow, Mary K; Touma, Zahi; Urowitz, Murray B; van Vollenhoven, Ronald; Ruiz-Irastorza, Guillermo; Hughes, Graham

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. Women of childbearing age and certain racial groups are typically predisposed to developing the condition. Rare, inherited, single-gene complement deficiencies are strongly associated with SLE, but the disease is inherited in a polygenic manner in most patients. Genetic interactions with environmental factors, particularly UV light exposure, Epstein-Barr virus infection and hormonal factors, might initiate the disease, resulting in immune dysregulation at the level of cytokines, T cells, B cells and macrophages. Diagnosis is primarily clinical and remains challenging because of the heterogeneity of SLE. Classification criteria have aided clinical trials, but, despite this, only one drug (that is, belimumab) has been approved for use in SLE in the past 60 years. The 10-year mortality has improved and toxic adverse effects of older medications such as cyclophosphamide and glucocorticoids have been partially offset by newer drugs such as mycophenolate mofetil and glucocorticoid-sparing regimes. However, further improvements have been hampered by the adverse effects of renal and neuropsychiatric involvement and late diagnosis. Adding to this burden is the increased risk of premature cardiovascular disease in SLE together with the risk of infection made worse by immunosuppressive therapy. Challenges remain with treatment-resistant disease and symptoms such as fatigue. Newer therapies may bring hope of better outcomes, and the refinement to stem cell and genetic techniques might offer a cure in the future. PMID:27306639

  12. Lupus erythematosus revisited.

    PubMed

    Kuhn, Annegret; Wenzel, Joerg; Bijl, Marc

    2016-01-01

    Lupus erythematosus (LE) is a multifactorial autoimmune disease with clinical manifestations of differing severity. The exact pathomechanisms and interactions resulting in the inflammatory and immunological processes of this heterogeneous disease remain elusive. Approaches in the understanding of the pathomechanisms revealed that the clinical expression of LE is predisposed by susceptibility genes and that various environmental factors are responsible for an abnormal immune response. Several studies demonstrated that ultraviolet (UV) light is one of the major factors in the pathogenesis of the disease. Standardized photoprovocation in patients with LE has been shown to be a safe and efficient model for evaluating the underlying pathomechanisms which lead to the production of autoantibodies and immune complexes. In particular, interferons were defined as important players in the early activation of the immune system and were observed to play a specific role in the immunological interface between the innate and the adaptive immune system. Abnormalities or disturbances in the different processes of cell death, such as apoptosis or necrosis, have also been recognized as crucial in the pathogenesis of LE. Although each process is different and characterized by unique features, the processes are interrelated and result in a complex disease.

  13. Biomarkers for systemic lupus erythematosus.

    PubMed

    Ahearn, Joseph M; Liu, Chau-Ching; Kao, Amy H; Manzi, Susan

    2012-04-01

    The urgent need for lupus biomarkers was demonstrated in September 2011 during a Workshop sponsored by the Food and Drug Administration: Potential Biomarkers Predictive of Disease Flare. After 2 days of discussion and more than 2 dozen presentations from thought leaders in both industry and academia, it became apparent that highly sought biomarkers to predict lupus flare have not yet been identified. Even short of the elusive biomarker of flare, few biomarkers for systemic lupus erythematosus (SLE) diagnosis, monitoring, and stratification have been validated and employed for making clinical decisions. This lack of reliable, specific biomarkers for SLE hampers proper clinical management of patients with SLE and impedes development of new lupus therapeutics. As such, the intensity of investigation to identify lupus biomarkers is climbing a steep trajectory, lending cautious optimism that a validated panel of biomarkers for lupus diagnosis, monitoring, stratification, and prediction of flare may soon be in hand.

  14. Epratuzumab for systemic lupus erythematosus.

    PubMed

    Wallace, D J; Goldenberg, D M

    2013-04-01

    Epratuzumab (EMab, UCB, Immunomedics) is a humanized monoclonal antibody targeting CD22 that is being studied in clinical trials for patients with a variety of rheumatic and hematologic conditions, including systemic lupus erythematosus (SLE). An overview of its mechanism of action is followed by a summary of completed lupus studies, and a preview of studies in progress. The agent clearly has anti-inflammatory activity and is a potentially useful agent in the management of autoimmune disorders.

  15. Lupus Erythematosus Panniculitis in Pregnancy

    PubMed Central

    Gondane, Swati; Kothiwala, Rajkumar; Dangi, Sapna; Meherda, Ashok

    2015-01-01

    A case of lupus erythematosus (LE) panniculitis in pregnancy without any lesions of discoid LE or systemic LE is being reported. There were no systemic symptoms. Her ANA, anti-dsDNA, anti-Ro/SSA, and anti-La/SSB antibodies were within normal limits. Diagnosis of lupus panniculitis was considered on clinical and histopathological grounds. The condition responded favorably to systemic steroid therapy. PMID:26677307

  16. [Blepharitis--rare in systemic lupus erythematosus].

    PubMed

    Gyl, Fekete; Anna-Adrien, Csiszár; Oanţă, A; Irimie, M; Edit, Fekete Júlia

    2008-01-01

    Chronic lupus erythematosus often appear on the face, ears, and scalp. In exchange eyelids involvement as a chronic blepharitis is rare. We describe six cases of discoid lupus erythematosus with lesions on the face and ears who have eyelids involvement, too. Blepharitis is a rare involvement of the chronic lupus erythematosus and in case that is isolated, the diagnosis is belated and can lead to complications. The involvement of the lower eyelids is more frequently especially theirs lateral third.

  17. Cytokines in systemic lupus erythematosus.

    PubMed

    Lourenço, Elaine V; La Cava, Antonio

    2009-04-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by the production of autoantibodies that can form immune complexes and deposit in tissues, causing inflammation and organ damage. There is evidence that interferons and some interleukins can have an active role in the pathogenesis of SLE and can contribute significantly to the immune imbalance in the disease, whereas the role of some cytokines (such as TNF) is still debated. This review discusses the activity of several cytokines in SLE, their effects on the immune cells in relation to the disease pathogenesis, and the promise and limitations of cytokine-based therapies in clinical trials for lupus patients.

  18. Fatigue in systemic lupus erythematosus

    PubMed Central

    Ahn, Grace E; Ramsey-Goldman, Rosalind

    2012-01-01

    Systemic lupus erythematosus is a chronic inflammatory autoimmune disease often characterized by fatigue, with significant effects on physical functioning and wellbeing. The definition, prevalence and factors associated with fatigue, including physical activity, obesity, sleep, depression, anxiety, mood, cognitive dysfunction, vitamin D deficiency/insufficiency, pain, effects of medications and comorbidities, as well as potential therapeutic options of fatigue in the systemic lupus erythematosus population are reviewed. Due to variability in the reliability and validity of various fatigue measures used in clinical studies, clinical trial data have been challenging to interpret. Further investigation into the relationships between these risk factors and fatigue, and improved measures of fatigue, may lead to an improvement in the management of this chronic inflammatory disease. PMID:22737181

  19. Discoid lupus erythematosus presenting as unilateral blepharitis.

    PubMed

    Au, Leon

    2006-01-01

    A 39-year-old man presented with a 4-month history of unilateral blepharitis that did not respond to conventional treatment. Punch biopsy confirmed the diagnosis of discoid lupus erythematosus. Unilateral blepharitis as the only presenting sign of discoid lupus erythematosus is uncommon but should be considered in the differential diagnosis in patients with asymmetric blepharitis.

  20. Infections and systemic lupus erythematosus

    PubMed Central

    Skare, Thelma Larocca; Dagostini, Jéssica Scherer; Zanardi, Patricia Imai; Nisihara, Renato Mitsunori

    2016-01-01

    ABSTRACT Objective To determine the incidence of infections in a population of systemic lupus erythematosus individuals and the characteristics of infections regarding original site, as well as to study the possible associations between infections and treatment. Methods An analytical retrospective study using data from medical charts of systemic lupus erythematosus patients from a single university hospital. A total of 144 patients followed up for five years were included. Data collected comprised age of patients and age at onset of lupus, sex and ethnicity, disease duration before the study period, medications, cumulative dose of prednisone, occurrence of infections and their original site. Results The most frequent infections were urinary tract infections (correlated to use of prednisone − p<0.0001 and cyclophosphamide − p=0.045), upper airways infections (correlated to use of prednisone − p=0.0004, mycophenolate mofetil − p=0.0005, and cyclosporine − p=0.025), and pneumonia (associated to prednisone − p=0.017). Conclusion Prednisone was the drug more often associated with presence of infections, pointing to the need for a more judicious management of this drug. PMID:27074234

  1. Belimumab in Systemic Lupus Erythematosus.

    PubMed

    Srivastava, Ankita

    2016-01-01

    Belimumab is the only approved biological agent for the treatment of systemic lupus erythematosus (SLE). It is a fully humanized IgG1γ monoclonal antibody directed against soluble B lymphocyte stimulator (BLyS). It is indicated as an add-on therapy for the treatment of adult patients with active, autoantibody-positive SLE, who are receiving standard therapy. Belimumab is generally well-tolerated, common adverse effects include infections, infusion reactions, hypersensitivity, headache, nausea, and fatigue. Psychiatric events including suicidal tendency, progressive multifocal leukoencephalopathy and malignancies too have been reported. Apart from SLE, the drug is also being tried for other autoimmune disorders. PMID:27688447

  2. Belimumab in Systemic Lupus Erythematosus

    PubMed Central

    Srivastava, Ankita

    2016-01-01

    Belimumab is the only approved biological agent for the treatment of systemic lupus erythematosus (SLE). It is a fully humanized IgG1γ monoclonal antibody directed against soluble B lymphocyte stimulator (BLyS). It is indicated as an add-on therapy for the treatment of adult patients with active, autoantibody-positive SLE, who are receiving standard therapy. Belimumab is generally well-tolerated, common adverse effects include infections, infusion reactions, hypersensitivity, headache, nausea, and fatigue. Psychiatric events including suicidal tendency, progressive multifocal leukoencephalopathy and malignancies too have been reported. Apart from SLE, the drug is also being tried for other autoimmune disorders.

  3. Belimumab in Systemic Lupus Erythematosus

    PubMed Central

    Srivastava, Ankita

    2016-01-01

    Belimumab is the only approved biological agent for the treatment of systemic lupus erythematosus (SLE). It is a fully humanized IgG1γ monoclonal antibody directed against soluble B lymphocyte stimulator (BLyS). It is indicated as an add-on therapy for the treatment of adult patients with active, autoantibody-positive SLE, who are receiving standard therapy. Belimumab is generally well-tolerated, common adverse effects include infections, infusion reactions, hypersensitivity, headache, nausea, and fatigue. Psychiatric events including suicidal tendency, progressive multifocal leukoencephalopathy and malignancies too have been reported. Apart from SLE, the drug is also being tried for other autoimmune disorders. PMID:27688447

  4. Belimumab in systemic lupus erythematosus

    PubMed Central

    Vilas-Boas, Andreia; Morais, Sandra A; Isenberg, David A

    2015-01-01

    Systemic lupus erythematosus (SLE) is one of the most challenging autoimmune disorders with a complex pathophysiology and diverse clinical presentation. Many drugs have been used to treat SLE with suboptimal results, especially in patients with moderate-to-severe disease. Belimumab is the first biological drug to be approved for the treatment of SLE in more than 50 years. This monoclonal antibody blocks B-cell activating factor, a cytokine important for B-cell differentiation and survival. In this review we focus on the activity of belimumab in patients with SLE and discuss the controversies of its use. PMID:26509047

  5. Epigenetics in systemic lupus erythematosus

    PubMed Central

    XIAO, GONG; ZUO, XIAOXIA

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, with mechanisms that remain to be elucidated. Previous studies have proposed that genes and environments are required for lupus to develop and flare. It has been found that epigenetics have a significant influence on SLE. The present review will concentrate on epigenetics in SLE. There are a number of studies reporting that autoreactive T cells and B cells in patients with SLE have evidence of altered patterns of DNA methylation, modifications of histones and microRNA (miRNA). Long noncoding RNAs (lncRNAs) are another type of noncoding RNAs, which have an important role in epigenetics. lncRNAs may possibly become a new hotspot in SLE. PMID:26893827

  6. Malignancies in systemic lupus erythematosus

    PubMed Central

    Kale, Mruganka; Ramsey-Goldman, Rosalind; Gordon, Caroline; Clarke, Ann E; Bernatsky, Sasha

    2013-01-01

    The purpose of this review is to underline important advancements in the understanding of cancer risks in systemic lupus erythematosus (SLE). In SLE, there is an increased risk of specific kinds of malignancy. For example, the risk of non-Hodgkin’s lymphoma is increased several-fold in SLE versus the general population. In addition, heightened risks for lung cancer, thyroid cancer and cervical dysplasia in SLE have been found. Some have postulated that immunosuppressive drugs play a role, as well as other important mediators, such as lupus disease activity itself. One new frontier being explored is the significant finding of a decreased risk of certain nonhematologic cancers (e.g., breast, ovarian, endometrial and prostate) in SLE. The reasons for this are currently under study. PMID:19643208

  7. [Salmonella enteritidis arthritis complicating systemic lupus erythematosus].

    PubMed

    Marzouk, S; El Aoud, S; Hriz, H; Jallouli, M; Zribi, W; Bahloul, Z

    2013-12-01

    Septic arthritis due to Salmonella in systemic lupus erythematosus is rare. We report a case of septic arthritis by Salmonella enteritidis which occurred during the evolution of systemic lupus erythematosus. A 23-year-old man was diagnosed as suffering from systemic lupus erythematosus. This diagnosis was taken on the basis of general symptoms, skin lesions, hemolytic anemia, thrombocytopenia and glomerulonephritis (class III). He was treated with three methylprednisolone boli related by high-dose regimen of prednisolone. A month and a half later, he presented fever with monoarthritis of the left elbow without any other new sign of underlying systemic disease. Bacteriological examinations isolated S. enteritidis. The patient improved with antibiotics and joint lavage. Feverish monoarthritis in systemic lupus erythematosus should be suspect to be septic arthritis. Appropriate treatment should be promptly instituted to improve the prognosis.

  8. Successful treatment of severe refractory lupus hepatitis with mycophenolate mofetil.

    PubMed

    Tagawa, Y; Saito, T; Takada, K; Kawahata, K; Kohsaka, H

    2016-04-01

    Systemic lupus erythematosus-related hepatitis, known as lupus hepatitis, is a rare manifestation of systemic lupus erythematosus, and is usually subclinical with mild abnormalities of serum liver enzymes. While cases with clinically significant and refractory lupus hepatitis are uncommon, treatment options for lupus hepatitis are to be established. Here, we report the case of a 45-year-old man with progressive lupus hepatitis accompanied by autoimmune haemolytic anaemia. Lupus hepatitis of this patient was refractory to tacrolimus, azathioprine and cyclophosphamide, but was successfully treated by mycophenolate mofetil. Mycophenolate mofetil might be an effective therapeutic option for refractory lupus hepatitis.

  9. Successful Detection of Renal Involvement in Sjögren's Syndrome Secondary to Systemic Lupus Erythematosus by Renal Biopsy.

    PubMed

    Okada, Akira; Yoshida, Taiko; Takemura, Koji; Ishigaki, Kazuyoshi; Shimizu, Akira; Takano, Hideki

    2015-01-01

    An 80-year-old man presented with a mildly decreased renal function and increased anti-double-stranded-DNA (anti-dsDNA) antibody levels, and met the diagnostic criteria of the American College of Rheumatology for systemic lupus erythematosus (SLE). However, the incremental increase in creatinine levels and the mild proteinuria were inconsistent with lupus nephritis. We performed a renal biopsy, which revealed interstitial nephritis and minor glomerular abnormalities. Further examinations determined that the renal lesion was due to Sjögren's syndrome secondary to SLE. Following treatment with oral prednisolone, the patient's renal function improved as his anti-dsDNA antibody levels decreased. This case report indicates that renal biopsy should be considered even in elderly individuals when it may assist in the diagnosis, treatment, and prognosis of the patient.

  10. Childhood-onset bullous systemic lupus erythematosus.

    PubMed

    Lourenço, D M R; Gomes, R Cunha; Aikawa, N E; Campos, L M A; Romiti, R; Silva, C A

    2014-11-01

    Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations.

  11. [Cutaneous lupus erythematosus, a multidimensional entity].

    PubMed

    Méndez-Flores, Silvia; Tinoco-Fragoso, Fátima; Hernández-Molina, Gabriela

    2015-01-01

    Skin lesions caused by systemic lupus erythematosus are among the most frequent manifestations of this disease. These lesions show great variability in both their clinical and histological expression, making their understanding and study difficult. Patients presenting with cutaneous lupus do not necessarily have serious systemic complications, but they do have significant morbidity from impact on quality of life given the extent of the lesions, chronic tendency, and the risk of scarring; hence the importance of establishing a fast and effective treatment. This paper addresses the different varieties of specific injuries attributed to lupus erythematosus, correlation with systemic activity, quality of life, and the treatments available.

  12. Drug-induced lupus erythematosus.

    PubMed

    Marzano, A V; Tavecchio, S; Menicanti, C; Crosti, C

    2014-06-01

    Drug-induced lupus erythematosus (DI-LE) is defined as an entity characterized by clinical manifestations and immunopathological serum findings similar to those of idiopathic lupus but which is temporally related to drug exposure and resolves after withdrawal of the implicated drug. Similarly to idiopathic lupus, DI-LE can be divided into systemic LE, subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE) and cutaneous LE tumidus. DI-SCLE is the most frequent variant of drug-induced cutaneous LE and presents mainly with annular-polycyclic lesions; the clinical picture is often widespread, with involvement of the lower legs that are usually spared in idiopathic SCLE. ANA and anti-Ro/SSA antibodies are typically present, whereas antihistone antibodies are uncommonly found. We have recently addressed the question whether DI-SCLE differs significantly from its idiopathic counterpart by virtue of clinical features and, based on our findings, we have suggested that the frequent occurrence of malar rash and bullous, erythema multiforme-like and vasculitic manifestations can be regarded as the hallmark of DI-SCLE. In contrast, the histology is not a useful diagnostic criterion for DI-SCLE, considering that the typical pattern of lichenoid interface dermatitis is seen only in the early stage of disease and tissue eosinophilia does not represent a differentiating histopathological feature. DI-CCLE and DI-LE tumidus, albeit possibly misdiagnosed, are rarely observed and are characterized by classic discoid lesions and erythematous-oedematous plaques on sun exposed areas, respectively. Management of DI-LE is based on the discontinuation of the offending drug; topical and/or systemic corticosteroids and other immunomodulating/immunosuppressive agents should be reserved for resistant cases.

  13. Humor in systemic lupus erythematosus

    PubMed Central

    Moura, Cristiano S.; Li, Rui; Lawrie, Sarah; Bar-Or, Amit; Clarke, Ann E.; Da Costa, Deborah; Banerjee, Devi; Bernatsky, Sasha; Lee, Jennifer L.; Pineau, Christian A.

    2015-01-01

    Objective Humor has neurophysiological effects influencing the release of cortisol, which may have a direct impact on the immune system. Laughter is associated with a decreased production of inflammatory cytokines both in the general population and in rheumatoid arthritis (RA). Our objective was to explore the effects of humor on serum cytokines [particularly interleukin-6 (IL-6)] and cortisol levels in systemic lupus erythematosus (SLE), after a standard intervention (120 min of visual comedy). Material and Methods We enrolled 58 females with SLE from consecutive patients assessed in the Montreal General Hospital lupus clinic. The subjects who consented to participate were randomized in a 1:1 ratio to the intervention (watching 120 min of comedy) or control group (watching a 120 min documentary). Measurements of cytokine and serum cortisol levels as well as 24-h urine cortisol were taken before, during, and after the interventions. We compared serum cytokine levels and serum and 24-h urine cortisol levels in the humor and control groups and performed regression analyses of these outcomes, adjusting for demographics and the current use of prednisone. Results There were no significant differences between the control and humor groups in demographics or clinical variables. Baseline serum levels of IL-6, IL-10, tumor necrosis factor-alpha, and B-cell activating factor were also similar in both groups. There was no evidence of a humor effect in terms of decreasing cytokine levels, although there was some suggestion of lowered cortisol secretion in the humor group based the 24-h urinary cortisol levels in a subgroup. Conclusion In contrast to what has been published for RA, we saw no clear effects of humor in altering cytokine levels in SLE, although interesting trends were seen for lower cortisol levels after humor intervention compared with the control group. PMID:27708912

  14. Successful experience of rituximab therapy for systemic sclerosis-associated interstitial lung disease with concomitant systemic lupus erythematosus.

    PubMed

    Sumida, Hayakazu; Asano, Yoshihide; Tamaki, Zenshiro; Aozasa, Naohiko; Taniguchi, Takashi; Takahashi, Takehiro; Toyama, Tetsuo; Ichimura, Yohei; Noda, Shinji; Akamata, Kaname; Miyazaki, Miki; Kuwano, Yoshihiro; Yanaba, Koichi; Sato, Shinichi

    2014-05-01

    Previous studies have demonstrated that B cells play critical roles in autoimmune disorders including systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). However, the effectiveness of rituximab (RTX), a chimeric anti-CD20 antibody, for SSc-associated interstitial lung disease (ILD) or SLE disease activity remains controversial. We herein report an SSc patient with severely progressed ILD and concomitant SLE treated by two cycles of RTX at baseline and half a year later. This treatment improved ILD and SLE activities, along with reduction of dermal sclerosis and serum anti-topoisomerase I antibody levels. In addition, our detailed time-course data indicate that half a year may be appropriate as an interval between each cycle of RTX therapy aimed at SSc-associated ILD or SLE. Overall, the current report could pave the way to establish RTX as a disease-modifying drug for patients with SSc and/or SLE showing resistance to other already approved medications.

  15. Periodontitis and systemic lupus erythematosus.

    PubMed

    Sete, Manuela Rubim Camara; Figueredo, Carlos Marcelo da Silva; Sztajnbok, Flavio

    2016-01-01

    A large number of studies have shown a potential association between periodontal and autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus (SLE). Similar mechanisms of tissue destruction concerning periodontitis and other autoimmune diseases have stimulated the study of a possible relationship between these conditions. This study aims to review the literature about this potential association and their different pathogenic mechanisms. Considering that periodontal disease is a disease characterized by inflammation influenced by infectious factors, such as SLE, it is plausible to suggest that SLE would influence periodontal disease and vice versa. However, this issue is not yet fully elucidated and several mechanisms have been proposed to explain this association, as deregulation mainly in innate immune system, with action of phagocytic cells and proinflammatory cytokines such as IL-1β and IL-18 in both conditions' pathogenesis, leading to tissue destruction. However, studies assessing the relationship between these diseases are scarce, and more studies focused on common immunological mechanisms should be conducted to further understanding. PMID:27267530

  16. Periodontitis and systemic lupus erythematosus.

    PubMed

    Sete, Manuela Rubim Camara; Figueredo, Carlos Marcelo da Silva; Sztajnbok, Flavio

    2016-01-01

    A large number of studies have shown a potential association between periodontal and autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus (SLE). Similar mechanisms of tissue destruction concerning periodontitis and other autoimmune diseases have stimulated the study of a possible relationship between these conditions. This study aims to review the literature about this potential association and their different pathogenic mechanisms. Considering that periodontal disease is a disease characterized by inflammation influenced by infectious factors, such as SLE, it is plausible to suggest that SLE would influence periodontal disease and vice versa. However, this issue is not yet fully elucidated and several mechanisms have been proposed to explain this association, as deregulation mainly in innate immune system, with action of phagocytic cells and proinflammatory cytokines such as IL-1β and IL-18 in both conditions' pathogenesis, leading to tissue destruction. However, studies assessing the relationship between these diseases are scarce, and more studies focused on common immunological mechanisms should be conducted to further understanding.

  17. Systemic lupus erythematosus presenting as morbid jealousy.

    PubMed Central

    Ravindran, A.; Carney, M. W.; Denman, A. M.

    1980-01-01

    A patient fulfilling the diagnostic criteria for systemic lupus erythematosus and presenting with morbid jealousy is described. There was evidence of cerebral lupus. Her physical and mental symptoms responded to a combination of chlorpromazine and steroids. The morbid mental process was probably caused by her physical condition while the content of her disordered thought and behaviour was determined by her introverted premorbid personality, religiosity, unhappy childhood experiences and frustrated desire for children. PMID:7413541

  18. Thrombocytopenia in Systemic Lupus Erythematosus

    PubMed Central

    Jung, Jin-Hee; Soh, Moon-Seung; Ahn, Young-Hwan; Um, Yoo-Jin; Jung, Ju-Yang; Suh, Chang-Hee; Kim, Hyoun-Ah

    2016-01-01

    Abstract The aim of the study was to examine the clinical characteristics and prognosis according to severity of thrombocytopenia and response to treatment for thrombocytopenia in patients with systemic lupus erythematosus (SLE). We retrospectively evaluated 230 SLE patients with thrombocytopenia, and reviewed their clinical data and laboratory findings. Thrombocytopenia was defined as platelet counts under 100,000/mm3, and patients were divided into 3 thrombocytopenia groups according to severity: mild (platelet counts >50,000/mm3), moderate (>20,000/mm3, ≤50,000/mm3), and severe (≤20,000/mm3). Clinical characteristics, treatments, and prognoses were compared among the groups. Furthermore, complete remission of thrombocytopenia was defined as platelet counts >100,000/mm3 after treatment. There was no significant difference in clinical or laboratory findings among the groups according to severity of thrombocytopenia. However, hemorrhagic complications were more frequent in severe thrombocytopenia (P < 0.001) and mortality was also higher (P = 0.001). Complete remission was achieved in 85.2% of patients. The clinical characteristics and modality of treatment did not differ between the patients with and without complete remission. Mortality in patients with complete remission (1.5%) was significantly lower than in those without complete remission (29.4%, P < 0.001). Survival was significantly higher in patients with complete remission from thrombocytopenia (odds ratio = 0.049, 95% confidence interval: 0.013–0.191, P < 0.001). The severity of thrombocytopenia in SLE patients can be a useful independent prognostic factor to predict survival. Moreover, complete remission of thrombocytopenia after treatment is an important prognostic factor. The severity of thrombocytopenia and response to treatment should be closely monitored to predict prognosis in SLE patients. PMID:26871854

  19. Systemic lupus erythematosus and Raynaud's phenomenon*

    PubMed Central

    Heimovski, Flavia Emilie; Simioni, Juliana A.; Skare, Thelma Larocca

    2015-01-01

    BACKGROUND Patients with systemic lupus erythematosus seem to belong to different serological and clinical subgroups of the disease. Genetic background can cause the appearance of these subgroups. OBJECTIVE To determine whether Brazilian patients who have systemic lupus erythematosus and Raynaud's phenomenon differ from those who do not. METHODS Retrospective analysis of 373 medical records of systemic lupus erythematosus patients studied for demographic, clinical and serological data. A comparative analysis was performed of individuals with and without RP. RESULTS There was a positive association between Raynaud's phenomenon and age at diagnosis (p=0.02), presence of anti-Sm (p=0.01) antibodies and anti-RNP (p<0.0001). Furthermore, a negative association was found between Raynaud's phenomenon and hemolysis (p=0.01), serositis (p=0.01), glomerulonephritis (p=0.0004) and IgM aCL (p=0.004) antibodies. CONCLUSION Raynaud's phenomenon patients appear to belong to a systemic lupus erythematosus subset with a spectrum of clinical manifestations located in a more benign pole of the disease. PMID:26734864

  20. Thyroid disorders in systemic lupus erythematosus.

    PubMed Central

    Goh, K L; Wang, F

    1986-01-01

    Of 319 patients with systemic lupus erythematosus (SLE), nine had thyrotoxicosis, three had hypothyroidism, and two had thyroiditis. This prevalence seems greater than that of similar thyroid disorders seen in the general population. It is suggested that patients with autoimmune thyroid disorders may develop SLE or vice versa. This association requires confirmation by prospective study. PMID:3740982

  1. Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus.

    PubMed Central

    Simeon-Aznar, C P; Cuenca-Luque, R; Fonollosa-Pla, V; Bosch-Gil, J A

    1992-01-01

    The case of a patient admitted with thrombotic thrombocytopenic purpura nine years after developing systemic lupus erythematosus (SLE) is reported. Thrombotic thrombocytopenic purpura associated with SLE has been described on other occasions, but in most patients the diagnosis of SLE precedes that of thrombotic thrombocytopenic purpura. The unusual sequence and the chronological separation of the two diseases is emphasised. PMID:1575591

  2. Apoptosis in chronic cutaneous lupus erythematosus, discoid lupus, and lupus profundus

    PubMed Central

    Sáenz-Corral, Claudia Ileana; Vega-Memíje, María Elisa; Martínez-Luna, Eduwiges; Cuevas-González, Juan Carlos; Rodríguez-Carreón, Alma Angélica; de la Rosa, Juan José Bollain-y-Goytia; del Muro, Felipe de Jesús Torres; Avalos-Díaz, Esperanza

    2015-01-01

    Introduction: Lupus erythematosus is a multisystemic disease that is characterized by autoantibody production and immune complex deposition in such tissues as the mucosa, joints, the central nervous system, and skin. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Chronic cutaneous lupus erythematosus comprises discoid lupus erythematosus (DLE) and lupus profundus (LP). Aim: To analyze the expression of proapoptotic molecules in patients with lupus erythematosus discoid and lupus profundus. Material and methods: Descriptive study, the study groups comprised 10 cases of LP and 10 cases of DLE, and a control. Skin samples of cases and controls were processed for immunohistochemistry and by TUNEL technique. The database and statistical analysis was performed (statistical test X2) SPSS (Chicago, IL, USA). Results: Apoptotic features were broadly distributed along the skin biopsies in epidermal keratinocytes as well as at dermis. By immunohistochemistry the expression of Fas receptor and Fas-L was higher in the skin of lupus patients compared with controls. We also noted differences in Fas-L, -Fas, and -Bax proteins expression intensity in discoid lupus erythematosus patients in the epidermis, and hair follicles. Conclusions: Fas and Fas-L are expressed similarly in LP and DLE. PMID:26261624

  3. Targeted therapies in systemic lupus erythematosus.

    PubMed

    Grech, P; Khamashta, Ma

    2013-09-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem disorder characterised by loss of tolerance to endogenous nuclear antigens and autoantibody formation. Recent insight into the immunopathogenesis of lupus has provided the foundation for a novel class of agents which target specific, dysregulated components of the immune system. Efforts have focused predominantly on B-cell depleting therapies, of which belimumab was the first to demonstrate success in phase III studies and thus receive marketing authorisation. Off-label prescribing of rituximab in refractory cases is common and supported by uncontrolled studies, which suggest a favourable risk:benefit profile. However, two placebo-controlled trials failed to show benefit, possibly because of inappropriate patient selection and other aspects of trial methodology. Inhibition of dysregulated co-stimulatory signals and cytokines are other therapeutic strategies currently under investigation. Some candidate drugs failed to meet primary endpoints in early-phase clinical trials, yet demonstrated clinical benefit when alternative assessment criteria were applied or specific patient sub-groups analysed. Well-designed studies of greater size and duration are needed to clarify the therapeutic utility of these agents. Future immunomodulatory strategies targeting interferon-alpha, T cells, oxidative stress and epigenetic abnormalities may reduce multisystem disease activity and prolong survival in this complex and heterogeneic disease. PMID:23963429

  4. Clinical outcome measures for Cutaneous Lupus Erythematosus

    PubMed Central

    Albrecht, Joerg; Werth, Victoria P.

    2011-01-01

    Cutaneous lupus erythematosus is a clinically heterogeneous group of rare skin diseases that only rarely have been subjected to controlled clinical trials. This may be have been partly due to a lack of suitable validated outcome instruments. Recently the FDA mandated that organ specific trials for lupus erythematosus need to use a combination of different outcome measures. The patient’s condition needs to be assessed in terms of quality of life, the patient’s global response and organ specific instruments that measure activity of the disease as well as damage due to the disease. For the skin the only formally validated and published instrument is currently the Cutaneous Lupus Erythematosis Disease Area and Severity Index (CLASI). This paper discusses the background of the development of the CLASI as well as issues related to its use and interpretation in the context of clinical research of CLE. PMID:20693208

  5. Successful treatment of facial systemic lupus erythematosus lesions with Dr Michaels® (Soratinex®) product family. A case report.

    PubMed

    Tirant, M; Bayer, P; Hercogovấ, J; Fioranelli, M; Gianfaldoni, S; Chokoeva, A A; Tchernev, G; Wollina, U; Novotny, F; Roccia, M G; Maximov, G K; França, K; Lotti, T

    2016-01-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease in which the body’s immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain and other organs. We report the case of a 7-year-old female patient with facial lesions of SLE since the age of 5. There was no significant family history and patient had been a healthy child from birth. The child presented with a malar rash, also known as a butterfly rash, with distribution over the cheeks but sparing the nasal bridge. This case represents the efficacy of the Dr. Michaels® (Soratinex®) product family in the successful resolution of facial lesions of SLE. PMID:27498665

  6. Libman-sacks endocarditis exclusively involving the tricuspid valve in a patient with systemic lupus erythematosus.

    PubMed

    Wang, Yonghuai; Ma, Chunyan; Yang, Jun; Liu, Shuang; Zhang, Yan; Zhao, Lanting; Guan, Zhengyu; Wei, Hong; Gu, Tianxiang

    2014-06-13

    Libman-Sacks endocarditis, characterized by sterile verrucous vegetations, is a rare but typical cardiac manifestation of systemic lupus erythematosus. It primarily leads to lesions of the mitral and aortic valves, but isolated tricuspid valve involvement is exceptional. We report the case of a 40-year-old woman with large tricuspid valve vegetations, thickening, and regurgitation. Clinical findings and laboratory tests confirmed the diagnosis of systemic lupus erythematosus. The patient successfully recovered following tricuspid valve replacement. Echocardiography is the definitive imaging modality for assessing cardiac valvular involvement, choosing appropriate therapy, and evaluating the prognosis of Libman-Sacks endocarditis in systemic lupus erythematosus. © 2014 Wiley Periodicals, Inc. J Clin Ultrasound, 2014.

  7. Topical calcineurin inhibitors in systemic lupus erythematosus

    PubMed Central

    Lampropoulos, Christos E; D’Cruz, David P

    2010-01-01

    Cutaneous lupus erythematosus (CLE) encompasses a variety of lesions that may be refractory to systemic or topical agents. Discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE) are the most common lesions in clinical practice. The topical calcineurin inhibitors, tacrolimus and pimecrolimus, have been used to treat resistant cutaneous lupus since 2002 and inhibit the proliferation and activation of T-cells and suppress immune-mediated cutaneous inflammation. This article reviews the mechanism of action, efficacy, adverse effects, and the recent concern about their possible carcinogenic effect. Although the total number of patients is small and there is only one relevant randomized controlled study, the data are encouraging. Many patients, previously resistant to systemic agents or topical steroids, improved after four weeks of treatment. DLE and SCLE lesions were less responsive, reflecting the chronicity of the lesions, although more than 50% of patients still showed improvement. Topical calcineurin inhibitors may be a safe and effective alternative to topical steroids for CLE although the only approved indication is for atopic dermatitis. PMID:20421909

  8. Spontaneous ureteral rupture in a patient with systemic lupus erythematosus

    SciTech Connect

    Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.

    1983-08-01

    A patient with known systemic lupus erythematosus had fever and symptoms of a lower urinary tract infection. Bone scintigraphy showed left ureteral perforation and necrosis with no demonstrable nephrolithiasis. It is speculated that this episode was due to lupus vasculitis.

  9. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies.

    PubMed

    Schreiber, K

    2016-04-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial advice against pregnancy in the 1950s has been replaced by a common understanding that women with SLE often have successful pregnancy outcomes, and clinicians therefore advise on pregnancy planning, including possible drug adjustments, timing and close surveillance. The recently published Predictors of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from the PROMISSE cohort will hopefully identify serological biomarkers, possibly genes, and in addition, give valuable information about underlying disease mechanisms.

  10. Systemic lupus erythematosus: Is it one disease?

    PubMed

    Rivas-Larrauri, Francisco; Yamazaki-Nakashimada, Marco Antonio

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with a variety of clinical presentations. Monogenic predisposing conditions to the development of this disease have been described. As examples, an impaired expression of interferon-α regulated genes or complement deficiencies have been reported in patients with SLE, with particular clinical presentations. Those defects present particular presentations and a different severity, making an argument that lupus is not a single disease but many. Treatment could be individualized depending on the underlying defect generating the subtype of the disease.

  11. Anastrozole-induced subacute cutaneous lupus erythematosus.

    PubMed

    Fisher, Juliya; Patel, Mital; Miller, Michael; Burris, Katy

    2016-08-01

    Drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) has been associated with numerous drugs, but there are limited reports of its association with aromatase inhibitor anastrozole. We report the case of a patient undergoing treatment with anastrozole for breast cancer who presented with clinical, serological, and histological evidence consistent with DI-SCLE. Her condition quickly began to improve after the use of anastrozole was discontinued and hydroxychloroquine therapy was initiated. Cases such as ours as well as several others that implicate antiestrogen drugs in association with DI-SCLE seem to be contradictory to studies looking at the usefulness of treating systemic lupus erythematosus (SLE) with antiestrogen therapy. Further research on this relationship is warranted. PMID:27622265

  12. Anastrozole-induced subacute cutaneous lupus erythematosus.

    PubMed

    Fisher, Juliya; Patel, Mital; Miller, Michael; Burris, Katy

    2016-08-01

    Drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) has been associated with numerous drugs, but there are limited reports of its association with aromatase inhibitor anastrozole. We report the case of a patient undergoing treatment with anastrozole for breast cancer who presented with clinical, serological, and histological evidence consistent with DI-SCLE. Her condition quickly began to improve after the use of anastrozole was discontinued and hydroxychloroquine therapy was initiated. Cases such as ours as well as several others that implicate antiestrogen drugs in association with DI-SCLE seem to be contradictory to studies looking at the usefulness of treating systemic lupus erythematosus (SLE) with antiestrogen therapy. Further research on this relationship is warranted.

  13. Eosinophilic gastroenteritis associated with systemic lupus erythematosus.

    PubMed

    Barbie, David A; Mangi, Abeel A; Lauwers, Gregory Y

    2004-01-01

    Eosinophilic gastroenteritis is an uncommon disease with an obscure etiology, although associations with allergy, the idiopathic hypereosinophilic syndrome, and connective tissue disease have been reported. We present the case of a 37-year-old woman with a history of idiopathic thrombocytopenic purpura who presented with refractory nausea, vomiting, and abdominal pain. Imaging studies were significant for bowel wall thickening and ascites, while laboratory studies revealed a positive antinuclear antibody (ANA), a positive anti-double stranded (DS) DNA antibody, low complement, and proteinuria. Exploratory laparotomy with gastric and small bowel biopsies established the diagnosis of eosinophilic gastroenteritis. In addition, the patient met clinical criteria for the diagnosis of systemic lupus erythematosus. Previous studies have described eosinophilic gastroenteritis in patients with scleroderma, polymyositis, or dermatomyositis. This is the first report to our knowledge of an individual with eosinophilic gastroenteritis and systemic lupus erythematosus. PMID:15492606

  14. Clinical characteristics of cutaneous lupus erythematosus

    PubMed Central

    Szczęch, Justyna; Rutka, Maja; Samotij, Dominik; Zalewska, Agnieszka

    2016-01-01

    Introduction Lupus erythematosus (LE) shows a wide variety of clinical manifestations, skin involvement being one of the most important. Aim To analyze the clinical presentation of cutaneous variants of lupus erythematosus in terms of skin lesion spectrum and extracutaneous involvement. Material and methods A total of 64 patients with cutaneous LE (CLE) were included. The study was based on the “Core Set Questionnaire” developed by the European Society of Cutaneous Lupus Erythematosus (EUSCLE). Clinical severity of skin lesions was evaluated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). All results were subjected to statistical analysis. Results Fifteen (23.4%) patients had an acute CLE (ACLE), 26 (40.6%) subacute CLE (SCLE) and 21 (32.8%) chronic CLE (CCLE). Two (3.2%) individuals only demonstrated urticarial vasculitis as a cutaneous manifestation of LE and these patients were excluded. Patients with ACLE were characterized by the earliest onset of the disease (mean age of 31.9 ±15.0 years; p < 0.001). On average, 4.8 ±1.8 criteria of systemic LE were found in the ACLE group compared to 2.7 ±1.3 criteria in SCLE and 2.5 ±1.5 criteria in CCLE (p < 0.001). The highest activity of skin lesions according to CLASI was found in the SCLE group (p = 0.002). On the other hand, the most severe skin damage was observed in CCLE (p < 0.01). Conclusions Each variant of CLE differs significantly from the others in respect of various aspects of clinical manifestations. Due to a number of different variants of LE skin lesions, a unified classification of CLE still remains a challenge. PMID:26985173

  15. What is new in systemic lupus erythematosus.

    PubMed

    Rúa-Figueroa Fernández de Larrinoa, Iñigo

    2015-01-01

    Systemic lupus erythematosus is a heterogeneous rheumatic systemic disease with extremely varied clinical manifestations and a diverse pathogenesis, as illustrated in this review on the most relevant new knowledge related to the disease. Topics such as anemia, pathogenesis, cardiovascular risk assessment, antiphospholipid syndrome, prediction of damage and recent advances in treatment, including tolerogenic and biological agents, are discussed. Relevant contributions regarding classical therapies such as corticosteroid and antimalarials and their optimal use, as well as the roll of vitamin D, are also referred.

  16. Systemic Lupus Erythematosus in Children and Adolescents

    PubMed Central

    Levy, Deborah M.; Kamphuis, Sylvia

    2012-01-01

    Synopsis Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system with a wide range of disease manifestations, and can lead to significant morbidity and even mortality. This article reviews the epidemiology, common clinical features, complications of disease, and briefly discusses the available treatment options. In addition, important medical and psychosocial issues relevant to the pediatrician caring for children and adolescents with SLE are discussed. PMID:22560574

  17. Vaccination, atherosclerosis and systemic lupus erythematosus.

    PubMed

    Carvalho, J F; Pereira, R M R; Shoenfeld, Y

    2009-11-01

    Atherosclerosis is an inflammatory disease, leading to the formation of pro-inflammatory and pro-oxidative lipids that generate an immune response. Several antigens have been shown to activate the immune response and affect the development of atherogenesis. Systemic lupus erythematosus is an autoimmune and inflammatory disease strongly associated with premature development of atherosclerotic plaques. Modulation of the immune system could represent a useful approach to prevent and/or treat atherosclerosis. A vaccination-based approach might be a useful, effective tool in the modern arsenal of cardiovascular therapies and could be used on a large scale at a low cost. In non-systemic lupus erythematosus populations, vaccines against oxidized low-density lipoprotein, beta-2-glycoprotein I, heat shock proteins, lipoproteins, cholesterol, molecules involved in cholesterol metabolism, and other molecules (CD99, vascular endothelial growth factor-receptor, and interleukin-2) have been tested, with promising results. However, there are no studies of vaccination against atherosclerosis in systemic lupus erythematosus.

  18. Lupus Panniculitis as an Initial Manifestation of Systemic Lupus Erythematosus

    PubMed Central

    Zhao, Yu-Kun; Wang, Fang; Chen, Wen-Na; Xu, Rui; Wang, Zhuo; Jiang, Yuan-Wen; Luo, Di-Qing; Han, Jian-De

    2016-01-01

    Abstract Lupus erythematosus panniculitis (LEP) is a variant of chronic cutaneous lupus erythematosus (CCLE). Reported cases of LEP lesions before the diagnosis of systemic lupus erythematosus (SLE) were very rare; only 9 cases have been reported, to the best of our knowledge. We now describe the case of a 19-year-old male patient, with an overall review of the English literature. In the earliest stage of the present case, nodules and ulcers involved his left leg and face, with no other accompanied symptoms. The skin lesions disappeared after treatment with methylprednisolone, 16 mg/d for 1 month. Seven months after discontinuing methylprednisolone, the cutaneous nodules and ulcers on his back recurred and were accompanied by fever, hair loss, and polyarthritis. Blood tests revealed leucopenia, positive antinuclear antibody and Smith antibody, and proteinuria. Histopathological findings were most consistent with LEP. This was followed sequentially by the diagnosis of SLE. The patient improved again after treatment with methylprednisolone and cyclophosphamide. Patients with LEP should have regular follow-ups because the development of SLE is possible. Early diagnosis and proper treatment is pivotal to improve the prognosis of such patients. PMID:27100438

  19. Premature vascular damage in systemic lupus erythematosus.

    PubMed

    Kaplan, Mariana J

    2009-11-01

    Systemic lupus erythematosus (SLE) is a disease associated with a striking increase in the risk of premature cardiovascular (CV) complications due to accelerated atherosclerosis. Traditional CV risk factors seem to be less important predictors of CV events than the presence of active SLE. Immune dysregulation characteristic of lupus appears to play the dominant role in atherogenesis. While both SLE-specific and non-specific mechanisms have been proposed to play a prominent role in the induction of premature vascular damage in this disease, the exact etiology remains unclear. We have proposed that an imbalance between vascular damage and repair likely induced by Interferon- could play a prominent role in the induction of accelerated atherosclerosis in SLE. This review summarizes some of the proposed mechanisms that may promote accelerated vascular damage in lupus and explores potential targets for CV risk prevention in this patient population.

  20. Chronic aseptic meningitis in a patient with systemic lupus erythematosus.

    PubMed

    Lancman, M E; Mesropian, H; Granillo, R J

    1989-08-01

    Chronic aseptic meningitis is a rare manifestation of systemic lupus erythematosus. It may occur early in the course of the disease and sometimes may be the initial symptom. We report a patient with chronic aseptic meningitis associated with systemic lupus erythematosus. Magnetic resonance imaging showed several ischemic lesions and an appearance which was compatible with chronic inflammation of the ependyma of the lateral ventricles.

  1. Bullous Systemic Lupus Erythematosus: Case report

    PubMed Central

    Miziara, Ivan Dieb; Mahmoud, Ali; Chagury, Azis Arruda; Alves, Ricardo Dourado

    2013-01-01

    Summary Introduction: Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. Case Report: A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. She sought advice from emergency dermatology staff 15 days after onset and was hospitalized with suspected bullous disease. Intravenous antibiotics and steroids were administered initially, but the patient showed no improvement during hospitalization. She displayed further extensive injuries to the trunk, axillae, and vulva as well as disruption of the bullous lesions, which remained as hyperemic scars. Incisional biopsy of a lesion in the left buccal mucosa was performed, and pathological results indicated mucositis with extensive erosion and the presence of a predominantly neutrophilic infiltrate with degeneration of basal cells and apoptotic keratinocytes. Under direct immunofluorescence, the skin showed anti-IgA, anti-IgM, and anti-IgG linear fluorescence on the continuous dermal side of the cleavage. Indirect immunofluorescence of the skin showed conjugated anti-IgA, was anti-IgM negative, and displayed pemphigus in conjunction with anti-IgG fluorescence in the nucleus of keratinocytes, consistent with a diagnosis of bullous lupus erythematosus. Discussion: BSLE is an acquired autoimmune bullous disease caused by autoantibodies against type VII collagen or other components of the junctional zone, epidermis, and dermis. It must be differentiated from the secondary bubbles and vacuolar degeneration of the basement membrane that may occur in acute and subacute cutaneous lupus erythematosus. PMID:25992032

  2. Peptide-based immunotherapy of systemic lupus erythematosus.

    PubMed

    Monneaux, Fanny; Muller, Sylviane

    2004-01-01

    Current drug-based therapy for systemic lupus erythematosus (SLE) are non-specific and often counterbalanced by adverse effects. Current research aims at developing specific treatments that target deleterious cells only and not the whole immune system. This strategy requires the identification of sequences derived from major lupus autoantigens, responsible for the activation of autoreactive B and T cells. This review summarizes the identification and characterization of peptides, which are able to modulate T cells ex vivo, and describes the promising results obtained after administration of some of these peptides in lupus mice. Although these therapeutic trials are encouraging, the precise mode of action of peptide-based immunotherapy is still elusive. Here, we discuss the possible mechanisms leading to T-cell tolerance induction and the feasibility of extending the success of peptide-based therapy from animal models to human.

  3. Hydroxychloroquine and pregnancy on lupus flares in Korean patients with systemic lupus erythematosus.

    PubMed

    Koh, J H; Ko, H S; Kwok, S-K; Ju, J H; Park, S-H

    2015-02-01

    We investigated the clinical and laboratory characteristics of pregnancies with systemic lupus erythematosus (SLE) and identified lupus flare predictors during pregnancy. Additionally, we examined lupus activity and pregnancy outcomes in SLE patients who continued, discontinued or underwent no hydroxychloroquine (HCQ) treatment during pregnancy. We retrospectively analyzed 179 pregnancies in 128 SLE patients at Seoul St. Mary's Hospital, Korea, between 1998 and 2012 and then assessed the clinical profiles and maternal and fetal outcomes. Overall, 90.5% of pregnancies resulted in a successful delivery and were divided into two groups: those who experienced lupus flares (80 pregnancies, 44.7%) and those who did not (99 pregnancies, 55.3%). Increased preeclampsia, preterm births, low birth weight, intrauterine growth restriction (IUGR), and low 1-minute Apgar scores occurred in pregnancies with lupus flares compared to pregnancies in quiescent disease. Lupus flares were predicted by HCQ discontinuation, a history of lupus nephritis, high pre-pregnancy serum uric acid and low C4 levels. Our study indicates that achieving pre-pregnancy remission and continuing HCQ treatment during pregnancy are important for preventing lupus flares.

  4. Cytokine disturbances in systemic lupus erythematosus.

    PubMed

    Jacob, Noam; Stohl, William

    2011-07-06

    The pathogenesis of systemic lupus erythematosus (SLE) is complex, and the resulting disease manifestations are heterogeneous. Cytokine dysregulation is pervasive, and their protein and gene expression profiles may serve as markers of disease activity and severity. Importantly, biologic agents that target specific cytokines may represent novel therapies for SLE. Four cytokines (IL-6, TNFα, IFNα, and BLyS) are being evaluated as therapeutic targets in SLE. The present review will examine the roles of each of these cytokines in murine and human SLE, and will summarize results from clinical trials of agents that target these cytokines.

  5. Systemic lupus erythematosus: Clinical and experimental aspects

    SciTech Connect

    Smolen, J.S.

    1987-01-01

    This text covers questions related to the history, etiology, pathogenesis, clinical aspects and therapy of systematic lupus erythematosus (SLE). Both animal models and human SLE are considered. With regard to basic science, concise information on cellular immunology, autoantibodies, viral aspects and molecular biology in SLE is provided. Clinical topics then deal with medical, dermatologic, neurologic, radiologic, pathologic, and therapeutic aspects. The book not only presents the most recent information on clinical and experimental insights, but also looks at future aspects related to the diagnosis and therapy of SLE.

  6. Acquired enophthalmos with systemic lupus erythematosus.

    PubMed

    Park, K R; Seo, M R; Ryu, H J; Chi, M J; Baek, H J; Choi, H J

    2016-01-01

    Ocular involvement sometimes occurs with systemic lupus erythematosus (SLE) but enophthalmos with SLE is rare. We report a case of enophthalmos with SLE. A 25-year-old male was admitted for two weeks of fever, sore throat, arthralgia, chest pain and right arm weakness with pain. We diagnosed him with SLE with malar rash, arthritis, pleural effusion, proteinuria, leukopenia, positive antinuclear antibody, anti-dsDNA, and lupus anticoagulant. The patient was prescribed high-dose prednisolone and hydroxychloroquine 400 mg. One week after discharge, he complained about a sensation of a sunken right eye. CT showed right enophthalmos, a post-inflammatory change and chronic inflammation. Proteinuria increased to 3.8 g/day after the patient stopped taking prednisolone. Cyclophosphamide therapy was administered for three months without improvement. We decided to restart prednisolone and change cyclophosphamide to mycophenolate mofetil. Proteinuria decreased but enophthalmos remains as of this reporting.

  7. Ultraviolet radiation and systemic lupus erythematosus.

    PubMed

    Barbhaiya, M; Costenbader, K H

    2014-05-01

    Exposure to ultraviolet (UV) radiation is among the environmental factors that have been proposed and studied in association with systemic lupus erythematosus (SLE). While it is known that UV radiation exposure may exacerbate pre-existing lupus, it remains unclear whether UV exposure is a risk factor for the development of SLE. Experimental studies show a significant immunomodulatory role for UV radiation, but strong epidemiologic data regarding its role in triggering SLE onset are lacking. Further studies are needed to assess the role of UV radiation in relation to development of incident SLE, yet they are challenging to design due to difficulties in accurate exposure assessment, the heterogeneous nature of SLE, and the challenge of assessing photosensitivity, a feature of SLE, which often precedes its diagnosis.

  8. Environmental Factors, Toxicants and Systemic Lupus Erythematosus

    PubMed Central

    Mak, Anselm; Tay, Sen Hee

    2014-01-01

    Systemic lupus erythematosus (SLE) is an immune-complex-mediated multi-systemic autoimmune condition of multifactorial etiology, which mainly affects young women. It is currently believed that the onset of SLE and lupus flares are triggered by various environmental factors in genetically susceptible individuals. Various environmental agents and toxicants, such as cigarette smoke, alcohol, occupationally- and non-occupationally-related chemicals, ultraviolet light, infections, sex hormones and certain medications and vaccines, have been implicated to induce SLE onset or flares in a number case series, case-control and population-based cohort studies and very few randomized controlled trials. Here, we will describe some of these recognized environmental lupus triggering and perpetuating factors and explain how these factors potentially bias the immune system towards autoimmunity through their interactions with genetic and epigenetic alterations. Further in-depth exploration of how potentially important environmental factors mechanistically interact with the immune system and the genome, which trigger the onset of SLE and lupus flares, will certainly be one of the plausible steps to prevent the onset and to decelerate the progress of the disease. PMID:25216337

  9. [Systemic lupus erythematosus presenting as Stevens-Johnson syndrome].

    PubMed

    Bellakhal, S; Ben Kaab, B; Teyeb, Z; Souissi, A; Derbel, F; Douggui, M-H

    2015-09-01

    Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening dermatological conditions. Their most common cause is medication. However, in a small proportion of patients these dermatological conditions could be the first presentation of systemic lupus erythematosus. We now describe a 34-year-old patient who presented with manifestations of Stevens-Johnson as a first feature of systemic lupus erythematosus. Systemic lupus erythematosus reveled by Stevens-Johnson syndrome has been infrequently reviewed in the previous literature. This diagnosis should be considered when cutaneous adverse drug reactions occur without clear drug causality.

  10. Childhood-onset systemic lupus erythematosus.

    PubMed Central

    Olowu, Wasiu

    2007-01-01

    OBJECTIVES: To describe the initial clinicolaboratory manifestations and short-term outcome in a series of Nigerian children with systemic lupus erythematosus (SLE). METHODS: A nonrandomized prospective study of consecutive cases of childhood-onset SLE. Baseline and follow-up clinicolaboratory data were collected and analyzed. Each patient was followed up for 12 months. RESULTS: Eleven children were studied. There were seven girls (F:M, 1.75). Mean ages at lupus onset and diagnosis were 10.0 +/- 2.53 years and 11.2 +/- 2.53 years, respectively. Mean time at onset of renal disease following SLE symptoms onset was 1.22 +/- 0.93 years. All cases were misdiagnosed prior to presentation; diagnosis was delayed in nine patients. Lupus activity was mild, moderate and severe in two, five and four patients, respectively. Hypertension (n = 5), nephrotic syndrome (n = 6), microerythrocyturia (n = 6) and acute renal failure (n = 7) were associated morbidities. Of the 27 presenting clinical features, 17 were nondiagnostic, while 10 were diagnostic. Fever (n = 9) was a major nondiagnostic symptom; major diagnostic manifestations were lupus nephritis (n = 11), arthritis (n = 10) and serositis (n = 7). Catastrophic antiphospholipid syndrome was diagnosed in three. The glomerular lesions were nonproliferative (n = 1), focal (n = 3) and diffuse (n = 7) proliferative lupus nephritis. Complete remission rate at end-point was 71.4%. Fourteen percent of the patients relapsed. Renal survival and mortality rates were 86.0% and 30.0%, respectively. CONCLUSION: In this study, severe renal and extrarenal comorbidities were common; mortality rate was also high. High frequency of misdiagnosis and delayed diagnosis were probably responsible for these. PMID:17668644

  11. Elevated sacroilac joint uptake ratios in systemic lupus erythematosus

    SciTech Connect

    De Smet, A.A.; Mahmood, T.; Robinson, R.G.; Lindsley, H.B.

    1984-08-01

    Sacroiliac joint radiographs and radionuclide sacroiliac joint uptake ratios were obtained on 14 patients with active systemic lupus erythematosus. Elevated joint ratios were found unilaterally in two patients and bilaterally in seven patients when their lupus was active. In patients whose disease became quiescent, the uptake ratios returned to normal. Two patients had persistently elevated ratios with continued clinical and laboratory evidence of active lupus. Mild sacroiliac joint sclerosis and erosions were detected on pelvic radiographs in these same two patients. Elevated quantitative sacroiliac joint uptake ratios may occur as a manifestation of active systemic lupus erythematosus.

  12. Acquired hemophilia A in a patient with systemic lupus erythematosus.

    PubMed

    Ishikawa, T; Tsukamoto, N; Suto, M; Uchiumi, H; Mitsuhashi, H; Yokohama, A; Maesawa, A; Nojima, Y; Naruse, T

    2001-06-01

    A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical. PMID:11446683

  13. Toxocara canis infection: Unusual trigger of systemic lupus erythematosus.

    PubMed

    Levy, Michaël; Bourrat, Emmanuelle; Baudouin, Véronique; Guillem, Colette; Peuchmaur, Michel; Deschênes, Georges; Fila, Marc

    2015-08-01

    Infection by Toxocara canis can cause systemic vasculitis. We report here a unique case of systemic lupus erythematosus (SLE) triggered by T. canis infection. An 8-year-old girl was treated with albendazole therapy for common toxocariasis, but she developed two weeks later, asthenia, fever, infiltrated maculopapular eruption of the face, peripheral vascular disease with necrosis of the fingers and inflammatory anemia with proteinuria. Anti-nuclear, anti-DNA and anti-Sm antibodies positivity, together with minimal change nephritis with mesangial exclusive IgM deposit on renal biopsy and clinical relapse after initially successful steroid therapy, led to the diagnosis of SLE. T. canis infection can trigger systemic lupus but must also be ruled out of the differential diagnosis given its association with autoimmunity. PMID:26147636

  14. Thrombosis in Systemic Lupus Erythematosus: A Review Article

    PubMed Central

    Al-Homood, Ibrahim A.

    2012-01-01

    Thrombosis is a well-known clinical entity in systemic lupus erythematosus (SLE), and it is multifactorial. The most important risk factor is the presence of antiphospholipid antibodies (APLAs). However, approximately 40% of adults with SLE who are negative for APL A are diagnosed with thrombosis, indicating the importance of other risk factors. Thus, the thrombosis risk factors should be evaluated extensively and regularly and treated aggressively in every patient with systemic lupus erythematosus. PMID:22900201

  15. Update on systemic lupus erythematosus pregnancy

    PubMed Central

    Iozza, Irene; Cianci, Stefano; Di Natale, Angela; Garofalo, Giovanna; Giacobbe, Anna Maria; Giorgio, Elsa; De Oronzo, Maria Antonietta; Politi, Salvatore

    2010-01-01

    Women with Systemic Lupus Erythematosus (SLE) still face significant risks when embarking on a pregnancy. Improvements in the field of pathophysiology, in diagnosis and a greater number of therapeutic options in the treatment of SLE, have made the medical community regard these patients with less trepidation. Despite these advances, however, the risk of significant morbidity to both the mother and the fetus still exists. The interaction of lupus and pregnancy is very complex: the consensus is that pregnancy can worsen the lupus disease process, even if this is not predictable, and pregnancy can mimic the clinical manifestations of lupus, particularly preeclampsia/eclampsia. More specifically, pregnancy is associated in 50 to 60% of cases with a clinical flare manifesting as renalor hematological symptoms. Severe flares are uncommon (10%) and the risk of maternal death is now2 to 3%. The risk of the fetus remains high, however with increased risk of spontaneous fetal wastage and premature births, by 4.8 and 6.8 times, respectively. It is well documented that antiphospholipid syndrome and antiphospholipid antibodies are strongly associated with fetal wastage. Low-dose aspirin orheparin improves fetal outcome in these cases. Timing a pregnancy to coincide with a period of disease quiescence for at least 6 months strongly increases the chances for a healthy and uneventful pregnancy for both mother and baby. Close surveillance, with monitoring of blood pressure, proteinuria and placental blood flow by doppler studies helps the early diagnosis and treatment of complications such as preeclampsia andfoetal distress. Women with SLE frequently need treatment throughout pregnancy based on hydroxychloroquine, lowdose steroids and azathioprine. This update, based on previous available literature, should inform rheumatologists, obstetricians and neonatologists who guide patients in their reproductive decisions. PMID:22439065

  16. Lupus erythematosus: considerations about clinical, cutaneous and therapeutic aspects*

    PubMed Central

    Moura Filho, Jucélio Pereira; Peixoto, Raiza Luna; Martins, Lívia Gomes; de Melo, Sillas Duarte; de Carvalho, Ligiana Leite; Pereira, Ana Karine F. da Trindade C.; Freire, Eutilia Andrade Medeiros

    2014-01-01

    Systemic Lupus Erythematosus is a chronic inflammatory disease with multifactorial etiology. Although clinical manifestations are varied, the skin is an important target-organ, which contributes to the inclusion of skin lesions in 4 out of the 17 new criteria for the diagnosis of the disease, according to the Systemic Lupus International Collaborating Clinics. The cutaneous manifestations of lupus are pleomorphic. Depending on their clinical characteristics, they can be classified into Acute Cutaneous Lupus Erythematosus, Subacute Cutaneous Lupus Erythematosus, Chronic Cutaneous Lupus Erythematosus and Intermittent Cutaneous Lupus Erythematosus. Treatment is based on preventive measures, reversal of inflammation, prevention of damage to target organs and relief of adverse events due to pharmacological therapy. The most commonly used treatment options are topical, systemic and surgical treatment, as well as phototherapy. The correct handling of the cases depends on a careful evaluation of the morphology of the lesions and the patient's general status, always taking into consideration not only the benefits but also the side effects of each therapeutic proposal. PMID:24626656

  17. Neuropsychiatric Systemic Lupus Erythematosus: A Diagnostic Conundrum

    PubMed Central

    Joseph, Vivek; Anil, Rahul; Aristy, Sary

    2016-01-01

    A 70-year-old man presented with complaints of rapid cognitive decline and new onset leukopenia. The patient had a 17-year history of refractory seizures. Detailed review of symptoms and investigations revealed the patient met American College of Rheumatology (ACR) diagnostic criteria for systemic lupus erythematosus (SLE). The patient had high titer ANA with a strongly positive dsDNA. Immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil led to significant improvement in cognition and seizures. Neuropsychiatric SLE should be considered a potential differential diagnosis for patients presenting with seizures or cognitive decline. Moreover, neuropsychiatric manifestations especially seizures are an early event in the disease course of SLE. Hence, we believe that early diagnosis of SLE by neuropsychiatric manifestations will not only lead to better control of CNS symptoms but early immunosuppressive therapy could control the progression of the underlying autoimmune disease. PMID:27635183

  18. Neuropsychiatric Systemic Lupus Erythematosus: A Diagnostic Conundrum

    PubMed Central

    Joseph, Vivek; Anil, Rahul; Aristy, Sary

    2016-01-01

    A 70-year-old man presented with complaints of rapid cognitive decline and new onset leukopenia. The patient had a 17-year history of refractory seizures. Detailed review of symptoms and investigations revealed the patient met American College of Rheumatology (ACR) diagnostic criteria for systemic lupus erythematosus (SLE). The patient had high titer ANA with a strongly positive dsDNA. Immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil led to significant improvement in cognition and seizures. Neuropsychiatric SLE should be considered a potential differential diagnosis for patients presenting with seizures or cognitive decline. Moreover, neuropsychiatric manifestations especially seizures are an early event in the disease course of SLE. Hence, we believe that early diagnosis of SLE by neuropsychiatric manifestations will not only lead to better control of CNS symptoms but early immunosuppressive therapy could control the progression of the underlying autoimmune disease.

  19. New therapies for systemic lupus erythematosus

    PubMed Central

    Goldblatt, F; Isenberg, D A

    2005-01-01

    In the past 40 years, prognosis for patients with systemic lupus erythematosus (SLE) has improved, with 10-year survival now approximately 90%. This is due probably to a combination of earlier disease diagnosis and diagnosis of milder disease, due in part to availability of multiple serological tests for SLE, use of steroids and other immunosuppressive agents, and availability of renal dialysis and transplantation. Despite this, however, the potential for significant morbidity and mortality remains in the group of patients with partially responsive or treatment resistant disease. More recently, advancements in the understanding of molecular mechanisms involved in the pathogenesis of SLE have translated to the development of novel therapies, offering possible alternatives to this patient cohort. Discussion of these pharmacological options and ongoing research forms the basis of this review. PMID:15807843

  20. New biologic therapy for systemic lupus erythematosus.

    PubMed

    Ding, Hui Jen; Gordon, Caroline

    2013-06-01

    Systemic lupus erythematosus (SLE) is a heterogenous multi-systemic autoimmune disease that is associated with considerable morbidity and mortality. Rituximab is one of the earliest biologic therapies used in SLE. It performed well in off-label studies but failed to demonstrate efficacy in randomised controlled trials. Abatacept is a biologic developed for inflammatory arthritis but has shown promise in SLE. Belimumab is the first biologically approved therapy in fifty years for treatment of SLE. The development of biological therapies for SLE parallels the increasing understanding of the immunopathogenesis of SLE and looks promising. New drugs in development are those targeting the co-stimulatory modulation, cytokines and the B and T cells. Of interest are epratuzumab, the interferon antagonists and peptide-based therapies.

  1. Systemic lupus erythematosus: An update for ophthalmologists.

    PubMed

    Papagiannuli, Efrosini; Rhodes, Benjamin; Wallace, Graham R; Gordon, Caroline; Murray, Philip I; Denniston, Alastair K

    2016-01-01

    Systemic lupus erythematosus (SLE) is a life-threatening multisystem inflammatory condition that may affect almost any part of the eye. We provide an update for the practicing ophthalmologist comprising a systematic review of the recent literature presented in the context of current knowledge of the pathogenesis, diagnosis, and treatment of this condition. We review recent advances in the understanding of the influence of genetic and environmental factors on the development of SLE. Recent changes in the diagnostic criteria for SLE are considered. We assess the potential for novel molecular biomarkers to find a clinical application in disease diagnosis and stratification and in the development of therapeutic agents. We discuss limited forms of SLE and their differentiation from other collagen vascular disorders and review recent evidence underlying the use of established and novel therapeutics in this condition, including specific implications regarding monitoring for ocular toxicity associated with antimalarials.

  2. Neuropsychiatric Systemic Lupus Erythematosus: A Diagnostic Conundrum.

    PubMed

    Joseph, Vivek; Anil, Rahul; Aristy, Sary

    2016-10-01

    A 70-year-old man presented with complaints of rapid cognitive decline and new onset leukopenia. The patient had a 17-year history of refractory seizures. Detailed review of symptoms and investigations revealed the patient met American College of Rheumatology (ACR) diagnostic criteria for systemic lupus erythematosus (SLE). The patient had high titer ANA with a strongly positive dsDNA. Immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil led to significant improvement in cognition and seizures. Neuropsychiatric SLE should be considered a potential differential diagnosis for patients presenting with seizures or cognitive decline. Moreover, neuropsychiatric manifestations especially seizures are an early event in the disease course of SLE. Hence, we believe that early diagnosis of SLE by neuropsychiatric manifestations will not only lead to better control of CNS symptoms but early immunosuppressive therapy could control the progression of the underlying autoimmune disease. PMID:27635183

  3. Breaking Immunological Tolerance in Systemic Lupus Erythematosus

    PubMed Central

    Pieterse, Elmar; van der Vlag, Johan

    2014-01-01

    Systemic lupus erythematosus (SLE) is a fairly heterogeneous autoimmune disease of unknown etiology that mainly affects women in the childbearing age. SLE is a prototype type III hypersensitivity reaction in which immune complex depositions cause inflammation and tissue damage in multiple organs. Two distinct cell death pathways, apoptosis and NETosis, gained a great deal of interest among scientists, since both processes seem to be deregulated in SLE. There is growing evidence that histone modifications induced by these cell death pathways exert a central role in the induction of autoimmunity. In the current review, we discuss how abnormalities in apoptosis, NETosis, and histone modifications may lead to a break of immunological tolerance in SLE. PMID:24782867

  4. [A case of systemic lupus erythematosus complicated with psoriasis vulgaris].

    PubMed

    Shidara, Kumi; Soejima, Makoto; Shiseki, Mariko; Ohta, Syuji; Nishinarita, Makoto

    2003-12-01

    A 49-years-old female admitted to our hospital because of skin eruptions on the extremities in 1985. She had suffered from polyarthralgia, skin eruptions since 1983. Physical examinations revealed discoid lesion, central nervous system involvement, and polyarthritis. Laboratory tests revealed leukopenia, thrombocytopenia, and hypocomplementemia. Antinuclear antibody, ant-DNA antibody, LE test were positive. From these findings, she was diagnosed as systemic lupus erythematosus (SLE). She developed lupus peritonitis in 1990 and 1994, which was successfully treated by steroid pulse therapy. Since then, the activity of SLE was in good control under administration of prednisolone 10 mg/day. Chilblain lupus was seen from 1993, Raynaud's phenomenon from 1996, and she further developed subcutaneous induration on her chest, back and upper extremities in 1999. Skin biopsy findings were compatible with lupus panniculitis. In 2002, erythematous patches with scales were observed on her right hand and left knee, and these skin lesions were histologically diagnosed as psoriasis vulgaris. An autoimmune response similar to SLE is speculated in psoriasis. We describe a rare case of SLE with various skin lesions including psoriasis vulgaris.

  5. Lupus erythematosus--a case of facial swelling.

    PubMed

    Loescher, A; Edmondson, H D

    1988-04-01

    A case is reported of acute facial swelling following tooth extraction that failed to respond in a normal manner. The patient developed systemic signs and symptoms ultimately revealing the diagnosis of lupus erythematosus. The possibility of soft tissue lesions arising in some forms of lupus is emphasised by this report. PMID:3163493

  6. T-cell-directed therapies in systemic lupus erythematosus.

    PubMed

    Nandkumar, P; Furie, R

    2016-09-01

    Drug development for the treatment of systemic lupus erythematosus (SLE) has largely focused on B-cell therapies. A greater understanding of the immunopathogenesis of SLE coupled with advanced bioengineering has allowed for clinical trials centered on other targets for SLE therapy. The authors discuss the benefits and shortcomings of focusing on T-cell-directed therapies in SLE and lupus nephritis clinical trials.

  7. B cell abnormalities in systemic lupus erythematosus

    PubMed Central

    2003-01-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by the differentiation of short- and long-lived immunoglobulin secreting plasma cells that secrete pathogenic autoantibodies. Ectopic germinal centers and plasma cells secreting autoantibodies have been observed in lupus nephritis kidneys. Candidate genetic susceptibility loci for SLE include genes that affect differentiation and survival of plasma cells, such as those that influence activation, proliferation, cytokine and chemokine secretion/responsiveness, and apoptosis of the T and B cells that are involved in humoral immunity generated in germinal centers, as well as genes that are involved in presentation and clearance of apoptotic material and autoantigens by antigen presenting cells and other phagocytes. Emerging data have demonstrated that B lymphocytes are active participants in humoral immune responses that lead to T-dependent and T-independent differentiation of immunoglobulin-secreting plasma cells by homotypic CD154–CD40 interactions as well as continued stimulation by B cell activating factor through B cell maturation antigen, B cell activating factor receptor and transmembrane activater. PMID:15180894

  8. B cell abnormalities in systemic lupus erythematosus.

    PubMed

    Grammer, Amrie C; Lipsky, Peter E

    2003-01-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by the differentiation of short- and long-lived immunoglobulin secreting plasma cells that secrete pathogenic autoantibodies. Ectopic germinal centers and plasma cells secreting autoantibodies have been observed in lupus nephritis kidneys. Candidate genetic susceptibility loci for SLE include genes that affect differentiation and survival of plasma cells, such as those that influence activation, proliferation, cytokine and chemokine secretion/responsiveness, and apoptosis of the T and B cells that are involved in humoral immunity generated in germinal centers, as well as genes that are involved in presentation and clearance of apoptotic material and autoantigens by antigen presenting cells and other phagocytes. Emerging data have demonstrated that B lymphocytes are active participants in humoral immune responses that lead to T-dependent and T-independent differentiation of immunoglobulin-secreting plasma cells by homotypic CD154-CD40 interactions as well as continued stimulation by B cell activating factor through B cell maturation antigen, B cell activating factor receptor and transmembrane activater.

  9. Bromocriptine treatment of systemic lupus erythematosus.

    PubMed

    Walker, S E

    2001-01-01

    Prolactin, a peptide hormone, acts as a cytokine. It has been hypothesized that bromocriptine, a dopamine analog that suppresses pituitary secretion of prolactin, suppresses circulating prolactin and, through this mechanism, has the potential to suppress autoimmune disease. This rationale has been applied to the treatment of systemic lupus erythematosus (SLE), a prototype autoimmune illness that occurs spontaneously in animal models such as the F1 hybrid NZBxNZW mouse, and in humans. Treatment with bromocriptine was effective in treating some induced and spontaneous autoimmune disease in experimental models. Bromocriptine did slow the course of SLE in NZBxNZW mice when treatment was started before the appearance of clinical disease. In addition, bromocriptine was effective in treating established disease in this model. In three separate clinical trials, bromocriptine showed evidence that it had a therapeutic effect in treating human lupus. Bromocriptine is currently considered an unproven therapy for SLE. Its use is entirely experimental. The fact that bromocriptine was effective in treating NZBxNZW mice, the beneficial therapeutic effects in human trials, and the low toxicity of the drug form a solid rationale for undertaking further therapeutic trials.

  10. Natural autoantibodies in systemic lupus erythematosus.

    PubMed Central

    Matsiota, P; Druet, P; Dosquet, P; Guilbert, B; Avrameas, S

    1987-01-01

    We have tested the sera of 25 patients with systemic lupus erythematosus (SLE) for antibody activity against a panel of six antigens: DNA, TNP, actin, tubulin, myosin, albumin. Eluates from renal biopsy tissue were also tested. Sera from patients with lupus nephritis were found to contain high titres of IgA antibodies directed against the antigens of the panel, and marked IgG anti-DNA and anti-TNP antibody activity. The IgG anti-TNP antibodies isolated from SLE serum by affinity chromatography on a TNP-immunoadsorbent, were also found to possess anti-DNA activity. Kidney eluates obtained from biopsy specimens of SLE patients contained IgG antibodies strictly specific for DNA in three out of the nine patients tested, while three eluates from the remaining six patients reacted with DNA and TNP and three with DNA and all the other antigens of the panel. These results strongly suggest that in SLE sera there are at least three populations of circulating anti-DNA antibodies: those strictly specific for DNA, those recognizing DNA and TNP and those recognizing DNA and other macromolecules. Furthermore, because six out of nine of the eluates contained antibodies with an absolute or restricted specificity for DNA, this suggests that these antibodies are more often pathogenic than the polyspecific ones recognizing DNA and other macromolecules. PMID:3498588

  11. Sensory neuronopathy complicating systemic lupus erythematosus: a case report

    PubMed Central

    2014-01-01

    Introduction Systemic lupus erythematosus is a multi-system connective tissue disorder. Peripheral neuropathy is a known and underestimated complication in systemic lupus erythematosus. Ganglionopathy manifests when neuronal cell bodies in the dorsal root ganglion are involved. Autoimmune disorders are a known etiology, with systemic lupus erythematosus being a rare cause. Case presentation A 32-year-old South Asian woman presented with oral ulceration involving her lips following initiation of treatment for a febrile illness associated with dysuria. She had a history of progressively worsening numbness over a period of 4 months involving both the upper and lower limbs symmetrically while sparing the trunk. Her vibration sense was impaired, and her reflexes were diminished. For the past 4 years, she had had a bilateral, symmetrical, non-deforming arthritis involving the upper and lower limbs. Her anti-nuclear antibody and anti-double-stranded deoxyribonucleic acid status were positive. Although her anti-Ro antibodies were positive, she did not have clinical features suggestive of Sjögren syndrome. Nerve conduction studies revealed sensory neuronopathy. A diagnosis of systemic lupus erythematosus complicated by sensory neuronopathy was made. Treatment with intravenous immunoglobulin resulted in clinical and electrophysiological improvement. Conclusion Peripheral neuropathy in systemic lupus erythematosus can, by itself, be a disabling feature. Nerve conduction studies should be considered when relevant. Neuropathy in systemic lupus erythematosus should be given greater recognition, and rarer forms of presentation should be entertained in the differential diagnosis when the clinical picture is atypical. Intravenous immunoglobulin may have role in treatment of sensory neuronopathy in systemic lupus erythematosus. PMID:24884917

  12. Sinus node dysfunction in adult systemic lupus erythematosus flare: A case report.

    PubMed

    Yilmazer, Baris; Sali, Mursel; Cosan, Fulya; Cefle, Ayse

    2015-05-01

    Cardiac involvement can affect up to 50% of the systemic lupus erythematosus (SLE) patients but conduction system disturbances in SLE are less commonly described. For an early detection of this complication in the acute phase of SLE a whole cardiovascular examination and periodic electrocardiographic monitoring are recommended. We describe a patient who was diagnosed with flare up of lupus activity manifesting as sinus node dysfunction presenting as profound sinus bradycardia. She was successfully treated with high-dose methylprednisolone therapy.

  13. [Systemic lupus erythematosus and antiphospholipid syndrome: How to manage pregnancy?].

    PubMed

    Guettrot-Imbert, G; Le Guern, V; Morel, N; Vauthier, D; Tsatsaris, V; Pannier, E; Piette, J-C; Costedoat-Chalumeau, N

    2015-03-01

    Pregnancy in systemic lupus erythematosus patients is a common situation that remains associated with higher maternal and fetal mortality/morbidity than in the general population. Complications include lupus flares, obstetrical complications (fetal loss, in utero growth retardation, prematurity) and neonatal lupus syndrome. The association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetrical complications. Improving the care of these pregnancies depends upon a systematic pregnancy planning, ideally during a preconception counseling visit and a multidisciplinary approach (internist/rheumatologist, obstetrician and anesthetist). The absence of lupus activity, the use of appropriate medications during pregnancy adjusted to the patient's medical history and risk factors, and a regular monitoring are the best tools for a favorable outcome for these high-risk pregnancies. The aim of this review article is to perform an update on the medical care of pregnancy in systemic lupus erythematosus or antiphospholipid syndrome to reduce the risk of complications and to ensure the best maternal and fetal prognosis.

  14. Lupus

    MedlinePlus

    What is lupus? Lupus is an autoimmune disease. This means that your immune system attacks healthy cells and tissues by mistake. This can ... vessels, and brain. There are several kinds of lupus Systemic lupus erythematosus (SLE) is the most common ...

  15. [A severe course of autoimmune thrombocytopenia and the procedure for its treatment in systemic lupus erythematosus].

    PubMed

    Mendeleev, I M; Miasnikov, A A; Polezhaev, Iu N; Berliner, G B

    1991-01-01

    The authors describe three comparatively rare cases of extremely severe symptomatic autoimmune thrombocytopenia associated with systemic lupus erythematosus. The use of glucocorticoids in large doses and in two cases of splenectomy turned out ineffective. The next therapeutic measures are suggested in the following succession: glucocorticoids----cytostatic drugs (vincristine)----splenectomy to be performed only in special cases.

  16. Recent advances in cytokines in cutaneous and systemic lupus erythematosus.

    PubMed

    Mikita, Naoya; Ikeda, Takaharu; Ishiguro, Mariko; Furukawa, Fukumi

    2011-09-01

    Lupus erythematosus (LE) includes a broad spectrum of diseases from a cutaneous-limited type to a systemic type. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects multiple organs. Cutaneous lupus erythematosus (CLE) includes skin symptoms seen in SLE and cutaneous-limited LE. Although immune abnormalities, as well as heritable, hormonal and environmental factors, are involved in the pathology of LE, the actual pathogenesis is still unclear. Recently, the involvement of various cytokines has been shown in the pathogenesis of LE. Moreover, some trials with biological agents targeted specific cytokines are also ongoing for SLE. In this article, we review the contributions of major cytokines such as interferon, tumor necrosis factor-α and interleukin-18 to LE, especially SLE and CLE.

  17. Psychiatric disorders in Systemic Lupus Erythematosus.

    PubMed

    Hutchinson, G A; Nehall, J E; Simeon, D T

    1996-06-01

    The symptoms of Systemic Lupus Erythematosus (SLE) may include altered mental function. The present study sought to determine whether the psychiatric disorders are due to the disease itself or to the stress of having a chronic disease. Forty-five SLE patients attending outpatient clinics at the Port-of-Spain General Hospital in Trinidad were compared with two control groups: patients with chronic debilitating diseases similar to SLE in terms of chronicity and treatment (n = 44) and non-diseased individuals (n = 48). The Structured Clinical Interview for DSM III-R was used to identify psychiatric disorders. Both the SLE and the chronic illness groups had more psychiatric illness (44% and 39%, respectively) when compared with the non-diseased controls (2%) (p < 0.001). Major depression was the most common diagnosis among both diseased groups. However, psychotic illnesses (schizophrenic-type psychosis and bipolar disorders) were more prevalent in the SLE group (11.1% vs 0%, p = 0.02). These results indicate that major depression in SLE may be related more to the effects of a chronic illness than to SLE itself. However, the occurrence of psychotic symptoms may be related to SLE disease and needs further study.

  18. Intestinal Dysbiosis Associated with Systemic Lupus Erythematosus

    PubMed Central

    Hevia, Arancha; Milani, Christian; López, Patricia; Cuervo, Adriana; Arboleya, Silvia; Duranti, Sabrina; Turroni, Francesca; González, Sonia; Suárez, Ana; Gueimonde, Miguel; Ventura, Marco

    2014-01-01

    ABSTRACT Systemic lupus erythematosus (SLE) is the prototypical systemic autoimmune disease in humans and is characterized by the presence of hyperactive immune cells and aberrant antibody responses to nuclear and cytoplasmic antigens, including characteristic anti–double-stranded DNA antibodies. We performed a cross-sectional study in order to determine if an SLE-associated gut dysbiosis exists in patients without active disease. A group of 20 SLE patients in remission, for which there was strict inclusion and exclusion criteria, was recruited, and we used an optimized Ion Torrent 16S rRNA gene-based analysis protocol to decipher the fecal microbial profiles of these patients and compare them with those of 20 age- and sex-matched healthy control subjects. We found diversity to be comparable based on Shannon’s index. However, we saw a significantly lower Firmicutes/Bacteroidetes ratio in SLE individuals (median ratio, 1.97) than in healthy subjects (median ratio, 4.86; P < 0.002). A lower Firmicutes/Bacteroidetes ratio in SLE individuals was corroborated by quantitative PCR analysis. Notably, a decrease of some Firmicutes families was also detected. This dysbiosis is reflected, based on in silico functional inference, in an overrepresentation of oxidative phosphorylation and glycan utilization pathways in SLE patient microbiota. PMID:25271284

  19. 75 FR 35492 - Guidance for Industry on Lupus Nephritis Caused By Systemic Lupus Erythematosus-Developing...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-06-22

    ... Federal Register of March 29, 2005 (70 FR 15868), FDA announced the availability of a draft guidance... HUMAN SERVICES Food and Drug Administration Guidance for Industry on Lupus Nephritis Caused By Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Availability AGENCY: Food and...

  20. 77 FR 38305 - Guidance for Industry on Lupus Nephritis Caused by Systemic Lupus Erythematosus-Developing...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-27

    ... a notice published in the Federal Register of June 22, 2010 (75 FR 35492), FDA announced the... HUMAN SERVICES Food and Drug Administration Guidance for Industry on Lupus Nephritis Caused by Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Withdrawal of Guidance AGENCY: Food...

  1. Ethylenediaminetetraacetic acid-dependent pseudothrombocytopenia in a patient with systemic lupus erythematosus and lupus nephritis

    PubMed Central

    Akyol, Lütfi; Önem, Soner; Özgen, Metin; Sayarlıoğlu, Mehmet

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by several immunological abnormalities. We wish to communicate the case of a patient with SLE and lupus nephritis (LN) who developed pseudothrombocytopenia. Pseudothrombocytopenia can occur in patients with SLE and LN and should be considered when diagnosing patients with thrombocytopenia without bleeding.

  2. Coincident systemic lupus erythematosus and psoriasis vulgaris: a case report.

    PubMed

    Wang, Y; Da, G; Yu, Y; Han, J; Li, H

    2015-12-01

    Psoriasis vulgaris is an autoimmune chronic inflammatory skin disease, but its association with other typical autoimmune disease such as systemic lupus erythematosus has only occasionally been reported. We presented a 25-year-old female who developed systemic lupus erythematosus associated with psoriasis vulgaris. Her conditions were in good control after she got administration of prednisolone (5 mg/day) and Tripterygium Wilfordii Hook (20 mg/day). It is necessary to integrate past history and physical examination to diagnose coincident SLE and psoriasis, and combined treatment with prednisolone and Tripterygium Wilfordii Hook proves effective.

  3. Infection in systemic lupus erythematosus: friend or foe?

    PubMed Central

    Francis, Lisa; Perl, Andras

    2010-01-01

    Infectious agents have long been implicated in the pathogenesis of systemic lupus erythematosus. Common viruses, such as the Epstein-Barr virus, transfusion transmitted virus, parvovirus and cytomegalovirus, have an increased prevalence in patients with systemic lupus erythematosus. They may contribute to disease pathogenesis through triggering autoimmunity via structural or functional molecular mimicry, encoding proteins that induce cross-reactive immune responses to self antigens or modulate antigen processing, activation, or apoptosis of B and T cells, macrophages or dendritic cells. Alternatively, some infectious agents, such as malaria, Toxoplasma gondii and Helicobacter pylori, may have a protective effect. Vaccinations may play dual roles by protecting against friend and foe alike. PMID:20209114

  4. Kikuchi–Fujimoto disease and systemic lupus erythematosus

    PubMed Central

    Baenas, Diego F; Diehl, Fernando A; Haye Salinas, María J; Riva, Verónica; Diller, Ana; Lemos, Pablo A

    2016-01-01

    Kikuchi–Fujimoto disease, or histiocytic necrotizing lymphadenitis, is an infrequent idiopathic disorder. It has been associated with autoimmune disorders, of which systemic lupus erythematosus is the most outstanding. The basis of its diagnosis relies on the histological examination of lymph nodes, which typically reveals necrosis surrounded by histiocytes with crescentic nucleus, immunoblasts and plasma cells, and absence of neutrophils. We report the case of a 27-year-old Argentinian female patient without any relevant past medical history to demonstrate the correlation between Kikuchi–Fujimoto disease and systemic lupus erythematosus. PMID:27418858

  5. Lupus vulgaris in a patient with systemic lupus erythematosus and persistent IgG deficiency.

    PubMed

    Düzgün, N; Duman, M; Sonel, B; Peksari, Y; Erdem, C; Tokgöz, G

    1997-01-01

    We present the case of a patient with juvenile onset systemic lupus erythematosus (SLE) who developed a persistent, acquired hypogammaglobulinaemia with IgG deficiency. The hypogammaglobulinaemia was probably a complication of high dose corticosteroid treatment. The serum IgG level remained subnormal despite intravenous immunoglobulin therapy. Lupus vulgaris, which developed on the nasal cartilage in this patient with SLE, is not an expected finding. This patient is probably the first reported case of SLE associated with lupus vulgaris.

  6. Current role of rituximab in systemic lupus erythematosus.

    PubMed

    Mok, Chi Chiu

    2015-02-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by periods of flares and remission, resulting in organ damage over time caused by persistent disease activity and treatment-related complications. Conventional therapies are not ideal in terms of efficacy and safety. Novel biological therapies are being developed to enhance therapeutic efficacy, minimize disease exacerbation and reduce toxicities. As dysregulation of B cells is the hallmark of SLE, B-cell targeted therapies are the focus of recent clinical research. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been used with success in recalcitrant lupus manifestations. However, randomized controlled trials have failed to reveal its benefit in renal and non-renal SLE when combined with conventional immunosuppressive protocols. Although heterogeneity of SLE manifestations, pitfalls in study design and the limitations of the assessment tools for various clinical end points may have contributed to the discouraging results, rituximab remains an option in patients who are refractory or intolerant to conventional therapies. Recently, a regimen consisting of rituximab and mycophenolate mofetil without oral corticosteroids was reported to be effective in lupus nephritis. While the efficacy of this regimen has to be confirmed, future controlled trials should focus on the efficacy of rituximab in refractory lupus manifestations and its synergistic effect with other immunosuppressive agents such as cyclophosphamide. In short-term randomized controlled trials, a non-significant increase in serious adverse events was observed in SLE patients treated with rituximab. Long-term safety data of rituximab in SLE, in particular the incidence of hypogammaglobulinemia and serious/opportunistic infections, have to be continuously surveyed.

  7. [Relationship between Obesity, Adipokines and Systemic Lupus Erythematosus].

    PubMed

    Urrego, Tomas; Vásquez, Gloria M; Gómez-Puerta, Jose A

    2016-01-01

    Obesity is a pro-inflammatory state characterized by phenotypic changes in macrophages, alterationson cytokines balance, and increasing expression of regulatory molecules of the immune system derived from adipocytes and adipose tissue macrophages - also known as adipokines. Currently, leptin, adiponectin and resistin are, among others, one of the most known adipokines. Theseadipokinesmight play a possible role in systemic lupus erythematosus pathogenesis, by promotingdifferent pro-inflammatory conditions. Adipokines represent a possible treatment target in patients with lupus. PMID:27419894

  8. Systemic lupus erythematosus with membranous glomerulonephritis and uterine vasculitis.

    PubMed

    Feriozzi, S; Muda, A O; Amini, M; Faraggiana, T; Ancarani, E

    1997-02-01

    We report a case of lupus vasculitis with uterine localization and concurrent membranous nephropathy. Immunofluorescence study suggested the occurrence of an immune complex nephropathy and a pauci-immune pathogenesis of vasculitis. Our case points out the event of tissue damage in two organs mediated by different pathogenetic mechanisms. In addition, uterine vasculitis without pregnancy may be observed in patients with systemic lupus erythematosus nephritis.

  9. Systemic lupus erythematosus associated with Wells' syndrome.

    PubMed

    Yin, Geng; Xie, Qibing

    2012-04-01

    Wells' syndrome is a multifaceted dermatosis with a wide morphological spectrum, ranging from characteristic cellulitis-like erythema and papula to an unusual presentation of vesicles and pustules. The most important elements for diagnosis are erythemal plaques and histological picture of eosinophilic infiltration of the dermis with 'flame figures' (Plotz et al., in Hautarzt 51:182-186, 2000). Because of its original description as a distinct entity, it has come to be regarded as an abnormal eosinophilic response to a number of causative agents such as herpes simplex virus 2(HSV-2) and toxocara (Ludwig et al., in J Am Acad Dermatol 48:S60-S61, 2003; Bassukas et al., in Cases J 1:356, 2008). Concurrence of WS and malignant diseases as colon cancer, trachea squamous carcinoma, nasopharyngeal carcinoma or angioimmunoblastic lymphadenopathy has been reported (Hirsch et al., in J Dtsch Dermatol Ges 3:530-531, 2005; Renner et al., in Acta Derm Venereol 87:525-528, 2007). Autoimmune diseases, including Systemic lupus erythematosus (SLE) are multi-system disorders of unknown cause and are commonly characterized by protean cutaneous manifestations. To date, few autoimmne disease was found associated with WS except four previous reports of Churg-Strauss syndrome (CSS) and one case of ulcerative colitis (Fujimoto et al., in Clin Exp Dermatol, 2010; Sakaria et al., in J Gastroenterol 42:250-252, 2007). The coexistence of SLE and WS in one patient was not found in literature and our case is the first. Here we described the rare combination and discussed the treatment strategy for this condition.

  10. Circular RNAs and systemic lupus erythematosus.

    PubMed

    Li, Lian-Ju; Huang, Qing; Pan, Hai-Feng; Ye, Dong-Qing

    2016-08-15

    Circular RNAs (circRNAs) are a large class of noncoding RNAs that form covalently closed RNA circles. The discovery of circRNAs discloses a new layer of gene regulation occurred post-transcriptionally. Identification of endogenous circRNAs benefits from the advance in high-throughput RNA sequencing and remains challenging. Many studies probing into the mechanisms of circRNAs formation occurred cotranscriptionally or posttranscriptionally emerge and conclude that canonical splicing mechanism, sequence properties, and certain regulatory factors are at play in the process. Although our knowledge on functions of circRNAs is rather limited, a few circRNAs are shown to sponge miRNA and regulate gene transcription. The clearest case is one circRNA CDR1as that serves as sponge of miR-7. Researches on circRNAs in human diseases such as cancers highlight the function and physical relevance of circRNAs. Given the implication of miRNAs in the initiation and progression of systemic lupus erythematosus (SLE) and the roles of circRNAs in sponging miRNA and gene regulation, it is appealing to speculate that circRNAs may associate with SLE and may be potential therapeutic targets for treatment of SLE. Future studies should attach more importance to the relationship between circRNAs and SLE. This review will concern identification, biogenesis, and function of circRNAs, introduce reports exploring the association of circRNAs with human diseases, and conjecture the potential roles of circRNAs in SLE. PMID:27450756

  11. Severe Jaccoud's arthropathy in systemic lupus erythematosus.

    PubMed

    Santiago, Mittermayer B; Galvão, Verena; Ribeiro, Daniel Sá; Santos, Willer D; da Hora, Priscila R; Mota, Anna Paula; Pimenta, Emanuela; Oliveira, Isabela; Atta, Ajax M; Reis, Mitermayer G; Reis, Eliana A G; Lins, Carolina

    2015-10-01

    Jaccoud's arthropathy (JA) is a clinical situation nowadays present mostly in systemic lupus erythematosus (SLE). It is characterized by the presence of joint deformities such as "swan neck," ulnar deviation and "Z-thumb" resembling rheumatoid arthritis (RA) but that are passively correctable and without bone erosion on plain radiographs. From our cohort of SLE patients with JA, we selected a subgroup with a more severe form of this arthropathy and looked at their clinical and laboratory profile as well as studied the magnetic resonance imaging (MRI) findings or ultrasound (US) obtained from the hand with most evident deformities. Seven SLE patients with a severe form of JA were identified. All seven patients have "swan neck," ulnar deviation and "Z-thumb" deformities. Two out of seven had "mutilans-type JA" and four had fixed deformities in the metacarpophalangeal (MCP) joints. The MRI of the hand with more evident deformity clinically performed in six cases and US performed in one case showed mild synovitis in five and moderate synovitis in two patients, mild flexor tenosynovitis in six and severe tenosynovitis in one. Only two small bone erosions were observed in the second and third MCP joints of one patient with moderate synovitis. Severe JA compromises the functional capacity of the joints and imposes the risk of misdiagnosis of RA. With the improvement of the survival rate of SLE and the lack of specific prophylactic or therapeutical measures for JA, it is reasonable to assume that more and more cases of severe JA are going to be identified. PMID:26310503

  12. Anti-DNA autoantibodies and systemic lupus erythematosus.

    PubMed

    Blatt, N B; Glick, G D

    1999-08-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that affects most of the organs and tissues of the body, causing glomerulonephritis, arthritis, and cerebritis. SLE can be fatal with nephritis, in particular, predicting a poor outcome for patients. In this review, we highlight what has been learned about SLE from the study of mouse models, and pay particular attention to anti-DNA autoantibodies, both as pathological agents of lupus nephritis and as DNA-binding proteins. We summarize the current approaches used to treat SLE and discuss the targeting of anti-DNA autoantibodies as a new treatment for lupus nephritis.

  13. Paraneoplastic subacute cutaneous lupus erythematosus associated with cholangiocarcinoma.

    PubMed

    Jenkins, David; McPherson, Tess

    2016-02-01

    Subacute cutaneous lupus erythematosus (SCLE) is a dermatosis that occurs in genetically predisposed individuals. The exogenous stimulus that triggers this condition is usually unknown; however, medication is often implicated. Malignancy is a rare cause. We present a case of paraneoplastic SCLE to cholangiocarcinoma and briefly review the features of this interesting entity.

  14. Acute Pancreatitis as the Initial Presentation of Systematic Lupus Erythematosus

    PubMed Central

    Jia, Yi; Ortiz, Arleen; Mccallum, Richard; Salameh, Hasan; Serrato, Pedro

    2014-01-01

    Systematic lupus erythematosus (SLE) is a multisystem disease, including the gastrointestinal system in about half of SLE patients. As a rare complication of SLE, acute pancreatitis presents as generalized flare-ups in most cases of patients previously diagnosed with SLE. Here we report a rare case of acute pancreatitis as the initial presentation with later diagnosis of SLE. PMID:25197582

  15. Histuria and fibrinuria in cases of systemic lupus erythematosus

    PubMed Central

    Antoine, B.; Ward, P. Dorrington

    1970-01-01

    In thirty-nine urine specimens from twenty-seven patients with systemic lupus erythematosus, tissue-like and fibrinogen-like material was looked for by immuno-diffusion techniques, using specific antisera corresponding to both antigenic categories. Half the samples exhibited an abnormal outflow of tissue-like material (histuria). Fibrinuria appeared in only a few instances when cases with microscopic haematuria were discarded. Abnormal histuria had a significant correlation with the following signs of consistent lupoid renal involvement: significant urinary total protein output (>2 mg/min), impairment of renal function and extracapillary epithelial proliferation in the glomeruli seen on biopsy. In systemic lupus erythematosus with evidence of renal involvement, the major origin of abnormal histuria is probably a direct leakage of macromolecules from damaged kidney tissue. Abnormal histuria was also observed in cases of systemic lupus erythematosus during subacute stages of the disease without any signs of renal involvement. In the latter case, histuria is likely to originate from damaged tissues outside the kidney. It is concluded that abnormal histuria during the course of systemic lupus erythematosus may indicate various organic alterations, especially in the kidney, consequent to the primary mechanism of the systemic lupoid disease. On the contrary, fibrinuria may be diverse in origin. ImagesFig. 2 PMID:4984587

  16. Chorea in systemic lupus erythematosus: association with antiphospholipid antibodies.

    PubMed Central

    Khamashta, M A; Gil, A; Anciones, B; Lavilla, P; Valencia, M E; Pintado, V; Vázquez, J J

    1988-01-01

    Chorea is a rare manifestation of systemic lupus erythematosus (SLE). In this report the clinical features of two cases of chorea associated with SLE are presented. Of special interest were the raised titres of antiphospholipid antibodies in both cases. The possible pathogenic role of these antibodies is briefly discussed. PMID:3415367

  17. [Lichen ruber planus--lupus erythematosus/overlap syndrome].

    PubMed

    Stary, A; Schwarz, T; Duschet, P; Gschnait, F

    1987-03-01

    Lichen planus/lupus erythematosus/overlap syndrome (OS) comprises those dermatoses which show the clinical, histologic, and immunopathologic characteristics of both diseases. On account of this heterogeneity, the diagnosis of OS may be difficult. About 35 cases have been reported on in the literature so far. We are going to discuss the clinical, histologic, and immunofluorescence findings in OS in detail.

  18. Interleukin-17 in systemic lupus erythematosus: A comprehensive review.

    PubMed

    Li, Duo; Guo, Bin; Wu, Haijing; Tan, Lina; Chang, Christopher; Lu, Qianjin

    2015-01-01

    Systemic lupus erythematosus (SLE) is a complicated autoimmune disease of multifactorial pathoaetiology. One of the most serious manifestations is lupus nephritis. The pathogenesis of SLE has not been well elucidated, but it has been reported that interleukin-17 (IL-17) and Th17 cells play important roles in the pathogenesis of SLE. IL-17A, a member of IL-17 family, amplifies the immune response by inducing the local production of chemokines and cytokines, recruiting neutrophils and monocytes, augmenting the production of autoantibodies, and aggravating the inflammation and damage of target organs such as the kidney in SLE. In recent years, several IL-17A pathway inhibitors have advanced into clinical trials, including the anti-IL-17A monoclonal antibody and the anti-17RA monoclonal antibody. Several agents have shown great success in Phase II trials in multiple autoimmune diseases such as psoriasis, rheumatoid arthritis, ankylosing spondylitis, multiple sclerosis, and non-infectious uveitis, which has sparked the urgent need of anti-IL-17A as innovative therapeutic option in controlling disease activity of moderate-to-severe SLE. Here, we review and summarize current progress in IL-17A and SLE from in vitro studies, human expression studies, and animal models, providing novel insight into its therapeutic potential.

  19. Myocardial perfusion abnormalities in asymptomatic patients with systemic lupus erythematosus

    SciTech Connect

    Hosenpud, J.D.; Montanaro, A.; Hart, M.V.; Haines, J.E.; Specht, H.D.; Bennett, R.M.; Kloster, F.E.

    1984-08-01

    Accelerated coronary artery disease and myocardial infarction in young patients with systemic lupus erythematosus is well documented; however, the prevalence of coronary involvement is unknown. Accordingly, 26 patients with systemic lupus were selected irrespective of previous cardiac history to undergo exercise thallium-201 cardiac scintigraphy. Segmental perfusion abnormalities were present in 10 of the 26 studies (38.5 percent). Five patients had reversible defects suggesting ischemia, four patients had persistent defects consistent with scar, and one patient had both reversible and persistent defects in two areas. There was no correlation between positive thallium results and duration of disease, amount of corticosteroid treatment, major organ system involvement or age. Only a history of pericarditis appeared to be associated with positive thallium-201 results (p less than 0.05). It is concluded that segmental myocardial perfusion abnormalities are common in patients with systemic lupus erythematosus. Whether this reflects large-vessel coronary disease or small-vessel abnormalities remains to be determined.

  20. Statistical considerations for stopping systemic lupus erythematosus clinical trials earlier.

    PubMed

    Lew, Robert A; Liang, Matthew H; Doros, Gheorghe

    2015-12-04

    Group sequential designs are used to potentially shorten randomized clinical trials and thereby reduce subject burden, improve safety, and save time and resources. Clinical trials comparing treatments for systemic lupus erythematosus (SLE) might adopt such designs if the ordinal outcome scales for SLE, such as the Systemic Lupus Activity Measure and Systemic Lupus Erythematosus Disease Activity Index, were more like continuous outcome scales with interval properties. After describing the basic features of sequential trials and highlighting some major issues in their design, we propose approaches that mitigate these issues. In particular, high-speed computing has accelerated advances in sequential design, making available a variety of designs that can be implemented with minimal technical support. The challenge now is to understand the concepts behind such flexible designs and then to apply them to improve studies of SLE.

  1. Total lymphoid irradiation in refractory systemic lupus erythematosus

    SciTech Connect

    Ben-Chetrit, E.; Gross, D.J.; Braverman, A.; Weshler, Z.; Fuks, Z.; Slavin, S.; Eliakim, M.

    1986-07-01

    In two patients with systemic lupus erythematosus, conventional therapy was considered to have failed because of persistent disease activity and unacceptable side effects. Both were treated with total lymphoid irradiation without clinical benefit, despite adequate immunosuppression as documented by markedly reduced numbers of circulating T lymphocytes and T-lymphocyte-dependent proliferative responses in vitro. The first patient developed herpes zoster, gram-negative septicemia, neurologic symptoms, and deterioration of lupus nephritis. The second patient developed massive bronchopneumonia, necrotic cutaneous lesions, and progressive nephritis and died 2 weeks after completion of radiotherapy. These observations, although limited to two patients, indicate that total lymphoid irradiation in patients with severe systemic lupus erythematosus should be regarded as strictly experimental.

  2. Sweet syndrome associated with hydralazine-induced lupus erythematosus.

    PubMed

    Cartee, Todd V; Chen, Suephy C

    2012-03-01

    Sweet syndrome (SS) is a distinctive but poorly understood clinical syndrome, which likely represents an immunologic reaction pattern to a wide range of underlying or preceding conditions, including viral illnesses, inflammatory bowel disease, and malignancies. We report the case of a patient who presented with an acute eruption that was clinically and histologically consistent with SS. The patient also met diagnostic criteria for systemic lupus erythematosus with serositis, stomatitis, positive antinuclear antibody (ANA), and positive anti-double-stranded DNA antibodies. Additionally, positive antihistone antibodies and exposure to hydralazine supported the specific diagnosis of drug-induced lupus erythematosus, and we concluded that his SS was a manifestation of hydralazine-induced lupus. We also briefly review the precedence for this unusual dual diagnosis in the literature.

  3. Cardiovascular Events in Systemic Lupus Erythematosus

    PubMed Central

    Fernández-Nebro, Antonio; Rúa-Figueroa, Íñigo; López-Longo, Francisco J.; Galindo-Izquierdo, María; Calvo-Alén, Jaime; Olivé-Marqués, Alejandro; Ordóñez-Cañizares, Carmen; Martín-Martínez, María A.; Blanco, Ricardo; Melero-González, Rafael; Ibáñez-Rúan, Jesús; Bernal-Vidal, José Antonio; Tomero-Muriel, Eva; Uriarte-Isacelaya, Esther; Horcada-Rubio, Loreto; Freire-González, Mercedes; Narváez, Javier; Boteanu, Alina L.; Santos-Soler, Gregorio; Andreu, José L.; Pego-Reigosa, José M.

    2015-01-01

    Abstract This article estimates the frequency of cardiovascular (CV) events that occurred after diagnosis in a large Spanish cohort of patients with systemic lupus erythematosus (SLE) and investigates the main risk factors for atherosclerosis. RELESSER is a nationwide multicenter, hospital-based registry of SLE patients. This is a cross-sectional study. Demographic and clinical variables, the presence of traditional risk factors, and CV events were collected. A CV event was defined as a myocardial infarction, angina, stroke, and/or peripheral artery disease. Multiple logistic regression analysis was performed to investigate the possible risk factors for atherosclerosis. From 2011 to 2012, 3658 SLE patients were enrolled. Of these, 374 (10.9%) patients suffered at least a CV event. In 269 (7.4%) patients, the CV events occurred after SLE diagnosis (86.2% women, median [interquartile range] age 54.9 years [43.2–66.1], and SLE duration of 212.0 months [120.8–289.0]). Strokes (5.7%) were the most frequent CV event, followed by ischemic heart disease (3.8%) and peripheral artery disease (2.2%). Multivariate analysis identified age (odds ratio [95% confidence interval], 1.03 [1.02–1.04]), hypertension (1.71 [1.20–2.44]), smoking (1.48 [1.06–2.07]), diabetes (2.2 [1.32–3.74]), dyslipidemia (2.18 [1.54–3.09]), neurolupus (2.42 [1.56–3.75]), valvulopathy (2.44 [1.34–4.26]), serositis (1.54 [1.09–2.18]), antiphospholipid antibodies (1.57 [1.13–2.17]), low complement (1.81 [1.12–2.93]), and azathioprine (1.47 [1.04–2.07]) as risk factors for CV events. We have confirmed that SLE patients suffer a high prevalence of premature CV disease. Both traditional and nontraditional risk factors contribute to this higher prevalence. Although it needs to be verified with future studies, our study also shows—for the first time—an association between diabetes and CV events in SLE patients. PMID:26200625

  4. Circulating microparticles in systemic Lupus Erythematosus.

    PubMed

    Nielsen, Christoffer Tandrup

    2012-11-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease presenting with a wide array of clinical manifestations and an elusive pathogenesis. A characteristic feature in SLE is the occurrence of autoantibodies against chromatin, double-stranded DNA, and RNA-binding ribonucleoproteins. Observations of defective clearance of dying cells in SLE combined with the generation and exposure of nuclear autoantigens during apoptosis have led to the hypothesis that improperly cleared apoptotic debris constitutes a source of autoantigens capable of triggering autoimmune disease. In blood, circulating, heterogeneous subcellular microparticles (MPs) are released from cells and platelets constitutively and upon cellular activation or apoptosis. Such MPs may reflect the state of their parental cells and tissues, and could serve as markers of pathology. Particular in SLE MPs may serve as carriers of autoantigens and constituents of immune complexes (ICs). The purposes of this PhD thesis were to develop and apply qualitative and quantitative methods to characterize circulating MPs with respect to numbers, cellular origins and composition in a large cohort of well-characterized SLE patients compared to healthy and disease controls and to explore associations with clinical, biochemical and serological parameters. The PhD thesis consists of a review and three papers. In the first paper we show that SLE patients have significantly decreased numbers of annexin V binding MPs and MPs from platelets, leukocytes and endothelial cells using flow cytometry. Two morphologically distinguishable populations of annexin V non-binding MPs were increased in the SLE patients. The annexin V non-binding MPs of most likely cellular origin were associated with the presence of lupus nephritis, markers of increased disease activity and levels of endothelial cell-derived MPs. In the second paper we present the development of a proteomic method to characterize the protein composition of purified

  5. [Evaluation of systemic lupus erythematosus activity during pregnancy].

    PubMed

    Olesińska, Marzena; Wiesik-Szewczyk, Ewa; Chwalińska-Sadowska, Hanna

    2007-07-01

    Pregnancy in patients with systemic lupus erythematosus (SLE) is considered a high-risk pregnancy. It is complicated by preeclampsia, premature labour and miscarriage more frequently than in the general population. Improved prognosis depends on low disease activity during conception and on appropriate medical care (SLE activity monitoring, selection of therapy safe for the mother and the developing foetus, advances in neonatology). Because symptoms of physiological pregnancy and SLE exacerbation are similar, their correct interpretation is essential for skin lesions, arthralgias, arterial hypertension or results of laboratory tests: proteinuria, thrombocytopenia or leucopenia observed in the patient. In order to standardise the assessment of SLE activity during pregnancy, scores of this activity are used. In the past, scores validated on non-pregnant populations (including male patients) were used: Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Systemic Lupus Activity Measure (SLAM), European Consensus Lupus Activity Measurment (ECLAM). Only recently have SLE activity scores been introduced that are specific for pregnant women: Lupus Activity Index In Pregnancy (LAI-P), Systemic Lupus Erythematosus Pregnancy Disease Activity Index (SLEPDAI), modified--Systemic Lupus Activity Measure (m-SLAM) and a visual three-grade score modified--Physician Global Assessment (m-PGA). So far, only scores LAI-P and m-PGA have been validated. According to the LAI-P score, clinical data are divided into 4 groups. Group 1 includes mild clinical symptoms, group 2--symptoms of involvement of internal organs, group 3 pertains to modifications of treatment and group 4 to laboratory parameters. Point values are ascribed to individual parameters depending on their intensity.

  6. MR appearance of bilateral, spontaneous patellar tendon rupture in systemic lupus erythematosus.

    PubMed

    Gould, E S; Taylor, S; Naidich, J B; Furie, R; Lane, L

    1987-01-01

    Bilateral spontaneous patellar tendon rupture is an unusual complication in patients with systemic lupus erythematosus. The ability of magnetic resonance to detect these tendon abnormalities is demonstrated.

  7. Generation of self-peptides to treat systemic lupus erythematosus.

    PubMed

    Briand, Jean-Paul; Schall, Nicolas; Muller, Sylviane

    2014-01-01

    Synthetic peptides are attracting increasing attention as therapeutics. Despite their potential, however, only a few selected peptides have been able to enter in clinical trials for chronic autoimmune diseases and systemic lupus erythematosus (SLE) in particular. Here, we describe and discuss a series of assays, which may help in characterizing valuable candidate peptides that were applied in our laboratory to develop the lupus P140 peptide program. The different steps of selection include the choice of the initial autoantigen, the design, synthesis and purification of peptides, their preliminary screen by measuring cytokines produced ex vivo by T cells and their binding to major histocompatibility complex class II (MHCII) molecules, their capacity to lower peripheral cell hyperproliferation in lupus-prone MRL/lpr mice, and, as a final step, their ability to slow down the development of lupus disease in model animals.

  8. Palpebral involvement as a presenting and sole manifestation of discoid lupus erythematosus.

    PubMed

    Yaghoobi, Reza; Feily, Amir; Behrooz, Bahar; Yaghoobi, Elena; Mokhtarzadeh, Shabnam

    2010-11-04

    A 28-year-old woman presented with a 2-year history of idiopathic, chronic blepharitis unresponsive to several courses treatment of corticosteroid eye drops. Physical examination was notable for edematous, erythematous plaques of the lower eyelids with madarosis in the absence of preceding skin scarring. Biopsy specimen was obtained and diagnosis of discoid lupus erythematosus (DLE) was made. DLE is a chronic, cutaneous disease that is clinically characterized by a malar rash, acute erythema, and discoid lesions. Localized DLE occurs when the head and neck only are affected, while widespread DLE occurs when other areas are affected, regardless of whether disease of the head and neck is seen. Patients with widespread involvement often have hematologic and serologic abnormalities, are more likely to develop systemic lupus erythematosus, and are more difficult to treat. A number of skin diseases may be confused with DLE, such as psoriasis, seborrheic dermatitis, acne, rosacea, lupus vulgaris, sarcoidosis, Bowen's disease, polymorphous light eruption, lichen planopilaris, dermatomyositis, granuloma annulare, and granuloma faciale. Palpebral lesions may rarely be the presenting or sole manifestation of the disease and lower eyelid involvement is seen in 6% of patients with chronic, cutaneous lupus erythematosus. DLE should therefore be considered as a differential diagnosis in chronic blepharitis or madarosis that persists despite usual medical management and eyelid hygiene. The patient was treated successfully with hydroxychloroquine. The skin lesions resolved with minimal scarring.

  9. Renal tubular dysfunction presenting as recurrent hypokalemic periodic quadriparesis in systemic lupus erythematosus

    PubMed Central

    Prasad, D.; Agarwal, D.; Malhotra, V.; Beniwal, P.

    2014-01-01

    We report recurrent hypokalemic periodic quadriparesis in a 30-year-old woman. Patient had also symptoms of multiple large and small joint pain, recurrent oral ulceration, photosensitivity and hair loss that were persisting since last 6 months and investigations revealed systemic lupus erythematosus (SLE) with distal tubular acidosis. Our patient was successfully treated with oral potassium chloride, sodium bicarbonate, hydroxychloroquine and a short course of steroids. Thus, tubular dysfunction should be carefully assessed in patients with SLE. PMID:25249723

  10. Vitamin D and Systemic Lupus Erythematosus: Myth or Reality?

    PubMed

    Watad, Abdulla; Neumann, Shana G; Soriano, Alessandra; Amital, Howard; Shoenfeld, Yehuda

    2016-01-01

    There is growing interest in the contribution of vitamin D deficiency to autoimmunity. Several studies have shown an association between low levels of vitamin D and autoimmune disorders, including multiple sclerosis, rheumatoid arthritis, type 1 diabetes, autoimmune thyroid diseases, celiac disease, and systemic lupus erythematosus (SLE). Vitamin D receptor ligands can mediate immunosuppressive effects. It has been suggested that low levels of this hormone contribute to the immune activation in lupus and other autoimmune diseases. This review updates and summarizes the literature on the association between vitamin D and SLE, and discusses the various correlations between vitamin D and SLE activity, clinical expressions, serology, and gene polymorphisms of vitamin D receptors.

  11. Vitamin D and Systemic Lupus Erythematosus: Myth or Reality?

    PubMed

    Watad, Abdulla; Neumann, Shana G; Soriano, Alessandra; Amital, Howard; Shoenfeld, Yehuda

    2016-01-01

    There is growing interest in the contribution of vitamin D deficiency to autoimmunity. Several studies have shown an association between low levels of vitamin D and autoimmune disorders, including multiple sclerosis, rheumatoid arthritis, type 1 diabetes, autoimmune thyroid diseases, celiac disease, and systemic lupus erythematosus (SLE). Vitamin D receptor ligands can mediate immunosuppressive effects. It has been suggested that low levels of this hormone contribute to the immune activation in lupus and other autoimmune diseases. This review updates and summarizes the literature on the association between vitamin D and SLE, and discusses the various correlations between vitamin D and SLE activity, clinical expressions, serology, and gene polymorphisms of vitamin D receptors. PMID:27228639

  12. Neuropsychiatric systemic lupus erythematosus in elderly people: a case series.

    PubMed Central

    Dennis, M S; Byrne, E J; Hopkinson, N; Bendall, P

    1992-01-01

    Five elderly patients presenting with neuropsychiatric systemic lupus erythematosus were referred to the sectorised psychiatry service of the department of health care of the elderly. They represented 2% of patients admitted over a period of two years. Two patients presented with a subacute confusional state, two with dementia, and one with depression. Three patients responded well to treatment. This suggests that systemic lupus erythematosus (SLE) is more common in elderly people than was originally thought and is a potentially treatable cause of organic brain disorder. The absence of reports of elderly patients with SLE is likely to be due to the continued application of the American Rheumatism Association's revised 1982 classification criteria, which are inappropriate for this population. PMID:1479395

  13. [Cytokine production in patients with systemic lupus erythematosus].

    PubMed

    Müzes, G; González-Cabello, R; Van Vien, C; Fehér, J; Gergely, P

    1991-09-01

    The production of different cytokines, namely interleukin-2, interleukin-1 and tumor necrosis factor-alpha produced by peripheral immunocompetent cells was evaluated in patients with systemic lupus erythematosus in active and inactive stage of the disease. The results obtained were compared to healthy controls. It has been found that lymphocytes from both groups of SLE patients produced similarly less interleukin-2 activity. Interleukin-1 activity of monocytes was significantly reduced only in patients with active stage of the disease, whereas tumor necrosis factor-alpha production was diminished even in cases of inactive SLE. The simultaneous detection of the above mentioned cytokines may indicate further details concerning immunoregulatory disturbances of systemic lupus erythematosus. PMID:1923468

  14. Vitamin D and systemic lupus erythematosus: continued evolution.

    PubMed

    Yap, Kristy S; Morand, Eric F

    2015-02-01

    Vitamin D is a steroid hormone that has well-established roles in calcium and bone metabolism. Vitamin D has more recently become recognized for its role in the immune response and its potential immunomodulatory effects in autoimmune diseases, including systemic lupus erythematosus (SLE). This review provides a summary of the recent literature regarding vitamin D and SLE, as well as current recommendations for vitamin D supplementation in patients with SLE.

  15. Systemic Lupus Erythematosus with Deep Vein Thrombosis and Cutaneous Ulcer.

    PubMed

    Saigal, Renu; Goyal, Laxmikant; Agrawal, Abhishek; Wadhwani, Dileep; Mital, Pradeep; Sharma, Rajeev

    2015-09-01

    We are reporting a case of systemic lupus erythematosus (SLE) with left upper limb and lower limb deep vein thrombosis (DVT) due to protein S deficiency which was aggravated by anticoagulants. Oral anticoagulant-induced skin necrosis also developed in this patient. This patient was negative for anti-phospholipid antibodies (APLA). Such a case is rarity where SLE patient without APLA has protein S deficiency. PMID:27608879

  16. Systemic Lupus Erythematosus for General Practitioners: A Literature Review

    PubMed Central

    Karrar, Ali; AI-Dalaan, Abdullah

    1994-01-01

    Systemic lupus erythematosus (SLE) is a multisystem disease of unknown etiology or etiologies. The disease may be acute or chronic. A wide clinicopathological spectrum is expressed in each organ involved which is induced through multiple antibodies that result in. imnunologically mediated tissue injury. In this literature review, the clinical and pathological features as well as laboratory abnormalities, measures /or diagnosis, outlines of management, and prognosis are discussed. PMID:23008531

  17. Novel molecular targets in the treatment of systemic lupus erythematosus

    PubMed Central

    Crispín, JoséC; Tsokos, George C.

    2009-01-01

    T cells from patients with systemic lupus erythematosus (SLE) display a number of biochemical abnormalities which include altered expression of key signaling molecules, heightened calcium responses, and skewed expression of transcription factors. These defects are involved in the altered behavior of SLE T cells and are probably central in the disease pathogenesis. The aim of this communication is to review the defects that have been consistently documented in SLE T cells, highlighting molecules and pathways that represent therapeutic targets. PMID:18190888

  18. Monoclonal antibodies in the treatment of systemic lupus erythematosus.

    PubMed

    Robak, Ewa; Robak, Tadeusz

    2009-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by B cell hyperactivity and defective T-cell function, with production of high titer autoantibodies. In the recent years, conceptual advances and the introduction of new therapies are yielding improvements in the management of this disease. In recent years, clinical studies have been undertaken with selected monoclonal antibodies (mAbs) in the treatment of SLE. The important role of B cells in the pathogenesis of autoimmune disorders has provided a strong rationale to target B cells in SLE. Selective therapeutic depletion of B-cells became possible with the availability of the anti-CD20 antibody rituximab and anti-CD22 antibody epratuzumab. Several clinical studies confirm high activity of rituximab in SLE patients especially with lupus nephritis and neuropsychiatric involvement. Recently, several new mAbs reacting with CD20 have been developed. New mAbs directed against CD20 include fully human mAb ofatumumab (HuMax CD20), IMMU-106 (hA20) which has a >90% humanized framework and GA-101, a novel third-generation fully humanized and optimized mAb. These agents are highly cytotoxic against B-cell lymphoid cells. Proinflammatory cytokines such as tumor necrosis factor-alpha (TNF-alpha) and iterleukin-6 (IL-6) play an important role in propagating the inflammatory process responsible for tissue damage. Blocking of these cytokines by mAbs can be also a successful therapy for patients with SLE. Finally, mAb eculizumab that specifically inhibits terminal complement activation has been recently developed and investigated in the phase I single dose study in SLE. In this review, new mAbs, potentially useful in SLE are presented.

  19. Advances in the treatment of cutaneous lupus erythematosus.

    PubMed

    Kuhn, A; Landmann, A; Wenzel, J

    2016-07-01

    Lupus erythematosus (LE) is a multifactorial autoimmune disease with clinical manifestations of differing severity which may present with skin manifestations as primary sign of the disease (cutaneous lupus erythematosus, CLE) or as part of a disease spectrum (systemic lupus erythematosus, SLE). To date, no drugs are approved specifically for the treatment of CLE and only single agents have been applied in randomized controlled trials. Therefore, topical and systemic agents are used "off-label", primarily based on open-label studies, case series, retrospective analyses, and expert opinions. In contrast, several agents, such as hydroxychloroquine, chloroquine, cyclophosphamide, azathioprine, and belimumab, are approved for the treatment of SLE. Recent approaches in the understanding of the molecular pathogenesis of LE enabled the development of further new agents, which target molecules such as interleukin 6 (IL-6) and interferon (IFN). Only single trials, however, applied these new agents in patients with cutaneous involvement of the disease and/or included endpoints which evaluated the efficacy of these agents on skin manifestations. This article provides an updated review on new and recent approaches in the treatment of CLE. PMID:27252259

  20. Advances in the treatment of cutaneous lupus erythematosus.

    PubMed

    Kuhn, A; Landmann, A; Wenzel, J

    2016-07-01

    Lupus erythematosus (LE) is a multifactorial autoimmune disease with clinical manifestations of differing severity which may present with skin manifestations as primary sign of the disease (cutaneous lupus erythematosus, CLE) or as part of a disease spectrum (systemic lupus erythematosus, SLE). To date, no drugs are approved specifically for the treatment of CLE and only single agents have been applied in randomized controlled trials. Therefore, topical and systemic agents are used "off-label", primarily based on open-label studies, case series, retrospective analyses, and expert opinions. In contrast, several agents, such as hydroxychloroquine, chloroquine, cyclophosphamide, azathioprine, and belimumab, are approved for the treatment of SLE. Recent approaches in the understanding of the molecular pathogenesis of LE enabled the development of further new agents, which target molecules such as interleukin 6 (IL-6) and interferon (IFN). Only single trials, however, applied these new agents in patients with cutaneous involvement of the disease and/or included endpoints which evaluated the efficacy of these agents on skin manifestations. This article provides an updated review on new and recent approaches in the treatment of CLE.

  1. Blisters and Loss of Epidermis in Patients With Lupus Erythematosus

    PubMed Central

    Merklen-Djafri, Carine; Bessis, Didier; Frances, Camille; Poulalhon, Nicolas; Debarbieux, Sébastien; Cordel, Nadège; Lipsker, Dan

    2015-01-01

    Abstract The nosology of bullous lesions or equivalents (vesicles, erosions, and crusts) in patients with lupus erythematosus (LE) is rarely addressed. The primary aim of this study was to draw up a precise phenotypic inventory of such skin lesions; the secondary objective was to assess a potential relationship between the different types of loss of epidermis and extracutaneous lupus manifestations. We conducted a retrospective multicenter study including 22 patients with definite LE and bullous lesions or equivalents. All biopsies were reviewed. Patients were recruited in the dermatology departments of 6 centers. Patients were included if they met the diagnosis of systemic LE according to American College of Rheumatology and/or Systemic Lupus International Collaborating Clinics criteria or diagnosis of cutaneous LE based on classic clinical criteria and/or histological ascertainment of LE. Patients were recruited through clinician's memory and photographic collections. Three clinico-pathological patterns could be individualized. First, toxic epidermal necrolysis (TEN)-like, sheet-like, skin detachment; sun-exposure, mild mucosal involvement, and dermal mucin deposition allow differential diagnosis with classical Lyell syndrome. Second, vesiculo-bullae and/or crusting occurring on typical lesions of subacute cutaneous lupus erythematosus or chronic cutaneous lupus erythematosus. Third, tense vesicles and/or blisters with an underlying neutrophilic dermatosis and a usual response to dapsone. A careful analysis of 22 LE patients with epidermal detachment reveals 2 main pathomechanisms: a classic LE interface dermatitis, which can be hyperacute and lead to TEN-like skin detachment; and a neutrophilic dermatosis, with tense vesicles and/or blisters, including classic bullous LE. PMID:26579826

  2. Pyomyositis in childhood-systemic lupus erythematosus.

    PubMed

    Blay, Gabriela; Ferriani, Mariana P L; Buscatti, Izabel M; França, Camila M P; Campos, Lucia M A; Silva, Clovis A

    2016-01-01

    Pyomyositis is a pyogenic infection of skeletal muscle that arises from hematogenous spread and usually presents with localized abscess. This muscle infection has been rarely reported in adult-onset systemic lupus erythematous and, to the best of our knowledge, has not been diagnosed in pediatric lupus population. Among our childhood-onset systemic lupus erythematous population, including 289 patients, one presented pyomyositis. This patient was diagnosed with childhood-onset systemic lupus erythematous at the age of 10 years-old. After six years, while being treated with prednisone, azathioprine and hydroxychloroquine, she was hospitalized due to a 30-day history of insidious pain in the left thigh and no apparent trauma or fever were reported. Her physical examination showed muscle tenderness and woody induration. Laboratory tests revealed anemia, increased acute phase reactants and normal muscle enzymes. Computer tomography of the left thigh showed collection on the middle third of the vastus intermedius, suggesting purulent stage of pyomyositis. Treatment with broad-spectrum antibiotic was initiated, leading to a complete clinical resolution. In conclusion, we described the first case of pyomyositis during childhood in pediatric lupus population. This report reinforces that the presence of localized muscle pain in immunocompromised patients, even without elevation of muscle enzymes, should raise the suspicion of pyomyositis. A prompt antibiotic therapy is strongly recommended. PMID:27267338

  3. Pyomyositis in childhood-systemic lupus erythematosus.

    PubMed

    Blay, Gabriela; Ferriani, Mariana P L; Buscatti, Izabel M; França, Camila M P; Campos, Lucia M A; Silva, Clovis A

    2016-01-01

    Pyomyositis is a pyogenic infection of skeletal muscle that arises from hematogenous spread and usually presents with localized abscess. This muscle infection has been rarely reported in adult-onset systemic lupus erythematous and, to the best of our knowledge, has not been diagnosed in pediatric lupus population. Among our childhood-onset systemic lupus erythematous population, including 289 patients, one presented pyomyositis. This patient was diagnosed with childhood-onset systemic lupus erythematous at the age of 10 years-old. After six years, while being treated with prednisone, azathioprine and hydroxychloroquine, she was hospitalized due to a 30-day history of insidious pain in the left thigh and no apparent trauma or fever were reported. Her physical examination showed muscle tenderness and woody induration. Laboratory tests revealed anemia, increased acute phase reactants and normal muscle enzymes. Computer tomography of the left thigh showed collection on the middle third of the vastus intermedius, suggesting purulent stage of pyomyositis. Treatment with broad-spectrum antibiotic was initiated, leading to a complete clinical resolution. In conclusion, we described the first case of pyomyositis during childhood in pediatric lupus population. This report reinforces that the presence of localized muscle pain in immunocompromised patients, even without elevation of muscle enzymes, should raise the suspicion of pyomyositis. A prompt antibiotic therapy is strongly recommended.

  4. Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management.

    PubMed

    Lam, Nguyet-Cam Vu; Ghetu, Maria V; Bieniek, Marzena L

    2016-08-15

    Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskeletal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and reproductive systems. Family physicians should be familiar with the manifestations of lupus to aid in early diagnosis, monitoring patients with mild disease, recognizing warning signs that require referral to a rheumatologist, and helping to monitor disease activity and treatment in patients with moderate to severe disease. The American College of Rheumatology has 11 classification criteria for lupus. If a patient meets at least four criteria, lupus can be diagnosed with 95% specificity and 85% sensitivity. All patients with lupus should receive education, counseling, and support. Hydroxychloroquine is the cornerstone of treatment because it reduces disease flares and other constitutional symptoms. Low-dose glucocorticoids can be used to treat most manifestations of lupus. The use of immunosuppressive and cytotoxic agents depends on the body systems affected. Patients with mild disease that does not involve major organ systems can be monitored by their family physician. Patients with increased disease activity, complications, or adverse effects from treatment should be referred to a rheumatologist. To optimize treatment, it is important that a rheumatologist coordinate closely with the patient's family physician to improve chronic care as well as preventive health services. PMID:27548593

  5. Radiologic findings in late-onset systemic lupus erythematosus

    SciTech Connect

    Braunstein, E.M.; Weissman, B.N.; Sosman, J.L.; Schur, P.H.

    1983-03-01

    Systemic lupus erythematosus in the elderly has a different clinical and serologic course from that in young patients. Radiographic findings in patients in whom the diagnosis was made after age 50 were compared with findings in younger patients to see if the radiologic patterns are also different. The only significant radiographic difference between the two groups was that the older group had a greater incidence of soft-tissue swelling of the hands and wrists (p < 0.001). There was no significant difference in osteopenia, erosion, soft-tissue calcification, alignment abnormalities, or intrathoracic findings. Of 24 patients over age 50, two developed lymphoma and another developed multiple myeloma. The data agree with clinical observations that there is a higher incidence of arthritis in late-onset lupus, but clinical findings of increased incidence of pleuropericardial disease are not confirmed radiographically. The coincidence of hematologic malignancy with late-onset lupus in this series is noteworthy.

  6. [A case report of childhood systemic lupus erythematosus complicated with lupus cystitis].

    PubMed

    Kurosawa, Rumiko; Miyamae, Takako; Imagawa, Tomoyuki; Katakura, Shigeki; Mori, Masaaki; Aihara, Yuhkoh; Yokota, Shumpei

    2006-06-01

    The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught measles, she reported abdominal pain, and urinalysis indicated urinary protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with NSAIDs, but 2 months later fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed erythema at the orifice of the left ureter. The pathological examination indicated the presence of vasculitis, and finally lupus cystitis was diagnosed. Intravenous cyclophosphamide (IVCY)-pulse therapy was introduced to a total of 8 times over the period of a year, and maintenance therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse therapy, the hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus cystitis and successfully treated by IVCY-pulse therapy and maintenance predonisolone and azathioprin.

  7. 75 FR 35493 - Guidance for Industry on Systemic Lupus Erythematosus-Developing Medical Products for Treatment...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-06-22

    ... the Federal Register of March 29, 2005 (70 FR 15868), FDA announced the availability of a draft... Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Availability AGENCY: Food and Drug... availability of a guidance for industry entitled ``Systemic Lupus Erythematosus--Developing Medical...

  8. Case report: disseminated dermatophytosis by microsporum gypseum in a systemic lupus erythematosus patient

    PubMed Central

    Macêdo, Danielle Patrícia Cerqueira; Neves, Rejane Pereira; Lopes, Flávia Cadengue

    2008-01-01

    Mycosis is a major contributor to morbidity and mortality in patients with systemic lupus erythematosus and frequent exposition to an infectious source could enhance the development of dermatophytic infections. A case of disseminated dermatophytosis by Microsporum gypseum is reported in a systemic lupus erythematosus (SLE) patient. PMID:24031171

  9. Neurodevelopmental disorders in children born to mothers with systemic lupus erythematosus.

    PubMed

    Vinet, É; Pineau, C A; Clarke, A E; Fombonne, É; Platt, R W; Bernatsky, S

    2014-10-01

    Children born to women with systemic lupus erythematosus seem to have a potentially increased risk of neurodevelopmental disorders compared to children born to healthy women. Recent experimental data suggest in utero exposure to maternal antibodies and cytokines as important risk factors for neurodevelopmental disorders. Interestingly, women with systemic lupus erythematosus display high levels of autoantibodies and cytokines, which have been shown, in animal models, to alter fetal brain development and induce behavioral anomalies in offspring. Furthermore, subjects with systemic lupus erythematosus and neurodevelopmental disorders share a common genetic predisposition, which could impair the fetal immune response to in utero immunologic insults. Moreover, systemic lupus erythematosus pregnancies are at increased risk of adverse obstetrical outcomes and medication exposures, which have been implicated as potential risk factors for neurodevelopmental disorders. In this article, we review the current state of knowledge on neurodevelopmental disorders and their potential determinants in systemic lupus erythematosus offspring.

  10. Phenotypic associations of genetic susceptibility loci in systemic lupus erythematosus

    PubMed Central

    Sanchez, Elena; Nadig, Ajay; Richardson, Bruce C; Freedman, Barry I; Kaufman, Kenneth M; Kelly, Jennifer A; Niewold, Timothy B; Kamen, Diane L; Gilkeson, Gary S; Ziegler, Julie T; Langefeld, Carl D; Alarcón, Graciela S; Edberg, Jeffrey C; Ramsey-Goldman, Rosalind; Petri, Michelle; Brown, Elizabeth E; Kimberly, Robert P; Reveille, John D; Vilá, Luis M; Merrill, Joan T; Anaya, Juan-Manuel; James, Judith A; Pons-Estel, Bernardo A; Martin, Javier; Park, So-Yeon; Bang, So-Young; Bae, Sang-Cheol; Moser, Kathy L; Vyse, Timothy J; Criswell, Lindsey A; Gaffney, Patrick M; Tsao, Betty P; Jacob, Chaim O; Harley, John B; Alarcón-Riquelme, Marta E; Sawalha, Amr H

    2011-01-01

    Objective Systemic lupus erythematosus is a clinically heterogeneous autoimmune disease. A number of genetic loci that increase lupus susceptibility have been established. This study examines if these genetic loci also contribute to the clinical heterogeneity in lupus. Materials and methods 4001 European-derived, 1547 Hispanic, 1590 African-American and 1191 Asian lupus patients were genotyped for 16 confirmed lupus susceptibility loci. Ancestry informative markers were genotyped to calculate and adjust for admixture. The association between the risk allele in each locus was determined and compared in patients with and without the various clinical manifestations included in the ACR criteria. Results Renal disorder was significantly correlated with the lupus risk allele in ITGAM (p=5.0×10−6, OR 1.25, 95% CI 1.12 to 1.35) and in TNFSF4 (p=0.0013, OR 1.14, 95% CI 1.07 to 1.25). Other significant findings include the association between risk alleles in FCGR2A and malar rash (p=0.0031, OR 1.11, 95% CI 1.17 to 1.33), ITGAM and discoid rash (p=0.0020, OR 1.20, 95% CI 1.06 to 1.33), STAT4 and protection from oral ulcers (p=0.0027, OR 0.89, 95% CI 0.83 to 0.96) and IL21 and haematological disorder (p=0.0027, OR 1.13, 95% CI 1.04 to 1.22). All these associations are significant with a false discovery rate of <0.05 and pass the significance threshold using Bonferroni correction for multiple testing. Conclusion Significant associations were found between lupus clinical manifestations and the FCGR2A, ITGAM, STAT4, TNSF4 and IL21 genes. The findings suggest that genetic profiling might be a useful tool to predict disease manifestations in lupus patients in the future. PMID:21719445

  11. Interferon-alpha: a therapeutic target in systemic lupus erythematosus.

    PubMed

    Crow, Mary K

    2010-02-01

    The long history of elevated interferon (IFN)-alpha in association with disease activity in patients who have systemic lupus erythematosus (SLE) has assumed high significance in the past decade, with accumulating data strongly supporting broad activation of the type I IFN pathway in cells of patients who have lupus, and association of IFN pathway activation with significant clinical manifestations of SLE and increased disease activity based on validated measures. In addition, a convincing association of IFN pathway activation with the presence of autoantibodies specific for RNA-binding proteins has contributed to delineation of an important role for Toll-like receptor activation by RNA-containing immune complexes in amplifying innate immune system activation and IFN pathway activation. Although the primary triggers of SLE and the IFN pathway remain undefined, rapid progress in lupus genetics is helping define lupus-associated genetic variants with a functional relationship to IFN production or response in patients. Together, the explosion of data and understanding related to the IFN pathway in SLE have readied the lupus community for translation of those insights to improved patient care. Patience will be needed to allow collection of clinical data and biologic specimens across multiple clinical centers required to support testing of IFN activity, IFN-inducible gene expression and chemokine gene products as candidate biomarkers. Meanwhile, promising clinical trials are moving forward to test the safety and efficacy of monoclonal antibody inhibitors of IFN-alpha. Other therapeutic approaches to target the IFN pathway may follow close behind.

  12. Risk factors of systemic lupus erythematosus flares during pregnancy.

    PubMed

    Jara, Luis J; Medina, Gabriela; Cruz-Dominguez, Pilar; Navarro, Carmen; Vera-Lastra, Olga; Saavedra, Miguel A

    2014-12-01

    This review examines the risk factors for the development of systemic lupus erythematosus (SLE) flares during pregnancy. In preconception, anti-DNA, hypocomplementemia, previous thrombosis, triple antiphospholipid (aPL) antibody positivity, active lupus nephritis and discontinuation of medications such as hydroxychloroquine and azathioprine are factors associated with pregnancy failure. During pregnancy, SLE flares are associated with aPL antibodies, synergic changes of pregnancy on Th1 and TH2 cytokines, other cytokines and chemokines that interact with hormones such as estrogen and prolactin that amplify the inflammatory effect. From the clinical point of view, SLE activity at pregnancy onset, thrombocytopenia, lupus nephritis, arterial hypertension, aPL syndromes, preeclampsia is associated with lupus flares and fetal complications. In puerperium, the risk factors of flares are similar to pregnancy. Hyperactivity of immune system, autoantibodies, hyperprolactinemia, active lupus nephritis, decrease in TH2 cytokines with increase in TH1 cytokines probably participate in SLE flare. The SLE flares during pregnancy make the difference between an uncomplicated pregnancy and pregnancy with maternal and fetal complications. Therefore, the knowledge of risk factors leads the best treatment strategies to reduce flares and fetal complications in SLE patients.

  13. [Systemic lupus erythematosus in the pregnant patient. Implications for anesthesia].

    PubMed

    Pastor Tomás, E; Guillén Antón, J; Vaquerizo Gareta, A; Lirola Grajales, P; Martínez García, R; Cuartero Lobera, J

    2001-03-01

    A 28-year-old woman with systemic lupus erythematosus and a history of aseptic meningitis, digestive bleeding due to thrombopenia and deep venous thrombosis underwent elective cesarean for transverse presentation at 35 weeks. Preoperative blood work-up showed an antinuclear antibody titre that was slightly positive and steroid treatment was started. Surgery operation was performed with general anesthesia. The outcome was satisfactory even though serious complications can develop during the management of anesthesia in such patients. Systemic lupus erythematosus is a chronic, multisystemic disease that mainly affects women of childbearing age. Antibodies and immunocomplexes play a fundamental role. Given the multiorgan involvement in this disease, preoperative study of the lupus patient should assess all such involvement, including maternal-fetal risk, as well as consider the drug and anesthetic management to be applied. Among the clinical signs that can affect management of anesthesia are the following: aseptic meningitis, high blood pressure, pericarditis, pneumonitis and recurrent venous thrombosis. Anemia, thrombopenia and significantly altered coagulation events are common.

  14. C2 Deficiency DEVELOPMENT OF LUPUS ERYTHEMATOSUS

    PubMed Central

    Day, Noorbibi K.; Geiger, H.; McLean, R.; Michael, A.; Good, R. A.

    1973-01-01

    The study of serum from a patient with C2 deficiency is described. The patient had an episode of pneumococcal meningitis at 5 mo of age with seizures and transient hemiparesis and apparent purpuric skin lesions. He was first admitted to the University of Minnesota Hospitals at 10 yr of age following the discovery of proteinuria accidentally by his mother. Since then he has been admitted repeatedly to this hospital with numerous clinical findings including arthralgia, recurrent abdominal pain, proteinuria, membranous nephropathy, malar butterfly rash, seizures, personality aberrations, and recurrent fever. In June 1971, the patient developed positive DNA and DNP antibodies and positive LE cells. When the C profile was studied before and after recognition of lupus, C1q, C1s, and C4 dropped. C3 levels were elevated as were C5, C6, and C7, C3 proactivator had been reduced in the patient even before he developed lupus. Also because of a traumatic renal biopsy leading to a perirenal hematoma, he required surgery and a blood transfusion. 1 h after blood transfusion, a C2 titer of 23 hemolytic units was detected. Almost immediately levels of C3, C5, C6, and C7 dropped, C8 and C9 remained elevated. The addition of C2 from normal blood permitted dramatic activation of C3. These findings support the view that the rare deficiency in production of C2 predisposes to serious susceptibility to infection, vascular and mesenchymal disease as well as to renal disease and a lupus syndrome. Images PMID:4578155

  15. Response to Antimalarials in Cutaneous Lupus Erythematosus A Prospective Analysis

    PubMed Central

    Chang, Aileen Y.; Piette, Evan W.; Foering, Kristen P.; Tenhave, Thomas R.; Okawa, Joyce; Werth, Victoria P.

    2012-01-01

    Objective To demonstrate response to antimalarials in patients with cutaneous lupus erythematosus using activity scores from the Cutaneous Lupus Erythematosus Disease Area and Severity Index, a validated outcome measure. Design Prospective, longitudinal cohort study. Setting University cutaneous autoimmune disease clinic. Participants One hundred twenty-eight patients with cutaneous lupus erythematosus who presented from January 2007-July 2010 and had at least 2 visits with activity scores. Main Outcome Measures Response defined by 4-point or 20% decrease in activity score. Response to initiation determined with score before treatment and first visit at least 2 months after treatment. Response to continuation determined with score at first visit and most recent visit on treatment. Results Of 11 patients initiated on hydroxychloroquine, 55% were responders with a decrease in median (interquartile range) activity score from 8.0 (3.5-13) to 3.0 (1.8-7.3) (p=0.03). Of 15 patients who had failed hydroxychloroquine, 67% were responders to initiation of hydroxychloroquine-quinacrine, with a decrease in median (interquartile range) activity score from 6.0 (4.8-8.3) to 3.0 (0.75-5.0) (p=0.004). Nine out of 21 patients (43%) continued on hydroxychloroquine and 9 out of 21 patients (43%) continued on hydroxychloroquine-quinacrine were responders with a decrease in median (interquartile range) activity score from 6.0 (1.5-9.5) to 1.0 (0-4.5) (p=0.009) and 8.5 (4.25-17.5) to 5.0 (0.5-11.5) (p=0.01), respectively. Conclusion The use of quinacrine with hydroxychloroquine is associated with response in patients who fail hydroxychloroquine monotherapy. Further reduction in disease activity can be associated with continuation of antimalarials. PMID:21768444

  16. Lupus cystitis in Korean patients with systemic lupus erythematosus: risk factors and clinical outcomes.

    PubMed

    Koh, J H; Lee, J; Jung, S M; Ju, J H; Park, S-H; Kim, H-Y; Kwok, S-K

    2015-10-01

    This study was performed to investigate the clinical characteristics of lupus cystitis and determine the risk factors and clinical outcomes of lupus cystitis in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed 1064 patients at Seoul St. Mary's Hospital in Seoul, Korea, from 1998 to 2013. Twenty-four patients had lupus cystitis. Lupus cystitis was defined as unexplained ureteritis and/or cystitis as detected by imaging studies, cystoscopy, or bladder histopathology without urinary microorganisms or stones. Three-fourths of patients with lupus cystitis had concurrent lupus mesenteric vasculitis (LMV). The initial symptoms were gastrointestinal in nature for most patients (79.2%). High-dose methylprednisolone was initially administered to most patients (91.7%) with lupus cystitis. Two patients (8.3%) died of urinary tract infections. Sixty-five age- and sex-matched patients with SLE who were admitted with other manifestations were included as the control group. Patients with lupus cystitis showed a lower C3 level (p = 0.031), higher SLE Disease Activity Index score (p = 0.006), and higher ESR (p = 0.05) upon admission; more frequently had a history of LMV prior to admission (p < 0.001); and less frequently had a history of neuropsychiatric lupus (p = 0.031) than did patients with SLE but without lupus cystitis. The occurrence of lupus cystitis was associated with a history of LMV (OR, 21.794; 95% CI, 4.061-116.963). The median follow-up period was 3.4 years, and the cumulative one-year mortality rate was 20%. Complications developed in 33.3% of patients with lupus cystitis and were related to survival (log-rank p = 0.021). Our results suggest that the possibility of lupus cystitis should be considered when a patient with SLE and history of LMV presents with gastrointestinal symptoms or lower urinary tract symptoms. Development of complications in patients with lupus cystitis can be fatal. Thus, intensive treatment

  17. Takayasu's Arteritis with Systemic Lupus Erythematosus: A Rare Association.

    PubMed

    Bandyopadhyay, Dhrubajyoti; Ganesan, Vijayan; Bhar, Debarati; Bhowmick, Diptak; Sasmal, Sibnarayan; Choudhury, Cankatika; Mukhopadhyay, Sabyasachi; Hajra, Adrija; Layek, Manas; Karmakar, Partha Sarathi

    2015-01-01

    We report the case of a 24-year-old nondiabetic, nonhypertensive lady with history of fatigue, dyspnoea and limb claudication. She has been diagnosed with Takayasu's arteritis. Subsequently she developed rash, alopecia, joint pain, and various other laboratory abnormalities which led to a diagnosis of SLE. Takayasu's arteritis (TA) rarely coexists with systemic lupus erythematosus (SLE). The absence of specific SLE markers in patients with TA who subsequently develop SLE suggests that the coexistence of these conditions may be coincidental. The antiphospholipid syndrome in patients with SLE may mimic the occlusive vasculitis of TA. PMID:26167325

  18. Conditioning as an adjunct in the pharmacotherapy of lupus erythematosus.

    PubMed

    Olness, K; Ader, R

    1992-04-01

    Several studies have provided evidence suggesting that "placebo effects" represent conditioning phenomena and that learning processes influence the response to placebo medication. This case report describes an adolescent with severe lupus erythematosus who received cyclophosphamide (CY) paired with taste (cod liver oil) and smell (rose perfume) as conditioned stimuli. The regimen was based on conditioning experiments with animals who had lupuslike autoimmune disease. After the initial pairings, the taste alone was offered between CY treatments. Over 12 months, the patient received six rather than 12 CY treatments, half the cumulative dose that might have been administered. The patient improved clinically, and 5 years later continues to do well.

  19. Congenital heart block and maternal systemic lupus erythematosus.

    PubMed Central

    Esscher, E; Scott, J S

    1979-01-01

    The association between infants with congenital heart block (CHB) and the presence or later development of maternal systemic lupus erythematosus or other connective-tissue disease (CTD) was reviewed in 67 cases. In 24 cases CHB was diagnosed at or before birth. Of nine necropsies on affected infants, seven showed endomyocardial fibrosis. The results suggest that one in three mothers who deliver babies with CHB have or will develop CTD. The association is probably explained by placental transfer of a maternal antibody. Awareness of the association may lead to prevention of the birth of children with CHB and better neonatal care of affected children. PMID:455010

  20. One year in review 2016: systemic lupus erythematosus.

    PubMed

    Adinolfi, Antonella; Valentini, Eleonora; Calabresi, Emanuele; Tesei, Giulia; Signorini, Viola; Barsotti, Simone; Tani, Chiara

    2016-01-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a highly variable course and prognosis. The management of the disease is still a clinical challenge for the treating physicians as many aspects regarding the disease pathogenesis, clinical picture and outcomes remain to be elucidated. New and interesting data are emerging; here the recent literature on SLE pathogenesis, clinical and laboratory aspects, as well as treatments and comorbidities, are reviewed and the main findings summarised in order to provide a bird's eye on the relevant papers on these topics. PMID:27463977

  1. Emerging role of adipokines in systemic lupus erythematosus.

    PubMed

    Li, Hong-Miao; Zhang, Tian-Ping; Leng, Rui-Xue; Li, Xiang-Pei; Li, Xiao-Mei; Liu, Hai-Rong; Ye, Dong-Qing; Pan, Hai-Feng

    2016-08-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by multisystem organ involvement and unclear pathogenesis. Several adipokines synthesized in the adipose tissue, including leptin, adiponectin, resistin, and chemerin, have been explored in autoimmune rheumatic diseases, especially SLE, and results suggest that these mediators may be implicated in the pathogenesis of SLE. However, the current results are controversial. In this review, we will briefly discuss the expression and possible pathogenic role of several important adipokines, including leptin, adiponectin, resistin, and chemerin in SLE. PMID:27314594

  2. Vaccination of Adult Patients with Systemic Lupus Erythematosus in Portugal

    PubMed Central

    Moraes-Fontes, Maria Francisca; Antunes, Ana Margarida; Gruner, Heidi; Riso, Nuno

    2016-01-01

    In the wake of the Portuguese vaccination program 50th anniversary it seems appropriate to review vaccination in patients with systemic lupus erythematosus. Controversial issues as regards the association between autoimmune diseases, infections, and vaccines are discussed as well as vaccine safety and efficacy issues as regards chronic immunosuppressant (IS) drug therapy. After a brief overview of national policies, specific recommendations are made as regards vaccination for adult patients with SLE with a particular focus on current IS therapy and unmet needs. PMID:27069477

  3. Vitamin D and systemic lupus erythematosus: state of the art.

    PubMed

    Schneider, Laiana; Dos Santos, Amanda Senna Pereira; Santos, Marcele; da Silva Chakr, Rafael Mendonça; Monticielo, Odirlei Andre

    2014-08-01

    Systemic lupus erythematosus (SLE) is a systemic inflammatory disease associated with genetic, environmental, hormonal, and immunological factors. One of these factors is vitamin D deficiency. Vitamin D plays many roles in the immune system. Several studies have suggested a potential role in the development of autoimmune diseases. SLE patients have low serum levels of vitamin D, which increase the possibility of an association between vitamin deficiency and disease onset and evolution. This review of the literature presents an analysis of the aspects related to the immunoregulatory effects of vitamin D and its importance for SLE, as well as the recommendations for vitamin D supplementation in these patients.

  4. Poor specific antibody response immunodeficiency (dysgammaglobulinemia) predates systemic lupus erythematosus.

    PubMed

    Al Hamzi, H; Al Shaikh, A; Arnaout, R K

    2013-08-01

    Poor specific antibody response is a well-known primary immunodeficiency that is related to hypogammaglobulinemia or common variable immunodeficiency (CVID). The co-existence of CVID or hypogammaglobulinemia and systemic lupus erythematosus (SLE) has been rarely described. In all reported cases, the diagnosis of SLE antedates CVID. We report a 15-year-old Saudi girl who was diagnosed with poor specific antibody response at age 6 years in the form of poor or no antibody response and dysgammaglobulinemia. She developed SLE with musculoskeletal and hematological manifestations, positive antinuclear antibody and high anti-dsDNA nine years later. She was treated with rituximab with good response.

  5. One year in review 2016: systemic lupus erythematosus.

    PubMed

    Adinolfi, Antonella; Valentini, Eleonora; Calabresi, Emanuele; Tesei, Giulia; Signorini, Viola; Barsotti, Simone; Tani, Chiara

    2016-01-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a highly variable course and prognosis. The management of the disease is still a clinical challenge for the treating physicians as many aspects regarding the disease pathogenesis, clinical picture and outcomes remain to be elucidated. New and interesting data are emerging; here the recent literature on SLE pathogenesis, clinical and laboratory aspects, as well as treatments and comorbidities, are reviewed and the main findings summarised in order to provide a bird's eye on the relevant papers on these topics.

  6. Systemic lupus erythematosus and thyrotoxicosis: a hitherto little recognised association.

    PubMed Central

    Rodrigué, S; Laborde, H; Catoggio, P M

    1989-01-01

    Six patients are reported in whom systemic lupus erythematosus (SLE) and thyrotoxicosis coexisted. All had four or more American Rheumatism Association criteria (1982) for the diagnosis of SLE and had clinical manifestations and function test results characteristic of hyperthyroidism (except for one who had been thyroidectomised previously). In three patients the diagnosis of hyperthyroidism preceded that of SLE, in two patients both diseases began simultaneously, and only in one was the diagnosis of thyrotoxicosis made after that of SLE. It is suggested that hyperthyroidism associated with SLE may be a form of presentation of thyroiditis. This association may pass unnoticed because of the similarity of some clinical manifestations. PMID:2730168

  7. An Unusual Mimicker of Systemic Lupus Erythematosus: A Case Report

    PubMed Central

    Aluoch, Aloice O; Farbman, Mathew; Gladue, Heather

    2015-01-01

    We present a case of a 47 year-old African American female with 15 pack-years of tobacco use and heavy alcohol use who presented with arthritis and was found to have a positive antinuclear antibodies (ANA), anti double stranded DNA antibodies (anti-dsDNA), and anti-Sjogren’s syndrome-related antigen A and antigen B (anti-SSA and anti-SSB). She was subsequently found to have a lung adenocarcinoma associated with hypertrophic pulmonary osteoarthropathy (HPO). This demonstrates a case of positive antinuclear antibodies and arthritis in a patient with lung adenocarcinoma, which can be falsely diagnosed as systemic lupus erythematosus. PMID:26106457

  8. Parkinsonism and transient bilateral ptosis in systemic lupus erythematosus

    PubMed Central

    Teoh, P. C.; Richard, A. T. Ng; Wong, P. K.

    1974-01-01

    Many neurological abnormalities have been described in systemic lupus erythematosus (SLE), but transient bilateral ptosis and parkinsonism are rarely encountered. This paper describes a young Malay girl with SLE who develops psychosis, bilateral ptosis and parkinsonism during an exacerbation of her illness. These neurological features disappeared after adequate treatment with cyclophosphamide. Though the pathogenesis of these neurological abnormalities is not clearly known, it is likely that transient bilateral ptosis is due to myoneural dysfunction not unlike that of myasthenia gravis. As for parkinsonism, it can probably be explained on the basis of ‘vasculitis’ of the basal ganglia leading to microinfarcts and encephalomalacia. ImagesFig. 1Fig. 2

  9. Hydrocephalus in an elderly man with systemic lupus erythematosus.

    PubMed

    Chen, Wei-Sheng; Wu, Tsai-Hung; Chou, Chung-Tei; Tsai, Chang-Youh

    2009-06-01

    A 71-year-old man presented with quadriplegia, seizures, dysarthria, motor aphasia and urinary incontinence lasting for several years. The development of proteinuria and increased susceptibility to infections brought the physician's attention to possible underlying autoimmune diseases. Laboratory investigations revealed evidence for systemic lupus erythematosus (SLE) and antiphospholipid syndrome. Imaging studies showed obstructive hydrocephalus. Several courses of methylprednisolone therapies followed by maintenance therapy with low-dose steroid, ventriculoperitoneal shunt, and antihypertensives improved the proteinuria and dysarthria but not the urinary incontinence or dementia. A thromboembolic event in the central nervous system secondary to phospholipid antibodies or lupus activity may represent a pathogenetic basis for hydrocephalus. When encountering a patient with hydrocephalus but without apparent predisposing factors, it is always important to include SLE as a differential diagnosis.

  10. Cutaneous lupus erythematosus: recent lessons from animal models.

    PubMed

    Ghoreishi, M; Dutz, J P

    2010-08-01

    Cutaneous lupus erythematosus (CLE) may present as a clinically heterogeneous group of lupus-specific skin lesions that have common histopathological findings. Determination of the immunopathological sequence of events in this group of disorders has been challenging for dermatologists and immunologists but is vital for therapeutic targeting. We review animal models in which different aspects of immune alteration in CLE have been addressed. The MRL/lpr mouse develops spontaneous skin disease with some features of CLE. Study of this strain and related gene-manipulated strains has revealed roles for multiple cytokines, including interleukin (IL)-6, IL-18, and IL-21, in disease pathogenesis. A role for the growth factor colony stimulating factor 1 and the inflammatory protein high-mobility group box 1 has also been suggested. We discuss potential novel treatment options suggested by these models.

  11. Cavitary pulmonary lesions in systemic lupus erythematosus: an unusual manifestation.

    PubMed

    Dalili, Amir Reza; Lotfi, Reza; Mousavi, Seyedeh Maryam

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown pathogenesis. The frequency of SLE with cavitary lesion manifestation is very rare and is thought to be due to infection or pulmonary embolism. A 19-year-old female diagnosed with SLE complicated by lupus nephritis and cavitary pulmonary lesion is presented in this case report. Other diseases that can lead to such lesions were ruled out in the patient. The patient improved briefly after the initiation of immunosuppressive therapy, but was unresponsive to supportive treatment due to pneumothorax. Pneumothorax is caused by cavitary lesions and possibly bronchopleural fistulas - these later caused respiratory distress and death. The patient did not show any improvement in the lesions after the initiation of immunosuppressive therapy. This case report suggests that the differential diagnosis of cavitary lung lesions should include SLE. PMID:25763160

  12. Genetic Factors in Systemic Lupus Erythematosus: Contribution to Disease Phenotype

    PubMed Central

    Ceccarelli, Fulvia; Perricone, Carlo; Borgiani, Paola; Ciccacci, Cinzia; Rufini, Sara; Cipriano, Enrica; Alessandri, Cristiano; Spinelli, Francesca Romana; Sili Scavalli, Antonio; Novelli, Giuseppe; Valesini, Guido; Conti, Fabrizio

    2015-01-01

    Genetic factors exert an important role in determining Systemic Lupus Erythematosus (SLE) susceptibility, interplaying with environmental factors. Several genetic studies in various SLE populations have identified numerous susceptibility loci. From a clinical point of view, SLE is characterized by a great heterogeneity in terms of clinical and laboratory manifestations. As widely demonstrated, specific laboratory features are associated with clinical disease subset, with different severity degree. Similarly, in the last years, an association between specific phenotypes and genetic variants has been identified, allowing the possibility to elucidate different mechanisms and pathways accountable for disease manifestations. However, except for Lupus Nephritis (LN), no studies have been designed to identify the genetic variants associated with the development of different phenotypes. In this review, we will report data currently known about this specific association. PMID:26798662

  13. Presence of hepatitis-associated antigen in systemic lupus erythematosus

    PubMed Central

    Alarcón-Segovia, D.; Fishbein, Eugenia; Díaz-Jouanen, E.

    1972-01-01

    Presence of hepatitis-associated antigen (HAA) was investigated in 504 sera from 116 patients with SLE and was found in 41% of them. HAA was present in at least one serum in 75% of the patients but there were variations in presence and titres in the same patient at different times. Except for a tendency of HAA to appear or rise in titre with lupusi nactivation following corticosteroid or immunosuppresive therapy, there was no correlation between its presence and disease activity, specific organ involvement, antinuclear antibodies or immunoglobulin levels. All but one of twelve lupus patients with recurrent bacterial infections had HAA at high titres. HAA appeared in the serum of a patient upon development of IgA deficiency. HAA antigenaemia in systemic lupus erythematosus seems a consequence rather than a cause of the immunological derangement in this disease. PMID:4538860

  14. Amyloïdosis, sarcoidosis and systemic lupus erythematosus.

    PubMed

    Rezgui, Amel; Hassine, Imene Ben; Karmani, Monia; Fredj, Fatma Ben; Laouani, Chadia

    2016-01-01

    The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca syndrome. Biological findings showed an inflammatory syndrome, renal failure, proteinuria (1g / 24h), positive auto antibodies and anti DNA. Lung radiology revealed medistinal lymphadenopathy, pleural nodules, ground glass infiltrates and pleuritis. Bronchial biopsy showed non specific inflammation. The salivary gland biopsy showed amyloïd deposits. This case report reminds us that lupus and Amyloïdosis association, although exceptional remains possible. The occurrence of Lofgren syndrome in this situation make the originality of this report. PMID:27583087

  15. B cell biology: implications for treatment of systemic lupus erythematosus.

    PubMed

    Anolik, J H

    2013-04-01

    B cells are critical players in the orchestration of properly regulated immune responses, normally providing protective immunity without autoimmunity. Balance in the B cell compartment is achieved through the finely regulated participation of multiple B cell populations with different antibody-dependent and independent functions. Both types of functions allow B cells to modulate other components of the innate and adaptive immune system. Autoantibody-independent B cell functions include antigen presentation, T cell activation and polarization, and dendritic cell modulation. Several of these functions are mediated by the ability of B cells to produce immunoregulatory cytokines and chemokines and by their critical contribution to lymphoid tissue development and organization including the development of ectopic tertiary lymphoid tissue. Additionally, the functional versatility of B cells enables them to play either protective or pathogenic roles in autoimmunity. In turn, B cell dysfunction has been critically implicated in the pathophysiology of systemic lupus erythematosus (SLE), a complex disease characterized by the production of autoantibodies and heterogeneous clinical involvement. Thus, the breakdown of B cell tolerance is a defining and early event in the disease process and may occur by multiple pathways, including alterations in factors that affect B cell activation thresholds, B cell longevity, and apoptotic cell processing. Once tolerance is broken, autoantibodies contribute to autoimmunity by multiple mechanisms including immune-complex mediated Type III hypersensitivity reactions, type II antibody-dependent cytotoxicity, and by instructing innate immune cells to produce pathogenic cytokines including IFNα, TNF and IL-1. The complexity of B cell functions has been highlighted by the variable success of B cell-targeted therapies in multiple autoimmune diseases, including those conventionally viewed as T cell-mediated conditions. Given the widespread

  16. Ten cases of systemic lupus erythematosus related to hepatitis B vaccine.

    PubMed

    Agmon-Levin, N; Zafrir, Y; Paz, Z; Shilton, T; Zandman-Goddard, G; Shoenfeld, Y

    2009-11-01

    The objective of this article is to identify common and atypical features of systemic lupus erythematosus diagnosed following hepatitis B vaccination. We analyzed retrospectively the medical records of 10 systemic lupus erythematosus patients from different centers, who developed the disease following hepatitis B vaccination and determined the prevalence of different manifestations and the time association to vaccination. In this case series, 80% of the patients were female, mean age 35 +/- 9 years, of which 20% received one inoculation, 20% received two doses and 60% received all three inoculations. The mean latency period from the first hepatitis B virus immunization and onset of autoimmune symptoms was 56.3 days. All patients were diagnosed with systemic lupus erythematosus, according to the American College of Rheumatology revised criteria within 1 year. The prevalence of some systemic lupus erythematosus manifestations was typical and included involvement of the joints (100%), skin (80%), muscles (60%) and photosensitivity (30%). Other symptoms differed in this unique group of systemic lupus erythematosus patients such as low rate of kidney and hematologic involvement, and a relatively high rate of hepatitis (20%). Neurological (80%) and pulmonary (70%) symptoms were also common in this group. Data from this case-series, and previously documented cases in the literature could only show a temporal relation between hepatitis B vaccination and the appearance of systemic lupus erythematosus. Systemic lupus erythematosus related to vaccine may differ from idiopathic systemic lupus erythematosus in its clinical presentation and may resemble drug-induced systemic lupus erythematosus. Thus, physicians should be alerted to this potential association, its possible long latency period and unique presentations, and be encouraged to report and analyze these cases.

  17. Mood Disorders in Systemic Lupus Erythematosus

    PubMed Central

    Hanly, John G.; Su, Li; Urowitz, Murray B.; Romero-Diaz, Juanita; Gordon, Caroline; Bae, Sang-Cheol; Bernatsky, Sasha; Clarke, Ann E.; Wallace, Daniel J.; Merrill, Joan T.; Isenberg, David A.; Rahman, Anisur; Ginzler, Ellen M.; Petri, Michelle; Bruce, Ian N.; Dooley, M. A.; Fortin, Paul; Gladman, Dafna D.; Sanchez-Guerrero, Jorge; Steinsson, Kristjan; Ramsey-Goldman, Rosalind; Khamashta, Munther A.; Aranow, Cynthia; Alarcón, Graciela S.; Fessler, Barri J.; Manzi, Susan; Nived, Ola; Sturfelt, Gunnar K.; Zoma, Asad A.; van Vollenhoven, Ronald F.; Ramos-Casals, Manuel; Ruiz-Irastorza, Guillermo; Lim, S. Sam; Kalunian, Kenneth C.; Inanc, Murat; Kamen, Diane L.; Peschken, Christine A.; Jacobsen, Soren; Askanase, Anca; Theriault, Chris; Thompson, Kara; Farewell, Vernon

    2015-01-01

    Objective To determine the frequency, clinical and autoantibody associations and outcome of mood disorders in a multi-ethnic/racial, prospective, inception cohort of SLE patients. Methods Patients were assessed annually for mood disorders (4 types as per DSM-IV) and 18 other neuropsychiatric (NP) events. Global disease activity (SLEDAI-2K), SLICC/ACR damage index (SDI) and SF-36 subscale, mental (MCS) and physical (PCS) component summary scores were collected. Time to event, linear and ordinal regressions and multi-state models were used as appropriate. Results Of 1,827 SLE patients, 88.9% were female, 48.9% Caucasian, mean ± SD age 35.1±13.3 years, disease duration 5.6±4.8 months and follow-up 4.73±3.45 years. Over the study 863 (47.2%) patients had 1,627 NP events. Mood disorders occurred in 232/1827 (12.7%) patients and 98/256 (38.3%) events were attributed to SLE. The estimated cumulative incidence of any mood disorder after 10 years was 17.7% (95%CI=[15.1%,20.2%]). There was a greater risk of mood disorder in patients with concurrent NP events (p ≤ 0.01) and lower risk with Asian race/ethnicity (p=0.01) and immunosuppressive drugs (p=0.003). Mood disorders were associated with lower mental health subscale and MCS scores but not with SLEDAI-2K, SDI scores or lupus autoantibodies. Antidepressants were used in 168/232 (72.4%) patients with depression. 126/256 (49.2%) mood disorders resolved in 117/232 (50.4%) patients. Conclusion Mood disorders, the second most frequent NP event in SLE patients, have a negative impact on HRQoL and improve over time. The lack of association with global SLE disease activity, cumulative organ damage and lupus autoantibodies emphasize their multifactorial etiology and a role for non-lupus specific therapies. PMID:25778456

  18. Mechanisms of Dyslipoproteinemias in Systemic Lupus Erythematosus

    PubMed Central

    Borba, Eduardo F.; Carvalho, Jozelio F.; Bonfá, Eloísa

    2006-01-01

    Autoimmunity and inflammation are associated with marked changes in lipid and lipoprotein metabolism in SLE. Autoantibodies and cytokines are able to modulate lipoprotein lipase (LPL) activity, a key enzyme in lipid metabolism, with a consequent “lupus pattern” of dyslipoproteinemia characterized by elevated levels of very low-density lipoprotein cholesterol (VLDL) and triglycerides (TG) and lower high-density lipoprotein cholesterol (HDL) levels. This pattern favors an enhanced LDL oxidation with a subsequent deleterious foam cell formation. Autoantibodies and immunocomplexes may aggravate this oxidative injury by inducing accumulation and deposition of oxLDL in endothelial cells. Drugs and associated diseases usually magnify the close interaction of these factors and further promote the proatherogenic environment of this disease. PMID:17162363

  19. Systemic Lupus Erythematosus Presenting as Neuroretinitis.

    PubMed

    Santra, Gouranga; Das, Indrani

    2015-10-01

    Neuroretinitis is the inflammation of retina and optic nerve. It is associated with optic disc edema accompanied by peripapillary or macular hard exudates. A 17 yr old female presented with headache and nausea of five days duration. She had periorbital edema and mild splenomegaly. Neurological assessment was non-contributory. She was found to have pancytopenia, albuminuria and a high ESR. Thereafter she developed blurring of vision of both eyes. Opthalmological examination showed it to be due to bilateral neuroretinitis. ANA and anti-ds DNA were strongly positive. Renal biopsy with immunofluorescence study revealed diffuse global proliferative lupus nephritis with active lesions [class IV-G (A)]. She was diagnosed as a case of SLE presenting with neuroretinitis. PMID:27608700

  20. Guillain–Barré syndrome occurring synchronously with systemic lupus erythematosus as initial manifestation treated successfully with low-dose cyclophosphamide

    PubMed Central

    Ali, Naveed; Rampure, Ritesh; Malik, Faizan; Jafri, Syed Imran Mustafa; Amberker, Deepa

    2016-01-01

    Systemic lupus erythematous (SLE) is frequently encountered in clinical practice; a widespread immunological response can involve any organ system, sometimes leading to rare and diagnostically challenging presentations. We describe a 38-year-old female who presented with symmetric numbness and tingling of the hands and feet, and cervical pain. Imaging studies were not diagnostic of any serious underlying pathology. The patient developed ascending paresis involving lower extremities and cranial muscles (dysphagia and facial weakness). Guillain–Barré syndrome (GBS) was diagnosed on the basis of electromyography and lumbar puncture showing albuminocytologic dissociation. Intravenous immunoglobulins (IVIG) were administered for 5 days. Supported by anti-dsDNA antibody, oral ulcers, proteinuria of 0.7 g in 24 h, and neurological manifestation, she was diagnosed with lupus. After completion of IVIG, she received pulse-dose corticosteroids and one dose of low-dose cyclophosphamide. Her neurological symptoms improved and she had complete neurological recovery several months after her initial presentation. Literature search provides evidence of co-occurrence of lupus and GBS occurring mostly later in the course of the disease. However, GBS as initial manifestation of SLE is exceedingly rare and less understood. The association of GBS with lupus is important to recognize for rapid initiation of appropriate therapy and for consideration of immunosuppressive therapy which may affect the outcome. PMID:27124163

  1. [Anticardiolipin antibodies in patients with systemic lupus erythematosus].

    PubMed

    Petrović, R; Petrović, M; Novicić-Sasić, D; Damjanov, N

    1994-01-01

    The aim of the study was to determine the prevalence and to evaluate clinical significance of anticardiolipin antibodies in cohort of 60 patients with systemic lupus erythematosus. The measurement of autoantibodies was carried out by standardized ELISA method using MELISA anticardiolipin IgG and IgM kits (Walker Diagnostics, Cambridgeshire, UK) A positive result indicated a value in GPL or MPL U/ml more than 3 SD above the mean value obtained with control sera of 48 healthy pearsons. IgG isotype alone, and both isotupe of anticardiolipin antibodies were found in 30 percent, in 6,7 percent and in 11,7 percent of patients, respectively. High or medium levels of IgG anticardiolipin antibodies were found in all 6 patients with actual venous or arterial thrombosis, but in only 3 out of 10 patients with history of thromboembolic features. All 6 patients with actual thrombocytopenia and 3 female with recent spontaneus abortion also had elevated levels of the same isotype. Total anticardiolipin antibodies (IgG and IgM) were significantly associated with recent or history of thrombocytopenia. In conclusion, we emphasize the association of IgG anticardiolipin antibodies with recent events of antiphospholipid syndrome in patients with systemic lupus erythematosus. PMID:18173204

  2. Severe Coronary Spasm in Systemic Lupus Erythematosus Resulting in Recurrent Occlusions and Guide Wire Fracture.

    PubMed

    Lai, Chih-Hung; Lu, Tse-Min; Juan, Yu-Hsiang; Chang, Szu-Ling; Lee, Wen-Lieng; Sung, Shih-Hsien

    2016-07-01

    Middle-aged female patients with systemic lupus erythematosus (SLE) have an increased risk of coronary artery disease and myocardial infarction (MI). We report a case of left anterior descending coronary artery (LAD) MI associated with severe coronary spasm in both the LAD and left circumflex artery, complicated with fracture of the distal wire within the microcatheter which was successfully removed by manual aspiration using an inflation device. From this series of rare complications of SLE with MI, severe coronary spasm and guide wire fracture, we underscore that clinicians performing coronary intervention should be aware of an elevated chance of possible severe coronary spasms in SLE patients. PMID:27471364

  3. Pregnancy-related issues in women with systemic lupus erythematosus.

    PubMed

    Singh, Abha G; Chowdhary, Vaidehi R

    2015-02-01

    While fertility is preserved in females with systemic lupus erythematosus (SLE), it is well established that pregnancy in these patients is associated with adverse maternal and fetal outcomes, including pregnancy loss, pre-eclampsia, preterm delivery and intrauterine growth retardation, as well as neonatal mortality. Mechanisms underlying these adverse outcomes are poorly understood, and better understanding of these would allow development of targeted and personalized treatment strategies. Established risk factors for adverse pregnancy outcomes include active disease within 6 months prior to conception and during pregnancy, active nephritis, maternal hypertension, antiphospholipid antibodies and hypocomplementemia. While intensive monitoring is recommended, the comparative effectiveness of appropriate management strategies is unclear. While current strategies are able to achieve live births in 85-90% of pregnancies, certain aspects such as prevention of preterm birth, treatment of congenital heart block due to neonatal lupus and recurrent pregnancy loss despite best management, remains challenging. Pregnancy is also associated with an increased risk of flare of lupus, particularly in patients with active disease at time of conception or within 6 months prior to conception. Pregnant patients with SLE should be followed in a high-risk obstetric clinic, and care should be closely coordinated between the obstetrician and rheumatologist. PMID:25545844

  4. Visceral leishmaniasis in a patient with systemic lupus erythematosus

    PubMed Central

    Santos Silva, André Filipe; Figueiredo Dias, João Paulo Branco Calheiros; Nuak, João Miguel Neves Gonçalves Santos; Rocha Aguiar, Francisca; Araújo Pinto, José António; Sarmento, António Carlos Eugénio Megre

    2015-01-01

    Visceral leishmaniasis is an infection with an insidious and disabling course caused by parasites of the genus Leishmania. In Europe, it is mostly associated with HIV infection. Systemic lupus erythematosus and its treatment are associated with increased risk of infection, neoplastic and concomitant autoimmune disorders. The association of these diseases may go unnoticed. A 60 year-old Caucasian woman with lupus presented with a one-year history of fever, malaise, weakness and weight loss. The highlights on physical examination were pallor, palpable hepatosplenomegaly and low-grade fever. Blood tests showed pancytopenia, hyperproteinemia with hypoalbuminemia and hypergammaglobulinemia; electrophoresis showed a polyclonal gamma curve. Full-body CT scan revealed massive hepatosplenomegaly. Microbiology investigation was negative for the most common pathogens, including tuberculosis. There were no signs of hematologic malignancy in the bone marrow smear. PCR for Leishmania infantum was positive both in blood and bone marrow. The patient was treated with liposomal amphotericin B, and immunosuppression was adjusted. She showed rapid clinical improvement and 6 months later had no signs of disease. The differential diagnosis in a patient with lupus presenting with fever and multisystemic manifestations includes infectious or neoplastic disorders. The patient lived in an endemic area of Leishmania, and typical clinical and analytical changes were all present, making this case highly educational. The case highlights the importance of a patient's epidemiological background and how it can lead to the diagnosis and timely treatment of a rare disease. PMID:26793472

  5. Orthopedic surgery and its complication in systemic lupus erythematosus

    PubMed Central

    Mak, Anselm

    2014-01-01

    Systemic lupus erythematosus (SLE) is a multi-systemic immune-complex mediated autoimmune condition which chiefly affects women during their prime year. While the management of the condition falls into the specialty of internal medicine, patients with SLE often present with signs and symptoms pertaining to the territory of orthopedic surgery such as tendon rupture, carpal tunnel syndrome, osteonecrosis, osteoporotic fracture and infection including septic arthritis, osteomyelitis and spondylodiscitis. While these orthopedic-related conditions are often debilitating in patients with SLE which necessitate management by orthopedic specialists, a high index of suspicion is necessary in diagnosing these conditions early because lupus patients with potentially severe orthopedic conditions such as osteomyelitis frequently present with mild symptoms and subtle signs such as low grade fever, mild hip pain and back tenderness. Additionally, even if these orthopedic conditions can be recognized, complications as a result of surgical procedures are indeed not uncommon. SLE per se and its various associated pharmacological treatments may pose lupus patients to certain surgical risks if they are not properly attended to and managed prior to, during and after surgery. Concerted effort of management and effective communication among orthopedic specialists and rheumatologists play an integral part in enhancing favorable outcome and reduction in postoperative complications for patients with SLE through thorough pre-operative evaluation, careful peri-operative monitoring and treatment, as well as judicious postoperative care. PMID:24653977

  6. Acute acalculous cholecystitis in systemic lupus erythematosus: a rare initial manifestation.

    PubMed

    Manuel, Valdano; Pedro, Gertrudes Maria; Cordeiro, Lemuel Bornelli; de Miranda, Sandra Maria da Rocha Neto

    2016-01-01

    Acute acalculous cholecystitis is a very rare gastrointestinal manifestation in systemic lupus erythematosus and becomes rarer as an initial manifestation. There are only two cases reported. The authors report a 20-year-old black woman that presented acute acalculous cholecystitis revealed by abdominal computed tomography. During hospitalization, she was diagnosed systemic lupus erythematosus. Conservative treatment with antibiotics was performed with complete remission of the symptoms. Corticosteroid was started in ambulatory. Cholecystectomy has been the treatment of choice in acute acalculous cholecystitis as a complication of systemic lupus erythematosus. The patient responded well to conservative treatment, and surgery was not required. This case is unique in the way that corticosteroid was started in ambulatory care. We should not forget that the acute acalculous cholecystitis can be the initial presentation of systemic lupus erythematosus although its occurrence is very rare. Conservative treatment should be considered. Abdominal computed tomography was a determinant exam for better assessment of acute acalculous cholecystitis. PMID:27267533

  7. Acute acalculous cholecystitis in systemic lupus erythematosus: a rare initial manifestation.

    PubMed

    Manuel, Valdano; Pedro, Gertrudes Maria; Cordeiro, Lemuel Bornelli; de Miranda, Sandra Maria da Rocha Neto

    2016-01-01

    Acute acalculous cholecystitis is a very rare gastrointestinal manifestation in systemic lupus erythematosus and becomes rarer as an initial manifestation. There are only two cases reported. The authors report a 20-year-old black woman that presented acute acalculous cholecystitis revealed by abdominal computed tomography. During hospitalization, she was diagnosed systemic lupus erythematosus. Conservative treatment with antibiotics was performed with complete remission of the symptoms. Corticosteroid was started in ambulatory. Cholecystectomy has been the treatment of choice in acute acalculous cholecystitis as a complication of systemic lupus erythematosus. The patient responded well to conservative treatment, and surgery was not required. This case is unique in the way that corticosteroid was started in ambulatory care. We should not forget that the acute acalculous cholecystitis can be the initial presentation of systemic lupus erythematosus although its occurrence is very rare. Conservative treatment should be considered. Abdominal computed tomography was a determinant exam for better assessment of acute acalculous cholecystitis.

  8. Insidious bilateral infrapatellar tendon rupture in a patient with systemic lupus erythematosus.

    PubMed Central

    Cooney, L M; Aversa, J M; Newman, J H

    1980-01-01

    A patient with systemic lupus erythematosus developed insidious bilateral infrapatellar tendon rupture initially diagnosed as steroid myopathy. Simultaneous loss of extension at the knee due to quadriceps or infrapatellar tendon ruptures is reviewed. Images PMID:7458438

  9. BAFF/BLyS inhibitors: A new prospect for treatment of systemic lupus erythematosus.

    PubMed

    Fairfax, Kirsten; Mackay, Ian R; Mackay, Fabienne

    2012-07-01

    In November 2009, Human Genome Sciences and Glaxo-Smith Kline [HGS (Rockville, Maryland) and GSK, respectively] announced that Belimumab, a neutralizing antibody to the tumour necrosis factor (TNF)-like ligand, B-cell activating factor (BAFF belonging to the TNF family, also named BLyS), met the primary endpoints in two phase III clinical trials in systemic lupus erythematosus (SLE, lupus). In March 2011, Belimumab was approved by the US Federal Drug Agency for treatment of SLE patients; this was followed in May with approval by the European Medicines Agency for use in the European Union. This is an exciting development as it is the first successful late-stage clinical trial in SLE in over 40 years. In the light of this breakthrough, we review the key data and research outcomes and examine how blocking BAFF in patients with SLE significantly improves clinical outcomes.

  10. Non-invasive imaging to monitor lupus nephritis and neuropsychiatric systemic lupus erythematosus

    PubMed Central

    Thurman, Joshua M.; Serkova, Natalie J.

    2015-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple different organs, including the kidneys and central nervous system (CNS). Conventional radiological examinations in SLE patients include volumetric/ anatomical computed tomography (CT), magnetic resonance imaging (MRI) and ultrasound (US). The utility of these modalities is limited, however, due to the complexity of the disease. Furthermore, standard CT and MRI contrast agents are contraindicated in patients with renal impairment. Various radiologic methods are currently being developed to improve disease characterization in patients with SLE beyond simple anatomical endpoints. Physiological non-contrast MRI protocols have been developed to assess tissue oxygenation, glomerular filtration, renal perfusion, interstitial diffusion, and inflammation-driven fibrosis in lupus nephritis (LN) patients. For neurological symptoms, vessel size imaging (VSI, an MRI approach utilizing T2-relaxing iron oxide nanoparticles) has shown promise as a diagnostic tool. Molecular imaging probes (mostly for MRI and nuclear medicine imaging) have also been developed for diagnosing SLE with high sensitivity, and for monitoring disease activity. This paper reviews the challenges in evaluating disease activity in patients with LN and neuropsychiatric systemic lupus erythematosus (NPSLE). We describe novel MRI and positron-emission tomography (PET) molecular imaging protocols using targeted iron oxide nanoparticles and radioactive ligands, respectively, for detection of SLE-associated inflammation. PMID:26309728

  11. Anti-dsDNA Antibodies are one of the many autoantibodies in systemic lupus erythematosus

    PubMed Central

    Fu, Shu Man; Dai, Chao; Zhao, Zhenhuan; Gaskin, Felicia

    2015-01-01

    Anti-dsDNA antibodies are the most studied antibodies of the lupus-related autoantibodies. The dogma is that these are the most important autoantibodies in systemic lupus erythematosus. In this review, evidence is presented to show that these antibodies (as measured by modern clinical laboratories) are not the most important autoantibodies in the diagnosis of systemic lupus erythematosus, and are of limited value in clinical correlation and in predicting disease flares. In addition, they are not likely to be the initiating autoantibodies in lupus nephritis. Thus, several pervasively held beliefs on anti-dsDNA antibodies are not valid. We suggest that anti-dsDNA antibodies should be considered as just one of the many autoantibodies associated with systemic lupus erythematosus. PMID:26594353

  12. Sjögren’s syndrome associated with systemic lupus erythematosus

    PubMed Central

    Taşdemir, Mehmet; Hasan, Chiar; Ağbaş, Ayşe; Kasapçopur, Özgür; Canpolat, Nur; Sever, Lale; Çalışkan, Salim

    2016-01-01

    Systemic lupus erythematosus and Sjögren’s syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren’s syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren’s syndrome for six years and then was observed to have overlap of systemic lupus erythematosus. In the baseline, she did not have any clinical or serological evidence for systemic lupus erythematosus. After six year, massive proteinuria and serological findings developed and systemic lupus erythematosus nephritis was diagnosed by kidney biopsy. Currently, systemic lupus erythematosus and Sjögren’s syndrome cannot be differentiated definetely. We need more valuable diagnostic and classification criteria to differentiate these two important conditions. PMID:27738403

  13. Curcumin aggravates CNS pathology in experimental systemic lupus erythematosus.

    PubMed

    Foxley, Sean; Zamora, Marta; Hack, Bradley; Alexander, Rebecca Rashmi; Roman, Brian; Quigg, Richard John; Alexander, Jessy John

    2013-04-01

    Complement activation and inflammation are key disease features of systemic lupus erythematosus. Curcumin is an anti-inflammatory agent that inhibits the complement cascade. Therefore, we hypothesized that curcumin will be protective in CNS lupus. To assess the effect of curcumin on CNS-lupus, MRL/lpr mice were used. Brain MRI showed that curcumin (30mg/kg body wt. i.p. from 12-20 weeks) worsened regional brain atrophy. The volumes of the lateral and third ventricles are significantly increased (150%-213% and 107%-140%, without and with treatment respectively compared to MRL+/+ controls). The hippocampus was reduced further (83%-81%) by curcumin treatment. In line with increased brain atrophy, there were edematous cells (41% increase in cell size in MRL/lpr compared to MRL+/+ mice. The cell size was further increased by 28% when treated with curcumin; p<0.02) in the cortex. In line with increased atrophy and edema, there was a significant increase (p<0.02) in the mRNA and protein expression of the water channel protein, aquaporin 4 in these mice. The increase in the matrix proteins, glial fibrillary acidic protein and vimentin in lupus mice in the hippocampus was prevented by curcumin. Curcumin increased IgG deposits and decreased C3 deposits in brain with a corresponding increase in immune complexes and decrease in C3 concentration (by 60% in MRL/lpr mice Vs. MRL+/+ mice and a further 26% decrease when treated with curcumin) in circulation. Decrease in C3 could alter the transport of immune complexes leading to an increase in IgG deposits which could induce inflammatory pathways thereby leading to worsening of the disease. The neurological outcome as measured by maze performance indicates that the curcumin treated mice performed poorly compared to the untreated counterparts. Our results for the first time provide evidence that at the dose used in this study, curcumin aggravates some CNS disease manifestations in experimental lupus brain. Therefore, until a safe

  14. A severe case of systemic lupus erythematosus with cerebral involvement.

    PubMed

    Wiedmann, M; Seidel, W; Mende, L; Petros, S; Engelmann, L

    2004-11-01

    We describe an 18-year-old girl who presented with severe systemic lupus erythematosus with multiple organ involvement. The disease was further complicated by recurrent seizures and intracerebral left parieto-occipital bleeding that required neurosurgical treatment. Postoperative rebleeding occurred due to disseminated intravascular coagulation and platelet dysfunction. Catastrophic antiphospholipid syndrome was suspected, but could not be confirmed during follow-up. Additional treatment with plasmapheresis and intravenous pulse cyclophosphamide in combination with corticosteroids was started. Liquor drainage via a ventriculo-peritoneal (vp)-shunt was necessary because of a hydrocephalus malresorptivus. The patient's recovery was slow and incomplete (cachexia and amaurosis persisted). Follow-up was further complicated by an intraperitoneal vp-shunt cyst, which was initially treated conservatively, but finally had to be revised operatively.

  15. The need to define treatment goals for systemic lupus erythematosus.

    PubMed

    Franklyn, Kate; Hoi, Alberta; Nikpour, Mandana; Morand, Eric F

    2014-09-01

    In the current therapeutic climate, mortality rates from systemic lupus erythematosus (SLE) remain unacceptably high. Although new therapies are on the horizon, pending their emergence and availability, optimization of the currently available therapies is potentially achievable. A 'treat-to-target' approach is now considered routine for many diseases, including rheumatoid arthritis, for which it has substantially improved patient outcomes. The heterogeneity of SLE, as well as lack of universal agreement over methods to measure disease activity and treatment responses, has impeded the development of such an approach for this disease. In this article, the potential benefits of a treatment-target definition are explored, obstacles to the development of a treatment target in SLE are identified, and possible strategies to achieve this goal are discussed.

  16. The evolution of drug discovery in systemic lupus erythematosus.

    PubMed

    Wallace, Daniel J

    2015-10-01

    Drug discovery in systemic lupus erythematosus (SLE) has lagged behind other rheumatic diseases, in large part because of difficulty in measuring change or improvement in a disorder that involves multiple organ systems to varying degrees at different times. The metrics currently used as primary endpoints are composite indices that rely mainly on disease assessment measures derived before the era of clinical trials of targeted therapies. Only one agent has been approved for the treatment of SLE since 1957. This monograph reviews the evolution of drug development for SLE, problems and pitfalls that have been encountered, and outlines the domains used to evaluate SLE in the clinic. Finally, several initiatives underway to improve clinical trial design are outlined.

  17. Imaging of cardiovascular complications in patients with systemic lupus erythematosus

    PubMed Central

    Lin, Kai; Lloyd-Jones, Donald M.; Li, Debiao; Liu, Ying; Yang, Jie; Markl, Michael; Carr, James C.

    2015-01-01

    In the long-term survivals of patients with systemic lupus erythematosus (SLE), cardiovascular disease (CVD) is a leading cause of death. Recently, multi-modality cardiovascular imaging methods have been adopted for the evaluation of cardiovascular risk, which has shown to be associated with both traditional cardiovascular risk factors and SLE-specific conditions. Quantitative imaging biomarkers, which can describe both morphological and functional abnormalities in the heart, are expected to provide new insights to stratify cardiovascular risks and to guide SLE management by assessing the individual responses to therapies either protecting the cardiovascular system or suppressing the autoimmune reactions. In this review, we will discuss cutting-edge cardiovascular imaging techniques and discuss potential clinical applications and limitations of those techniques for the evaluation of major SLE related heart disorders. PMID:26038342

  18. The rationale for BAFF inhibition in systemic lupus erythematosus.

    PubMed

    Davidson, Anne

    2012-08-01

    BAFF (B-cell-activating factor) is a critical survival factor for transitional and mature B cells and is a promising therapeutic target for systemic lupus erythematosus (SLE). In 2010-2011, two phase 3 clinical trials showed that the addition of the anti-BAFF antibody belimumab to standard-of-care therapy in patients with moderately active SLE results in a better outcome at 52 weeks than standard-of-care therapy alone. Belimumab has been US Food and Drug Administration approved for the treatment of SLE, and other drugs that target BAFF are now in various stages of clinical testing. This review describes the function of BAFF and its homolog APRIL (a proliferation-inducing ligand) and addresses the rationale for the treatment of SLE with BAFF/APRIL inhibitors.

  19. The role of CD40 ligand in systemic lupus erythematosus.

    PubMed

    Yazdany, J; Davis, J

    2004-01-01

    CD40 ligand (CD40L, also known as CD154 or gp39) is a member of the tumor necrosis superfamily of transmembrane proteins. The interaction of CD40L on activated T cells with its receptor, CD40 on B cells, is necessary for normal immune function, including B cell differentiation, germinal center formation, and antibody isotype switching. Abnormal expression of CD40L in patients with systemic lupus erythematosus (SLE) may contribute to autoantibody production and disease pathogenesis. Although murine models of monoclonal antibodies directed against CD40L initially showed promise, human trials either have failed to demonstrate efficacy or have been associated with adverse events. This review will summarize in vitro and murine model data and human clinical trials involving anti-CD40L monoclonal antibody.

  20. Antibody-based therapies in systemic lupus erythematosus.

    PubMed

    Iikuni, Noriko; La Cava, Antonio

    2009-06-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that is characterized by pathologic manifestations in multiple organs and elevated morbidity. Traditional management of SLE has included the use of non-steroidal anti-inflammatory drugs, anti-malarials and immunosuppressive drugs such glucocorticoids, azathioprine, cyclophosphamide, and mycophenolate mofetil. Although many of these therapies have shown great efficacy, they often associate with adverse effects, due to their systemic activity. The development of safer therapies for SLE has led to recent emphasis on targeting selected pathways that can be important in the inflammatory response in SLE. In this context, the use of biological agents such as monoclonal antibodies has seen a rapidly increasing progress, and is poised to be some part of the clinical practice for SLE in a near future. This review provides an update on the ongoing clinical trials and the promise and obstacles in the use of biologics in SLE.

  1. Cell-based therapies for systemic lupus erythematosus.

    PubMed

    Liao, Jieyue; Chang, Christopher; Wu, Haijing; Lu, Qianjin

    2015-01-01

    Systemic lupus erythematosus (SLE) is a female predominant autoimmune disease characterized by multi-organ disorders. The pathogenesis of SLE is complex. Corticosteroids and immunosuppressive drugs are widely used to treat patients with SLE. However, these indiscriminate suppressors of the immune-mediated inflammatory aberration treat SLE at the cost of considerable adverse effects. Undoubtedly, there is a need for safer and more effective treatments for SLE. Cell-based therapies, although very much in their infancy, are of increasing interest in the treatment of SLE due to their potential for long-term suppression or a possible cure of the disease. Several immunoregulatory cell types, including regulatory T cells, mesenchymal stem cells, B-cells and natural killer cells, have recently been developed as novel products for tolerance-promoting therapies. Here, we provide a brief overview of current research of new cell-based therapeutic approaches that have undergone pre-clinical or clinical trials in the treatment of SLE.

  2. [Ischemic colitis: an uncommon manifestation in systemic lupus erythematosus].

    PubMed

    Medina, Viviana; Bulgach, Valeria; Lagandara, Pamela; Berner, Enrique

    2013-04-01

    We present the case of an adolescent with ischemic colitis, an infrequent pathology in this age group, worsened in the presence of systemic lupus erythematosus (SLE). The patient, aged 20, was diagnosed SLE at 6. She consulted for fever, abdominal pain in the side and right iliac fossa and diarrhea lasting 48 hours. It was assumed as acute gastroenteritis but given the persistent pain, incoercible vomiting and abdominal distension she was hospitalized. The abdominal X-ray showed distended loops, abundant feces, without air-fluid levels. The ultrasound showed erosions and ulcerations, edema and bleeding in the descending colon submucosal layer. The CT scan evidenced an ischemic lesion in the right colon. Ischemic colitis is a severe condition, infrequent in young individuals. Signs, symptoms, abdominal CT scan and colonoscopy are the elements of choice for the diagnosis.

  3. Immunoregulation of NKT Cells in Systemic Lupus Erythematosus.

    PubMed

    Chen, Junwei; Wu, Meng; Wang, Jing; Li, Xiaofeng

    2015-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with different variety of clinical manifestations. Natural killer T (NKT) cells are innate lymphocytes that play a regulatory role during broad range of immune responses. A number of studies demonstrated that the quantity and quality of invariant NKT (iNKT) cells showed marked defects in SLE patients in comparison to healthy controls. This finding suggests that iNKT cells may play a regulatory role in the occurrence and development of this disease. In this review, we mainly summarized the most recent findings about the behavior of NKT cells in SLE patients and mouse models, as well as how NKT cells affect the proportion of T helper cells and the production of autoreactive antibodies in the progress of SLE. This will help people better understand the role of NKT cells in the development of SLE and improve the therapy strategy. PMID:26819956

  4. [Ischemic colitis: an uncommon manifestation in systemic lupus erythematosus].

    PubMed

    Medina, Viviana; Bulgach, Valeria; Lagandara, Pamela; Berner, Enrique

    2013-04-01

    We present the case of an adolescent with ischemic colitis, an infrequent pathology in this age group, worsened in the presence of systemic lupus erythematosus (SLE). The patient, aged 20, was diagnosed SLE at 6. She consulted for fever, abdominal pain in the side and right iliac fossa and diarrhea lasting 48 hours. It was assumed as acute gastroenteritis but given the persistent pain, incoercible vomiting and abdominal distension she was hospitalized. The abdominal X-ray showed distended loops, abundant feces, without air-fluid levels. The ultrasound showed erosions and ulcerations, edema and bleeding in the descending colon submucosal layer. The CT scan evidenced an ischemic lesion in the right colon. Ischemic colitis is a severe condition, infrequent in young individuals. Signs, symptoms, abdominal CT scan and colonoscopy are the elements of choice for the diagnosis. PMID:23568076

  5. Treat to target in systemic lupus erythematosus: a commentary.

    PubMed

    Ugarte-Gil, Manuel F; Burgos, Paula I; Alarcón, Graciela S

    2016-08-01

    Treat to target (T2T) strategies have proved to be useful in several chronic disorders, including Rheumatoid Arthritis. In systemic lupus erythematosus (SLE), T2T strategy has been proposed in order to control disease activity, improve health-related quality of life, and reduce morbidity and mortality. Remission would be the main target, but a low disease activity state (LDAS) could be an acceptable alternative. However, due to SLE protean manifestations, the operational definitions of both remission and LDAS are still in progress. The definitions of these targets, remission and LDAS, should include a validated disease activity index, the treatments allowed, and the minimum length of time the target should be maintained. Furthermore, achieving these targets should result in better disease outcomes such as reducing damage accrual. This review addresses the current state regarding these possible targets in SLE and the impact of achieving them in intermediate and long-term outcomes of this disease. PMID:27406378

  6. Vitamin D and systemic lupus erythematosus: an update.

    PubMed

    Mok, Chi Chiu

    2013-05-01

    Vitamin D is a steroid hormone that, in addition to its actions on calcium and bone metabolism, exhibits a plethora of regulatory effects on growth, proliferation, apoptosis and function of the cells of the immune system that are relevant to the pathophysiology of systemic lupus erythematosus (SLE). Hypovitaminosis D is highly prevalent in SLE as a result of avoidance of sunshine, photoprotection, renal insufficiency and the use of medications such as glucocorticoids, anticonvulsants, antimalarials and the calcineurin inhibitors, which alter the metabolism of vitamin D or downregulate the functions of the vitamin D receptor. Low levels of vitamin D correlate with disease activity, and is associated with osteoporosis, fatigue and certain cardiovascular risk factors in SLE patients. This review updates the recent evidence on the relationship between vitamin D status and the onset, activity and complications of SLE, and summarizes the recommendations for vitamin D supplementation.

  7. Imaging of cardiovascular complications in patients with systemic lupus erythematosus.

    PubMed

    Lin, K; Lloyd-Jones, D M; Li, D; Liu, Y; Yang, J; Markl, M; Carr, J C

    2015-10-01

    In the long-term survival of patients with systemic lupus erythematosus (SLE), cardiovascular disease (CVD) is a leading cause of death. Recently, multimodality cardiovascular imaging methods have been adopted for the evaluation of cardiovascular risk, which has shown to be associated with both traditional cardiovascular risk factors and SLE-specific conditions. Quantitative imaging biomarkers, which can describe both morphological and functional abnormalities in the heart, are expected to provide new insights to stratify cardiovascular risks and to guide SLE management by assessing individual responses to therapies either protecting the cardiovascular system or suppressing the autoimmune reactions. In this review, we will discuss cutting-edge cardiovascular imaging techniques and potential clinical applications and limitations of those techniques for the evaluation of major SLE-related heart disorders. PMID:26038342

  8. Monocyte enhancers are highly altered in systemic lupus erythematosus

    PubMed Central

    Shi, Lihua; Zhang, Zhe; Song, Li; Leung, Yiu Tak; Petri, Michelle A; Sullivan, Kathleen E

    2015-01-01

    Objective: Histone modifications set transcriptional competency and can perpetuate pathologic expression patterns. We defined systemic lupus erythematosus (SLE)-specific changes in H3K4me3 and K3K27me3, histone marks of gene activation and repression, respectively. Methods: We used ChIP-seq to define histone modifications in monocytes from SLE patients and controls. Results: Both promoters and enhancers exhibited significant changes in histone methylation in SLE. Regions with differential H3K4me3 in SLE were significantly enriched in potential interferon-related transcription factor binding sites and pioneer transcription factor sites. Conclusion: Enhancer activation defines the character of the cell and our data support extensive disease effects in monocytes, a particularly plastic lineage. Type I interferons not only drive altered gene expression but may also alter the character of the cell through chromatin modifications. PMID:26442457

  9. Immunoregulation of NKT Cells in Systemic Lupus Erythematosus

    PubMed Central

    Chen, Junwei; Wu, Meng; Wang, Jing; Li, Xiaofeng

    2015-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with different variety of clinical manifestations. Natural killer T (NKT) cells are innate lymphocytes that play a regulatory role during broad range of immune responses. A number of studies demonstrated that the quantity and quality of invariant NKT (iNKT) cells showed marked defects in SLE patients in comparison to healthy controls. This finding suggests that iNKT cells may play a regulatory role in the occurrence and development of this disease. In this review, we mainly summarized the most recent findings about the behavior of NKT cells in SLE patients and mouse models, as well as how NKT cells affect the proportion of T helper cells and the production of autoreactive antibodies in the progress of SLE. This will help people better understand the role of NKT cells in the development of SLE and improve the therapy strategy. PMID:26819956

  10. [Depression as a common complication of systemic lupus erythematosus].

    PubMed

    Lemaire, B; Geron, D; Malaise, O; Krzesinski, J M; Ansseau, M; Scantamburlo, G

    2015-04-01

    Systemic lupus erythematosus (SLE) is an inflammatory disease with multiple and disabling consequences, including the psychological status. The prevalence of major depressive episodes among patients suffering from SLE is significantly higher than in healthy people, or people suffering from other inflammatory diseases. While it is obvious that its chronic disease status with a frequently pejorative ending, as well as the number of treatments it requires, are contributing factors, it is likely that due to its pathogenic mechanisms, SLE causes direct injury to the brain, leading to a depressive symptomatology. Numerous hypotheses are under consideration. We shall review them all, recall a few epidemiologic features, add histology and medical imaging contributions and discuss the importance of setting up a fitting therapy for such patients. PMID:26054174

  11. Therapy of systemic lupus erythematosus: a look into the future.

    PubMed

    Smolen, Josef S

    2002-01-01

    The prognosis for patients with systemic lupus erythematosus has greatly improved over the past two decades. However, therapies that are more effective and that have fewer sequelae are needed to rescue patients from organ failure and further reduce mortality. Research under way, including that into induction of tolerance to self-antigens, prevention of the consequences of pathogenic autoantibody production, interference with the cytokine network and signal transduction, the identification and treatment of any infectious triggers, and stem cell therapy, offers hope of improved remedies or even of cure. Given the fact that a number of biological therapies for rheumatologic disease are already in use or are in the development stage, such progress may come soon. PMID:12110120

  12. Accelerated vascular disease in systemic lupus erythematosus: role of macrophage.

    PubMed

    Al Gadban, Mohammed M; Alwan, Mohamed M; Smith, Kent J; Hammad, Samar M

    2015-04-01

    Atherosclerosis is a chronic inflammatory condition that is considered a major cause of death worldwide. Striking phenomena of atherosclerosis associated with systemic lupus erythematosus (SLE) is its high incidence in young patients. Macrophages are heterogeneous cells that differentiate from hematopoietic progenitors and reside in different tissues to preserve tissue integrity. Macrophages scavenge modified lipids and play a major role in the development of atherosclerosis. When activated, macrophages secret inflammatory cytokines. This activation triggers apoptosis of cells in the vicinity of macrophages. As such, macrophages play a significant role in tissue remodeling including atherosclerotic plaque formation and rupture. In spite of studies carried on identifying the role of macrophages in atherosclerosis, this role has not been studied thoroughly in SLE-associated atherosclerosis. In this review, we address factors released by macrophages as well as extrinsic factors that may control macrophage behavior and their effect on accelerated development of atherosclerosis in SLE. PMID:25638414

  13. Tuberculosis in patients with systemic lupus erythematosus: Spain's situation.

    PubMed

    Arenas Miras, María del Mar; Hidalgo Tenorio, Carmen; Jimenez Alonso, Juan

    2013-01-01

    There has recently been an increase in the incidence of patients with systemic lupus erythematosus (SLE) due mainly to earlier diagnosis, and increased survival. Tuberculosis in our country is one of the most prevalent infectious diseases, and one of the underlying causes would be HIV infection and increased immigration from areas with high tuberculosis prevalence; this phenomenon is truly important in patients with autoimmune diseases, as clinical presentation, severity and prognosis of tuberculosis are often different to that of immunocompetent patients. Studies of tuberculosis in patients with SLE are scarce and inconclusive, with many doubts existing about the performance or non-tuberculous prophylaxis in this population and the absence of a protocol due to lack of conclusive studies. New techniques for diagnosis of tuberculosis (IGRAs) may be useful in this population due to higher sensitivity than Mantoux, helping avoid false negatives.

  14. Lichen Planopilaris Versus Discoid Lupus Erythematosus: A Trichoscopic Perspective

    PubMed Central

    Ankad, Balachandra S; Beergouder, Savitha L; Moodalgiri, Vishnu M

    2013-01-01

    Trichoscopy enables visualization of subsurface structures and color patterns of scalp and hair. Recently, its applications expanded to diagnose inflammatory conditions such as lichen planopilaris (LPP), scalp psoriasis, and discoid lupus erythematosus (DLE). Clinically, both LPP and DLE appear similar as cicatricial alopecia on the scalp making the diagnosis difficult. Here, we report the utility of trichoscopy in the clinical diagnosis of LPP and DLE. Clinically, suspected lesions of DLE and LPP on the scalp of each patient were observed under trichoscopy. Histopathology of lesions confirmed the clinical diagnosis. Authors observed characteristic trichoscopic features in LPP as well as in DLE proving the clinical diagnosis. Hence, trichoscopy can be used to diagnose LPP and DLE clinically avoiding skin biopsy. PMID:24778533

  15. [A case of skin cryptococcosis in systemic lupus erythematosus].

    PubMed

    Halweg, H; Korzeniewska-Koseła, M; Podsiadło, B; Krakówka, P

    1990-01-01

    Here is presented a case of woman treated by immunosuppressive preparations because of systemic lupus erythematosus with ski manifestations as tubercles and ulcerations on skin of trunk and extremities. On the basis of histological examination of tubercle skin specimens and mycological examinations of material obtained from skin ulcerations cryptococcosis was diagnosed. Disease was limited to skin that was an entry of infection. Patient was treated by Amphotericin B administered intravenously and Flucitosine per os. Amphotericin B was also applied topically. The results of cultures became gradually negative, up to total disappearance of fungus cells in direct specimens, prepared from examined material. After treatment continuing for 5 months only discoloured scars were observed on sick skin.

  16. Anti-endothelial antibodies and neuropsychiatric systemic lupus erythematosus.

    PubMed

    Valesini, Guido; Alessandri, Cristiano; Celestino, Domenico; Conti, Fabrizio

    2006-06-01

    The pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) has been attributed to autoantibody-mediated neural dysfunction, vasculopathy, and coagulopathy. Several autoantibodies specificities have been reported in serum and cerebrospinal fluid of NPSLE patients (i.e., antineuronal, antiribosomal P proteins, antiglial fibrillary acidic proteins, antiphospholipid, and anti-endothelial antibodies). We have recently demonstrated an association between serum anti-endothelial antibodies and psychosis or depression in patients with SLE. Subsequently, by screening a cDNA library from human umbilical artery endothelial cells with serum from a SLE patient with psychosis, one positive strongly reactive clone was identified encoding the C-terminal region (C-ter) of Nedd5, an intracytoplasmatic protein of the septin family. Anti-Nedd5 antibodies have been found significantly associated with psychiatric manifestations in SLE patients, strengthening the view of a possible implication of autoantibodies in the development of psychiatric disorders.

  17. [Skin manifestations in lupus erythematosus: clinical aspects and therapy].

    PubMed

    Kuhn, A; Ruland, V; Bonsmann, G

    2011-04-01

    Lupus erythematosus (LE) is an inflammatory autoimmune disorder, which is characterized by clinically heterogeneous manifestations of different organs. In systemic LE (SLE) the skin, the musculoskeletal system, the kidneys, the cardiovascular and central nervous systems can be involved. The skin lesions can be divided into LE-specific and LE-non-specific manifestations, the former represent the subtypes of cutaneous LE (CLE). The diagnosis is confirmed by clinical, histopathological, immunoserological and genetic features. The treatment is similar for the different subtypes of CLE; however, the therapeutic regimen should be individually defined in each patient. Antimalarials are still the first-line systemic therapy and in addition to sunscreens, glucocorticosteroids and calcineurin inhibitors have an important impact as topical agents in this disease.

  18. Systemic lupus erythematosus: strategies to improve pregnancy outcomes

    PubMed Central

    Yamamoto, Yuriko; Aoki, Shigeru

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with a high prevalence in females of childbearing age. Thus, reproduction in SLE patients is a major concern for clinicians. In the past, SLE patients were advised to defer pregnancy because of poor pregnancy outcomes and fear of SLE flares during pregnancy. Investigations to date show that maternal and fetal risks are higher in females with SLE than in the general population. However, with appropriate management of the disease, sufferers may have a relatively uncomplicated pregnancy course. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, intensive surveillance during and after pregnancy by both the obstetrician and rheumatologist, and appropriate interventions when necessary play a key role. This review describes the strategies to improve pregnancy outcomes in SLE patients at different time points in the reproduction cycle (preconception, during pregnancy, and postpartum period) and also details the neonatal concerns. PMID:27468250

  19. Systemic lupus erythematosus and thrombotic thrombocytopenia purpura: a refractory case without lupus activity.

    PubMed

    Garcia Boyero, Raimundo; Mas Esteve, Eva; Mas Esteve, Maria; Millá Perseguer, M Magdalena; Marco Buades, Josefa; Beltran Fabregat, Juan; Cañigral Ferrando, Guillermo; Belmonte Serrano, Miguel Angel

    2013-01-01

    The association between systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) has been infrequently reported. Usually, patients with TTP have more SLE activity and frequent renal involvement. Here we present a case of TTP associated to low-activity SLE. The absence of renal and major organ involvement increased the difficulty in making the initial diagnosis. ADAMTS13 activity in plasma in this patient was very low, as seen in other similar cases. The evolution of the patient was poor, needing plasma exchanges and immunosuppressive therapy, including the use of rituximab.

  20. Childhood systemic lupus erythematosus, vasculitis, and rheumatic fever and neonatal lupus.

    PubMed

    Pelkonen, P

    1995-09-01

    Studies on the long-term outcome of patients with systemic lupus erythematosus not only give us survival figures but also uncover flaws in our treatment strategies and reveal both disease-associated and other factors that affect prognosis. Among the latter, compliance with treatment and socioeconomic factors are noteworthy. The pathogenesis of neonatal lupus is under active investigation, and new approaches are being developed. This review also draws attention to a number of vasculitis syndromes that are common in adults but very rarely reported in children. Poststreptococcal reactive arthritis has been described as a new entity; however, such patients should probably receive the same attention as patients with rheumatic fever to avoid recurrences of the disease and cardiac sequelae.

  1. White Matter Correlates of Neuropsychological Dysfunction in Systemic Lupus Erythematosus

    PubMed Central

    Jung, Rex E.; Chavez, Robert S.; Flores, Ranee A.; Qualls, Clifford; Sibbitt, Wilmer L.; Roldan, Carlos A.

    2012-01-01

    Patients diagnosed with Systemic Lupus Erythematosus have similar levels of neuropsychological dysfunction (i.e., 20–50%) as those with Neuropsychiatric Systemic Lupus Erythematosus (NPSLE). We hypothesized a gradient between cognition and white matter integrity, such that strongest brain-behavior relationships would emerge in NPSLE, intermediate in non-NPSLE, and minimal in controls. We studied thirty-one patients (16 non-NPSLE; 15 NPSLE), ranging in age from 18 to 59 years old (100% female), and eighteen age and gender matched healthy controls. DTI examinations were performed on a 1.5T scanner. A broad neuropsychological battery was administered, tapping attention, memory, processing speed, and executive functioning. The Total z-score consisted of the combined sum of all neuropsychological measures. In control subjects, we found no significant FA-Total z-score correlations. NPSLE, non-NPSLE, and control subjects differed significantly in terms of Total z-score (NPSLE = −2.25+/−1.77, non-NPSLE = −1.22+/−1.03, Controls = −0.10+/−.57; F = 13.2, p<.001). In non-NPSLE subjects, FA within the right external capsule was significantly correlated with Total z-score. In NPSLE subjects, the largest FA-Total z-score clusters were observed within the left anterior thalamic radiation and right superior longitudinal fasciculus. In subsequent analyses the largest number of significant voxels linked FA with the Processing Speed z-score in NPSLE. The current results reflect objective white matter correlates of neuropsychological dysfunction in both NPSLE and (to a lesser degree) in non-NPSLE. non-NPSLE and NPSLE subjects did not differ significantly in terms of depression, as measured by the GDI; thus, previous hypotheses suggesting moderating effects of depression upon neuropsychological performance do not impact the current FA results. PMID:22291880

  2. Postextraction hemorrhage in a young male patient with systemic lupus erythematosus.

    PubMed

    Schwartz, S; Esseltine, D W

    1984-03-01

    A case of a 13-year-old boy with prolonged bleeding after tooth extraction is reported. This was the first manifestation of systemic lupus erythematosus found to be associated with circulating anticoagulants, including the "lupus anticoagulant," and possible hypoprothrombinemia. PMID:6608711

  3. Persistent scarring, atrophy, and dyspigmentation in a preteen girl with neonatal lupus erythematosus.

    PubMed

    High, Whitney A; Costner, Melissa I

    2003-04-01

    Neonatal lupus erythematosus is an uncommon autoimmune disease with distinctive cutaneous findings. Descriptions of chronic cutaneous sequelae are rare. We describe a 12-year-old girl with persistent dyspigmentation, scarring, and atrophy as a result of neonatal lupus occurring during infancy. PMID:12664034

  4. Premature aortic stiffness in systemic lupus erythematosus by transesophageal echocardiography.

    PubMed

    Roldan, C A; Joson, J; Qualls, C R; Sharrar, J; Sibbitt, W L

    2010-12-01

    To assess aortic stiffness by transesophageal echocardiography (TEE) and to determine its clinical predictors and relation to age, blood pressure, renal function, and atherosclerosis, 50 patients with systemic lupus erythematosus (SLE), 94% women, with a mean age of 38 ± 12 years, and 22 age and gender-matched healthy controls underwent clinical and laboratory evaluations and multiplane TEE to assess stiffness, intima-media thickness (IMT), and plaques of the proximal, mid, and distal descending thoracic aorta. Stiffness at each level and overall aortic stiffness by the pressure-strain elastic modulus was higher in patients than in controls after adjusting for age (overall, 8.25 ± 4.13 versus 6.1 ± 2.5 Pascal units, p = 0.01). Patients had higher aortic stiffness than controls after adjusting both groups to the same mean age, blood pressure, creatinine, and aortic IMT (p = 0.005). Neither IMT nor plaques were predictors of aortic stiffness. Moreover, normotensive patients, those without aortic plaques, and non-smokers had higher stiffness than controls (all p < 0.05). Age at SLE diagnosis and non-neurologic damage score were the only SLE-specific independent predictors of aortic stiffness (both p ≤ 0.01). Thus, increased aortic stiffness is an early manifestation of lupus vasculopathy that seems to precede the development of hypertension and atherosclerosis.

  5. The nature and outcome of infection in systemic lupus erythematosus.

    PubMed

    Gladman, D D; Hussain, F; Ibañez, D; Urowitz, M B

    2002-01-01

    Infection remains a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). To describe the nature and outcomes of infection and determine their associated risk factors in patients with SLE, we performed a nested case-control study at the University of Toronto Lupus Clinic, with prospective follow-up according to a standard protocol since 1970. Cases were SLE patients seen between January 1987 and January 1992 who had documented infections and controls were patients without infection from the same cohort matched for age, gender and time of visit. The type, site and outcome of infection were recorded for each case. A conditional logistic regression analysis was performed to compare factors associated with infection in cases and their controls. Ninety-three patients had 148 infection episodes; the majority were bacterial, but viral, fungal and protozoan organisms were also identified (multiple organisms in seven). Forty-eight patients required hospital admission and three patients died. Steroids at time of infection, as well as use ever, duration and dose, immunosuppressives at time of infection and use ever, active renal disease, CNS damage, SLEDAI at the time of infection, adjusted mean SLEDAI and variability measure were significantly associated with infection by univariate analysis. By multivariate analysis one factor remained statistically significant: use of steroids ever (P = 0.029). Infection carries a large burden for SLE patients. Until new medications which will control disease activity without predisposing to infection are developed, careful titration of steroids and cytotoxic drugs to control disease activity will remain crucial.

  6. Altered B cell receptor signaling in human systemic lupus erythematosus

    PubMed Central

    Jenks, Scott A.; Sanz, Iñaki

    2009-01-01

    Regulation of B cell receptor signaling is essential for the development of specific immunity while retaining tolerance to self. Systemic lupus erythematosus (SLE) is characterized by a loss of B cell tolerance and the production of anti-self antibodies. Accompanying this break down in tolerance are alterations in B cell receptor signal transduction including elevated induced calcium responses and increased protein phosphorylation. Specific pathways that negatively regulate B cell signaling have been shown to be impaired in some SLE patients. These patients have reduced levels of the kinase Lyn in lipid raft microdomains and this reduction is inversely correlated with increased CD45 in lipid rafts. Function and expression of the inhibitory immunoglobulin receptor FcγRIIB is also reduced in Lupus IgM- CD27+ memory cells. Because the relative contribution of different memory and transitional B cell subsets can be abnormal in SLE patients, we believe studies targeted to well defined B cell subsets will be necessary to further our understanding of signaling abnormalities in SLE. Intracellular flow cytometric analysis of signaling is a useful approach to accomplish this goal. PMID:18723129

  7. [Treatment of systemic lupus erythematosus: myths, certainties and doubts].

    PubMed

    Ruiz-Irastorza, Guillermo; Danza, Alvaro; Khamashta, Munther

    2013-12-21

    Systemic lupus erythematosus (SLE) is a complex disease with different clinical forms of presentation, including a wide range of severity and organic involvement. Such circumstance, along with the fact of the uncommon nature of the disease and the absence of clinically representative response criteria, make it difficult to design controlled clinical trials in SLE patients. As a result, observational studies have a special relevance, being a source of valuable information of SLE prognosis and outcome as well as of the efficacy and adverse effects of the different therapies. Herein we update some of the main treatments used in SLE. Steroids may have more risks than benefits if used at high doses. New mechanisms of action have been described, supporting the use of lower doses, possibly with the same efficacy and less adverse effects. Intravenous pulses of cyclophosphamide still have a role in the treatment of proliferative lupus nephritis and other serious SLE manifestations. Mycophenolate mofetil has shown its efficacy both as induction and maintenance therapy of selected cases of lupus nephritis. Biological therapies have emerged as new promising options. Although clinical trials have not confirmed a clear superiority of rituximab in SLE, observational studies have shown good response rates in severe SLE manifestations or refractory forms. Belimumab has recently been added to the therapeutic armamentarium of SLE; although its place in clinical practice is not well-defined, it may be recommended in active patients with no response or good tolerance to standard therapies. Hydroxichloroquine improves survival, decreases the risk of thrombosis and flares and is safe in pregnancy, and should be considered the baseline therapy in most SLE patients.

  8. CNS vasculitis and stroke in neonatal lupus erythematosus: a case report and review of literature.

    PubMed

    Saini, Arushi G; Sankhyan, Naveen; Bhattad, Sagar; Vyas, Sameer; Saikia, Biman; Singhi, Pratibha

    2014-05-01

    Neonatal lupus erythematosus refers to the clinical spectrum of cardiac, cutaneous and other systemic abnormalities in neonates born to mothers with autoantibodies against Ro/SSA and La/SSB antigens. Isolated central nervous system involvement is very rare and has been described as transient vasculopathy only. We describe a 2-months-old girl who presented with acute ischemic stroke secondary to central nervous system vasculitis without any cardiac, cutaneous or hematological manifestations. The mother was pauci-symptomatic with raised anti-Ro autoantibody titers; the baby was positive for autoantibodies against Ro-antigen. Angiography confirmed vasculitis in cerebral vasculature. Our case highlights that neonatal lupus erythematosus can present with isolated nervous system manifestations and the vascular damage can be permanent in the form of vasculitis. Early recognition will help pediatricians identify such possible permanent complications in newborns with neonatal lupus erythematosus. A review of previously reported central nervous system manifestations of neonatal lupus is also presented.

  9. [A case of systemic lupus erythematosus presenting with jaundice and lupus pneumonia].

    PubMed

    Kawai, Takako; Tominaga, Sizuo; Okouchi, Akiko; Kudo, Makoto; Katoh, Kiyoshi; Shoda, Masataka; Fujino, Masayuki A

    2005-02-01

    A 27-year-old Japanese woman was referred to our hospital for acute hepatitis in April 2002. She had been suffering from low grade fever and fatigue for a week. She also presented with dyspnea. On admission, ALT and AST were 857 U/l and 473 U/l respectively. Urine protein was 2 g/day. Chest radiograph showed bilateral infiltrative shadow and pleural effusion. She developed jaundice and her level of total bilirubin was increased to 9.6 mg/dl on May 9. Antibodies to hepatitis viruses were not detected. Testing for antimitochondrial antibodies, antismooth muscle antibodies, and antiribosomal P antibodies showed all negative. However, antinuclear antibodies were positive at titer 1:160 and anti-double stranded DNA antibodies were 130 U/ml. A diagnosis of systemic lupus erythematosus was made and oral administration of 60 mg/day prednisolon was started on May 10. Serum levels of ALT, AST and bilirubin were reduced to within normal range and pulmonary lesions were also improved. We conclude that this is a rare case of systemic lupus erythematosus presenting with acute hepatitis and jaundice.

  10. Anti-chromatin antibodies in systemic lupus erythematosus: a useful marker for lupus nephropathy

    PubMed Central

    Cervera, R; Vinas, O; Ramos-Casals, M; Font, J; Garcia-Carrasco, M; Siso, A; Ramirez, F; Machuca, Y; Vives, J; Ingelmo, M; Burlingame, R

    2003-01-01

    Background: Anti-chromatin antibodies have recently been described in patients with systemic lupus erythematosus (SLE) and it has been suggested that their presence is associated with lupus nephritis. Objective: To assess the prevalence and clinical associations of these antibodies in SLE. Methods: The presence of anti-chromatin antibodies in 100 patients with SLE was investigated by an enzyme linked immunosorbent assay (ELISA). To determine the specificity of these antibodies, 100 patients with primary Sjögren's syndrome, 30 with primary antiphospholipid syndrome (APS), 10 with systemic sclerosis, and 100 normal controls were also tested. Results: Positive levels were detected in 69/100 (69%) patients with SLE. In contrast, they were found in only 8/100 (8%) of those with primary Sjögren's syndrome, in 1/10 (10%) with systemic sclerosis, in 2/30 (7%) with primary APS, and in none of the 100 healthy controls. Patients with anti-chromatin antibodies had a twofold higher prevalence of lupus nephropathy than those without these antibodies (58% v 29%, p<0.01). A significant correlation was found between the levels of anti-chromatin antibodies and disease activity score as measured by the European Consensus Lupus Activity Measurement (ECLAM; p=0.011). Conclusions: The measurement of anti-chromatin antibodies appears to be a useful addition to the laboratory tests that can help in the diagnosis and treatment of SLE. These antibodies are both sensitive and specific for SLE, and are a useful marker for an increased risk of lupus nephritis. PMID:12695155

  11. Estrogen in Cardiovascular Disease during Systemic Lupus Erythematosus

    PubMed Central

    Gilbert, Emily L.; Ryan, Michael J.

    2015-01-01

    Purpose Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that disproportionately affects women during their childbearing years. Cardiovascular disease is the leading cause of mortality in this patient population at an age when women often have low cardiovascular risk. Hypertension is a major cardiovascular disease risk factor, and its prevalence is markedly increased in women with SLE. Estrogen has traditionally been implicated in SLE disease progression because of the prevalence of the disease in women; however, its role in cardiovascular risk factors such as hypertension is unclear. The objective of this review is to discuss evidence for the role of estrogen in both human and murine SLE with emphasis on the effect of estrogen on cardiovascular risk factors, including hypertension. Methods PubMed was used to search for articles with terms related to estradiol and SLE. The references of retrieved publications were also reviewed. Findings The potential permissive role of estrogen in SLE development is supported by studies from experimental animal models of lupus in which early removal of estrogen or its effects leads to attenuation of SLE disease parameters, including autoantibody production and renal injury. However, data about the role of estrogens in human SLE are much less clear, with most studies not reaching firm conclusions about positive or negative outcomes after hormonal manipulations involving estrogen during SLE (ie, oral contraceptives, hormone therapy). Significant gaps in knowledge remain about the effect of estrogen on cardiovascular risk factors during SLE. Studies in women with SLE were not designed to determine the effect of estrogen or hormone therapy on blood pressure even though hypertension is highly prevalent, and risk of premature ovarian failure could necessitate use of hormone therapy in women with SLE. Recent evidence from an experimental animal model of lupus found that estrogen may protect against

  12. Aplastic anemia associated to systemic lupus erythematosus in an AIDS patient: a case report

    PubMed Central

    de Oliveira, Leonardo Rodrigues; Ferreira, Thaís Camargos; Neves, Fernando de Freitas; Meneses, Antônio Carlos de Oliveira

    2013-01-01

    Aplastic anemia is a bone marrow failure syndrome characterized by peripheral cytopenias and hypocellular bone marrow. Although aplastic anemia is idiopathic in most cases, rheumatic diseases such as systemic lupus erythematosus are recognized as causes of aplastic anemia, with their possible etiological mechanisms being T and B lymphocyte dysfunction and pro-inflammatory cytokines and autoantibody production directed against bone marrow components. In the course of the human immunodeficiency virus infection/acquired immunodeficiency syndrome, the identification of autoantibodies and the occurrence of rheumatic events, in addition to the natural course of systemic lupus erythematosus which is modified by immune changes that are characteristic of human immunodeficiency virus infection/acquired immunodeficiency syndrome, make the diagnosis of systemic lupus erythematosus challenging. This study reports the case of a woman with acquired immunodeficiency syndrome treated with a highly active antiretroviral therapy, who had prolonged cytopenias and hypocellular bone marrow consistent with aplastic anemia. The clinical picture, high autoantibodies titers, and sustained remission of the patient's hematological status through immunosuppression supported the diagnosis of systemic lupus erythematosus-associated aplastic anemia. This is the first report of aplastic anemia concurrent with systemic lupus erythematosus and acquired immunodeficiency syndrome, providing additional evidence that immune dysfunction is a key part of the pathophysiological mechanism of aplastic anemia. PMID:24255622

  13. Neurologic complications of systemic lupus erythematosus, sjögren syndrome, and rheumatoid arthritis.

    PubMed

    Bhattacharyya, Shamik; Helfgott, Simon M

    2014-09-01

    Neurologic complications are frequent and often morbid in systemic lupus erythematosus, Sjögren syndrome, and rheumatoid arthritis. Although all are systemic inflammatory syndromes, each disease affects the nervous system distinctly, such as peripheral neuropathy in Sjögren syndrome, cerebrovascular disease in lupus, and cervical spine subluxation in rheumatoid arthritis. Some neurologic complications share convergent pathophysiology across diseases, such as neuromyelitis optica spectrum disorders in both Sjögren syndrome and lupus. Ill-defined cognitive complaints are especially common in lupus and Sjögren syndrome. For the majority of the complications, evidence for treatment efficacy is limited and requires further investigation.

  14. Pathogenic Inflammation and Its Therapeutic Targeting in Systemic Lupus Erythematosus

    PubMed Central

    Gottschalk, Timothy A.; Tsantikos, Evelyn; Hibbs, Margaret L.

    2015-01-01

    Systemic lupus erythematosus (SLE, lupus) is a highly complex and heterogeneous autoimmune disease that most often afflicts women in their child-bearing years. It is characterized by circulating self-reactive antibodies that deposit in tissues, including skin, kidneys, and brain, and the ensuing inflammatory response can lead to irreparable tissue damage. Over many years, clinical trials in SLE have focused on agents that control B- and T-lymphocyte activation, and, with the single exception of an agent known as belimumab which targets the B-cell survival factor BAFF, they have been disappointing. At present, standard therapy for SLE with mild disease is the agent hydroxychloroquine. During disease flares, steroids are often used, while the more severe manifestations with major organ involvement warrant potent, broad-spectrum immunosuppression with cyclophosphamide or mycophenolate. Current treatments have severe and dose-limiting toxicities and thus a more specific therapy targeting a causative factor or signaling pathway would be greatly beneficial in SLE treatment. Moreover, the ability to control inflammation alongside B-cell activation may be a superior approach for disease control. There has been a recent focus on the innate immune system and associated inflammation, which has uncovered key players in driving the pathogenesis of SLE. Delineating some of these intricate inflammatory mechanisms has been possible with studies using spontaneous mouse mutants and genetically engineered mice. These strains, to varying degrees, exhibit hallmarks of the human disease and therefore have been utilized to model human SLE and to test new drugs. Developing a better understanding of the initiation and perpetuation of disease in SLE may uncover suitable novel targets for therapeutic intervention. Here, we discuss the involvement of inflammation in SLE disease pathogenesis, with a focus on several key proinflammatory cytokines and myeloid growth factors, and review the known

  15. Borderline tuberculoid leprosy in childhood onset systemic lupus erythematosus patient.

    PubMed

    Lopes, V A P; Lourenço, D M R; Guariento, A; Trindade, M A; Avancini, J; Silva, C A

    2015-11-01

    Leprosy is a contagious and chronic systemic granulomatous disease caused by the bacillus Mycobacterium leprae. To our knowledge, no case of leprosy in a childhood-onset systemic lupus erythematosus (c-SLE) patient has been reported. For a period of 31 years, 312 c-SLE patients were followed at the Pediatric Rheumatology Unit of our University Hospital. One of them (0.3%) had tuberculoid leprosy skin lesions during the disease course and is here reported. A 10-year-old boy from Northwest of Brazil was diagnosed with c-SLE based on malar rash, photosensitivity, oral ulcers, lymphopenia, proteinuria, positive antinuclear antibodies, anti-double-stranded DNA, anti-Sm and anti-Ro/SSA autoantibodies. He was treated with prednisone, hydroxychloroquine and intravenous cyclophosphamide, followed by mycophenolate mofetil. At 12-years-old, he presented asymmetric skin lesions characterized by erythematous plaques with elevated external borders and hypochromic center with sensory loss. Peripheral nerve involvement was not evidenced. No history of familial cases of leprosy was reported, although the region where the patient resides is considered to be endemic for leprosy. Skin biopsy revealed a well-defined tuberculoid form. A marked thickening of nerves was observed, often destroyed by granulomas, without evidence of Mycobacterium leprae bacilli. At that time, the SLEDAI-2K score was 4 and he had been receiving prednisone 15 mg/day, hydroxychloroquine 200 mg/day and mycophenolate mofetil 3 g/day. Paucibacillary treatment for leprosy with dapsone and rifampicine was also introduced. In conclusion, we have reported a rare case of leprosy in the course of c-SLE. Leprosy should always be considered in children and adolescents with lupus who present skin abnormalities, particularly with hypoesthesic or anesthesic cutaneous lesions.

  16. Diffuse alveolar hemorrhage in Colombian patients with systemic lupus erythematosus.

    PubMed

    Cañas, Carlos; Tobón, Gabriel J; Granados, Marcela; Fernández, Liliana

    2007-11-01

    Diffuse alveolar hemorrhage (DAH) is a rare life-threatening complication seen in patients with systemic lupus erythematosus (SLE). This paper describes the clinical features and outcome of seven SLE patients with DAH admitted to a medical intensive care unit (ICU) in a referral center. Of a total of 122 SLE patients, seven patients presented this complication (5.7%). Five patients were women (71.4%). Mean age was 24.3 (range 4-51 years). Mean duration of SLE before clinical DAH was 15.7 months (range 0-48 months). DAH was the initial manifestation of SLE in two patients (29%). DAH recurrence was seen in two patients (29%). Active lupus was present in all seven patients. Fever, glomerulonephritis, arthritis, myositis, and peripheral neuropathy were observed in six, four, four, three, and two patients, respectively. Five patients who underwent to bronchoscopy had positive findings of DAH (71.4%; i.e., bloody return on bronchoalveolar lavage--with hemosiderin-laden macrophages). Treatment options included intravenous methylprednisolone (10 mg kg(-1) day(-1)--3 days) following by prednisolone 1 mg kg(-1) day(-1) and pulse cyclophosphamide (750 mg/m(2)). Plasmapheresis was added in four patients (57.1%) because of the persistence of DAH. All patients were treated in an ICU, six of them requiring mechanical respiratory support (85.1%). Mortality rate during ICU stay was 12% (one patient). Our results show that DAH is an uncommon complication in SLE patients, requiring a prompt and aggressive recognition and treatment with high-dose steroid, intravenous cyclophosphamide, and plasmapheresis. With all of these treatments, there is a trend to a low mortality rate in patients with SLE presenting DAH.

  17. Self-reported sleep in systemic lupus erythematosus.

    PubMed

    Greenwood, Kenneth Mark; Lederman, Leah; Lindner, Helen Dawn

    2008-09-01

    The objectives of the study were to assess sleep disturbances in systemic lupus erythematosus (SLE) and to compare these with a working sample and a treatment-seeking sample reporting insomnia. The primary sample was 172 people with SLE. This sample represented 32% of all members of two lupus support association. Two comparison samples were used: 223 adults who expressed interest in taking part in a psychological treatment for sleep problems and 456 Australian adults who were working at a large organization. All individuals completed the Pittsburgh Sleep Quality Index (PSQI; 6). Data derived from the PSQI included total sleep time, sleep onset latency, wake after sleep onset, sleep efficiency, as well as the global and seven component scores. The SLE sample reported significantly worse sleep on all parameters than the working sample, but significantly better sleep than the sample of those seeking treatment for sleep disorders, except for sleep onset latency. The percentages scoring >5 on the PSQI global score was 80.5% for SLE, 91.5% for those seeking treatment for sleep disorders, and 28.5% for the working sample. PSQI component scores for the SLE group more closely resembled those of the treatment-seeking group. Self-reported sleep in this sample of people with SLE was significantly better on most parameters than that of a group seeking treatment for sleep disorders. However, the values obtained tended to be worse than previous reports and indicated less than optimal sleep. However, the low response rate of the sample was of concern and may indicate that the sample was biased. The present results suggest that sleep disturbance is common in those with SLE and deserves more attention in a more representative sample.

  18. Bilateral sudden sensorineural hearing loss as a presenting feature of systemic lupus erythematosus

    PubMed Central

    Chawki, Sylvain; Aouizerate, Jessie; Trad, Selim; Prinseau, Jacques; Hanslik, Thomas

    2016-01-01

    Abstract Introduction: Sudden sensorineural hearing loss is an unusual presenting clinical feature of systemic lupus erythematosus. Case report: We report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement, thrombocytopenia, and consistent autoantibodies. Favorable outcome was obtained on high-dose corticosteroids, and the hearing fully recovered. Discussion: Sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present. Although management remains empirical, the high risk of permanent hearing impairment seems to justify emergency treatment with high-dose corticosteroids. When the clinical and laboratory criteria of antiphospholipid syndrome are met, antiplatelets agents or anticoagulation therapy shall be considered. PMID:27603334

  19. Severe chronic blepharitis and scarring ectropion associated with discoid lupus erythematosus.

    PubMed

    Kopsachilis, Nikolaos; Tsaousis, Konstantinos T; Tourtas, Theofilos; Tsinopoulos, Ioannis T

    2013-01-01

    Discoid lupus erythematosus is a common form of chronic cutaneous lupus erythematosus, an autoimmune inflammatory disease that affects most of the human organs, including the skin, kidneys, joints, heart and lungs. We describe a 45-year-old Caucasian woman with a 21-year history of eyelid redness and irritation. She had been treated with antibiotics, steroids and eyelid hygiene, a therapy that resulted to brief periods of relief of symptoms. In the last 12 months, her symptoms seemed to have exacerbated, despite the administration of a local therapy. Following biopsy, a diagnosis of discoid lupus erythematosus was confirmed and the patient was placed on a systemic therapy with hydroxychloroquine. The eyelid inflammation decreased but severe scarring of the marginal eyelids persisted, resulting in cicatricial ectropion.

  20. Glial and axonal changes in systemic lupus erythematosus measured with diffusion of intracellular metabolites.

    PubMed

    Ercan, Ece; Magro-Checa, Cesar; Valabregue, Romain; Branzoli, Francesca; Wood, Emily T; Steup-Beekman, Gerda M; Webb, Andrew G; Huizinga, Tom W J; van Buchem, Mark A; Ronen, Itamar

    2016-05-01

    Systemic lupus erythematosus is an inflammatory autoimmune disease with multi-organ involvement. Central nervous system involvement in systemic lupus erythematosus is common and results in several neurological and psychiatric symptoms that are poorly linked to standard magnetic resonance imaging outcome. Magnetic resonance imaging methods sensitive to tissue microstructural changes, such as diffusion tensor imaging and magnetization transfer imaging, show some correlation with neuropsychiatric systemic lupus erythematosus (NPSLE) symptoms. Histological examination of NPSLE brains reveals presence of cerebral oedema, loss of neurons and myelinated axons, microglial proliferation and reactive astrocytosis, microinfacrts and diffuse ischaemic changes, all of which can affect both diffusion tensor imaging and magnetization transfer imaging in a non-specific manner. Here we investigated the underlying cell-type specific microstructural alterations in the brain of patients with systemic lupus erythematosus with and without a history of central nervous system involvement. We did so combining diffusion tensor imaging with diffusion-weighted magnetic resonance spectroscopy, a powerful tool capable of characterizing cell-specific cytomorphological changes based on diffusion of intracellular metabolites. We used a 7 T magnetic resonance imaging scanner to acquire T1-weighted images, diffusion tensor imaging datasets, and single volume diffusion-weighted magnetic resonance spectroscopy data from the anterior body of the corpus callosum of 13 patients with systemic lupus erythematosus with past NPSLE, 16 patients with systemic lupus erythematosus without past NPSLE, and 19 healthy control subjects. Group comparisons were made between patients with systemic lupus erythematosus with/without past NPSLE and healthy controls on diffusion tensor imaging metrics and on diffusion coefficients of three brain metabolites: the exclusively neuronal/axonal N-acetylaspartate, and the

  1. Neglect leads to extremes: maggots and malignancy in a case of discoid lupus erythematosus.

    PubMed

    Bhari, N; Khaitan, B K; Gupta, P; Kumar, T; Srivastava, A

    2016-01-01

    Discoid lupus erythematosus (DLE) is a chronic form of cutaneous lupus erythematosus that runs an indolent course. The rare complications of DLE include scarring, mutilation, non-healing ulceration, cicatricial alopecia and malignancy. DLE progresses to systemic lupus erythematosus (SLE) in around 5% of localized cases and 22% of generalized cases. We report a case of DLE, presenting with a six-month history of ulcerated fungating plaques and small crusted nodules superimposed on DLE plaques over both the forearms. Two weeks prior to the presentation, maggots were also noticed on these plaques. Skin biopsies from these lesions were suggestive of squamous cell carcinoma (SCC) and keratoacanthoma. A wide surgical excision of the tumor followed by partial split-thickness skin grafting was performed with complete healing of the lesions. No recurrence has been noted 18 months from follow-up.

  2. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus

    PubMed Central

    Ünlü, Ozan; Zuily, Stephane; Erkan, Doruk

    2016-01-01

    Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage. The use of low-dose aspirin (LDA) is controversial for primary thrombosis and pregnancy morbidity prevention because of the lack of strong prospective controlled data. Similarly, the use of anticoagulation is controversial for patients with an aPL-related nephropathy. Until further studies are available, physicians should discuss the risk/benefits of LDA or anticoagulation as well as the available literature with patients. PMID:27708976

  3. Extracorporeal shockwave for hip necrosis in systemic lupus erythematosus.

    PubMed

    Wang, C J; Ko, J Y; Chan, Y S; Lee, M S; Chen, J M; Wang, F S; Yang, K D; Huang, C C

    2009-10-01

    Patients with systemic lupus erythematosus (SLE) frequently received corticosteroid therapy, resulting in osteonecrosis of the femoral head (ONFH). Prior studies demonstrated the effectiveness of extracorporeal shockwave treatment (ESWT) for ONFH.. This study evaluated the effectiveness of ESWT for ONFH in patients with SLE. We studied 39 patients, including 15 patients with SLE (26 hips) and 24 controls (29 hips). To each affected hip we applied ESWT (6000 impulses at 28 kV in a single session). Patients were ambulated with partial weight bearing for 4-6 weeks. The primary endpoint was the need for hip replacement. The secondary endpoints were improvement in hip pain and function and image changes on X-ray and MRI. Patients received total hip replacement in 12% of patients with SLE and in 14% of controls (P = 0.802). There was no statistically significant difference in pain scores (0.86 vs. 0.89; P = 0.467) and function scores (89% vs. 91%; P = 0.194) between patients with SLE and controls. SLE response to ESWT for ONFH is comparable with ONFH in patients without SLE.

  4. Infections and Systemic Lupus Erythematosus: Binding or Sparring Partners?

    PubMed Central

    Rigante, Donato; Esposito, Susanna

    2015-01-01

    Extensive work on experimental animal models clearly demonstrates that infectious agents can break immunological tolerance to self-antigens and induce autoimmune disorders, mainly systemic lupus erythematosus (SLE). The establishment of a causative link between infections and autoimmunity has been largely studied in a host of clinical studies, proving the role of infectious agents in the induction, as well as in the progression or exacerbation of SLE. However, we are far from a plain understanding of microbial-host interactions in the pathogenesis of SLE. Much serological, molecular and geoepidemiological evidence supports the relationship of different environmental infectious triggers in the inception of SLE-related autoimmune phenomena with adjuvant effects. The promotion of autoimmune responses through bystander activation or epitope spreading via multiple inflammatory pathways has been confirmed in animal models. Different viruses have been implicated in SLE pathogenesis, particularly Epstein-Barr virus, but also parvovirus B19, cytomegalovirus and retroviruses. SLE patients usually have an impaired immune response towards Epstein-Barr virus and dysregulation of the viral latency period. Furthermore, the accumulation of endogenous retroviral products might trigger the production of interferon and anti-DNA antibodies. In addition, protozoan infections might even protect from autoimmune processes and rescind an ongoing B cell activation. Herein, we discuss which type of infections induce, exacerbate or inhibit autoimmune disorders and analyze the principal infection-induced immunological mechanisms influencing the development of SLE. PMID:26230690

  5. Neuropsychiatric manifestations in systemic lupus erythematosus: epidemiology, pathophysiology and management.

    PubMed

    Postal, Mariana; Costallat, Lilian T L; Appenzeller, Simone

    2011-09-01

    Systemic lupus erythematosus (SLE) is a relapsing-remitting autoimmune disease with CNS involvement occurring in up to 75% of patients. However, the frequency of neuropsychiatric manifestations in SLE studies varies widely, depending on the type of manifestations included and the method used for evaluation. CNS involvement may be considered primary if directly related to SLE activity in the CNS or secondary when related to treatment, infections, metabolic abnormalities or other systemic manifestations such as uraemia and hypertension. The pathogenesis of neuropsychiatric SLE is as yet unknown, though numerous autoantibodies and cytokines have been suggested as possible mediators. However, independent of the aetiology of the insult, the final common pathway in neuropsychiatric SLE is the involvement of the cerebral microvasculature. The diagnosis of primary CNS involvement by SLE is often difficult, as both focal and diffuse manifestations may occur and there is no gold standard for diagnosis. A high index of clinical suspicion, in addition to laboratory and neuroimaging findings may support the diagnosis. Treatment is mostly empirical, although one randomized controlled trial has shown that cyclophosphamide in addition to methylprednisolone is superior to methylprednisolone alone in severe neuropsychiatric SLE.

  6. [Pathogenesis of cutaneous lupus erythematosus from LE-prone mice].

    PubMed

    Furukawa, Fukumi; Yoshimasu, Takashi; Kanazawa, Nobuo

    2010-01-01

    Mouse models are similar but not identical to human diseases. However, they are important for research into the pathogenesis underlying autoimmune diseases because they allow us to evaluate similarities and differences between human diseases and mouse models. There are many inbred strains of systemic lupus erythematosus (SLE)-prone mice including New Zealand Black (NZB), F1 hybrids of NZB x New Zealand White (NZW) (B/W F1), MRL/Mp-lpr/lpr (MRL/lpr), and BXSB mice. The postulated etiology of these murine diseases includes many genetic and extrinsic factors such as retroviruses, an impaired balance of T cell interaction, ultraviolet irradiation, etc. For examples, genetic studies of MRL/lpr mice revealed that the appearance of macroscopic LE-like skin lesions needs the lpr mutation plus an additional factor in an autosomal dominant fashion. The candidate is ultraviolet (UV) B light, the susceptibility to which is regulated by the genetic background. Such abnormalities described in SLE now span the spectrum from innate immunity to acquired immunity. In this review, based on historical review, we focus on skin lesions from the well-studied MRL/lpr and B/W F1 mouse and discuss how SLE-prone mice can contribute to a better understanding of cutaneous LE pathogenesis. PMID:20818144

  7. [Experimental modeling of nucleoprotein disposal disorders in systemic lupus erythematosus].

    PubMed

    Trofimenko, A S; Gontar, I P; Paramonova, O V; Simakova, E S; Zborovskaya, I A

    2015-01-01

    The objective of this research was to adapt the experimental model simulating the nucleoprotein disposal disorders in systemic lupus erythematosus (SLE) for further study of its extracorporeal correction, as well as to assess validity of the model by short-term experiment. Twenty to female Wistar rats were intraperitoneally injected with the chromatin-containing extract from bovine liver followed by intravenous administration of anti-DNA antibodies derived from SLE patients. After these procedures plasma concentrations of anti-dsDNA, circulating immune complexes and DNA became sharply increased, together with distinct elevation of leukocytes. On the contrary, changes in erythrocytes, platelets, total protein concentration, creatinine, asparagine and alanine aminotransferase activities, as well as blood coagulation time were changed insignificantly. Using direct immunofluorescence of cryosections, we detected human IgG deposition in rat kidneys treated in accordance with the simulation protocol. Thus, our model reproduces essential DNA disposal disorders in SLE without any animal death or the life-threatening changes in examined markers during short-term experiment. PMID:26539869

  8. Vitamin D levels in Jamaican patients with systemic lupus erythematosus.

    PubMed

    McGhie, T K; DeCeulaer, K; Walters, C A; Soyibo, A; Lee, M G

    2014-09-01

    Vitamin D has important effects on the immune system as it has been shown to exert antiproliferative and relative immunosuppressant effects. Low levels of this hormone may contribute to the immune activation in systemic lupus erythematosus (SLE) and other autoimmune diseases. Serum levels of 25-OH vitamin D were measured in 75 patients with SLE in Jamaica, using an enzyme-linked immunoassay. Correlations with clinical data and disease activity as determined by the BILAG index were determined. Of a total of 75 patients, 33 (44%) had vitamin D sufficiency with mean vitamin D level of 39.45 ng/ml (range, 30.35-58.16). Forty-two (56%) patients had either vitamin D deficiency or insufficiency, 30 (40%) had vitamin D insufficiency, mean 26.36 ng/ml (range, 20.26-29.88), and 12 (16%) had vitamin D deficiency, mean 16.07 ng/ml (range, 7.78-19.90). There was an overall negative relationship between the total disease activity score using the BILAG index and vitamin D levels, and this was influenced primarily by the relationship seen among the vitamin D-deficient subgroup. This was also impacted on by a patient population that was significantly skewed toward low disease activity. The negative association trended toward statistical significance. Vitamin D deficiency is prevalent among patients with SLE in Jamaica. A relationship between low serum levels of vitamin D and SLE activity may occur.

  9. Alternations of salivary antioxidant enzymes in systemic lupus erythematosus.

    PubMed

    Zaieni, S H; Derakhshan, Z; Sariri, R

    2015-11-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with chronic systemic inflammation. Oxidative stress may play a role in the pathogenesis of SLE. An increase in free radicals or an impaired antioxidant defense system in SLE causes oxidative stress. Therefore, oxidative damage plays an important role in the pathogenesis of SLE. Variations in antioxidant activity have been previously studied in serum of patients with this disease. However, salivary factors have not been evaluated. Considering that saliva, the noninvasive biological fluid, could be a reflection of the state of health, the purpose of this study was evaluation of peroxidase (POD), superoxide dismutase (SOD) and catalase (CAT) activity in the saliva of patients with SLE. During the course of the practical part of the project, 30 patients with SLE and 30 healthy controls were selected to donate their saliva samples. After centrifugation of un-stimulated saliva, biological activity of POD, CAT and SOD were evaluated on their appropriate substrates using spectrophotometric methods and the results were statistically analyzed. The results showed that activities of antioxidant enzymes SOD and CAT were significantly reduced in saliva of SLE patients as compared to controls. The results suggest that antioxidant status was impaired in the saliva of SLE patients, and antioxidant status of saliva could be one of the non-invasive markers for SLE.

  10. Subclinical Atherosclerosis in Rheumatoid Arthritis and Systemic Lupus Erythematosus

    PubMed Central

    Salmon, Jane E.; Roman, Mary J.

    2008-01-01

    Rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) are associated with increased mortality, largely as a consequence of cardiovascular disease. Increased cardiovascular morbidity and mortality in patients with RA and SLE cannot be entirely explained by traditional risk factors, suggesting that the systemic inflammation that characterizes these diseases may accelerate atherosclerosis. We used carotid ultrasonography to investigate the prevalence and correlates to preclinical atherosclerosis in patients with RA and SLE. Because atherosclerosis is a systemic disease, assessment of carotid plaque by ultrasonography provides a robust, direct measure of systemic atherosclerosis. We observed a substantially increased prevalence of carotid plaque in RA and SLE patients compared with age- and sex-matched controls, which remained after adjustment for traditional risk factors. The presence of carotid atherosclerosis was associated with disease duration in both RA and SLE and damage in SLE. These data support the hypothesis that inflammation associated with RA and SLE contributes to accelerated atherosclerosis and argue that RA and SLE disease activity should be more aggressively managed. PMID:18926167

  11. Aspirin therapy and thromboxane biosynthesis in systemic lupus erythematosus.

    PubMed

    Avalos, Ingrid; Chung, Cecilia P; Oeser, Annette; Milne, Ginger L; Borntrager, Holly; Morrow, Jason D; Raggi, Paolo; Solus, Joseph; Stein, C Michael

    2007-01-01

    Incomplete suppression of thromboxane biosynthesis during aspirin therapy is associated with increased cardiovascular risk. Since systemic lupus erythematosus (SLE) is associated with platelet activation and increased cardiovascular mortality, we compared thromboxane and prostacyclin biosynthesis in patients with SLE and control subjects, and measured inhibition of thromboxane excretion in aspirin-treated subjects. We measured the urinary excretion of 11-dehydro thromboxane B( 2) (TXB(2)) and 2,3-dinor 6-ketoPGF(1alpha) (PGI-M), the stable metabolites of thromboxane A(2) and prostacyclin, respectively, in 74 patients with SLE and 70 controls. In subjects who were not receiving aspirin, TXB(2) excretion was higher in patients with SLE [0.40 ng/mg creatinine (0.26-0.64), median (interquartile range)] than controls [0.31 ng/mg creatinine (0.23-0.44)] (P = 0.04), and in these patients, TXB(2) excretion correlated with disease activity (rho = 0.28, P = 0.03) and tumor necrosis factor alpha (rho = 0.48, P < 0.001). Aspirin therapy resulted in significantly lower TXB(2) excretion in controls (P = 0.01), but not in patients with SLE (P = 0.10), compared with subjects not receiving aspirin. Prostacyclin biosynthesis did not differ among patients and controls, and was not affected by aspirin (P all >0.35). Thromboxane biosynthesis is increased in SLE and is associated with disease activity. Additionally, response to aspirin may be attenuated in some patients with SLE. PMID:18042592

  12. Alveolar hemorrhage in systemic lupus erythematosus: a cohort review.

    PubMed

    Andrade, C; Mendonça, T; Farinha, F; Correia, J; Marinho, A; Almeida, I; Vasconcelos, C

    2016-01-01

    Diffuse alveolar hemorrhage (DAH) is a rare but potentially catastrophic manifestation with a high mortality. Among rheumatologic diseases, it occurs most frequently in patients with systemic lupus erythematosus (SLE) and systemic vasculitis. Despite new diagnostic tools and therapies, it remains a diagnostic and therapeutic challenge. The aim of this work was to characterize the SLE patients with an episode of alveolar hemorrhage followed in our Clinical Immunology Unit (CIU). A retrospective chart review was carried out for all patients with SLE followed in CIU between 1984 and the end of 2013. We reviewed the following data: demographic characteristics, clinical and laboratory data, radiologic investigations, histologic studies, treatment, and outcome. We identified 10 episodes of DAH, corresponding to seven patients, all female. These represent 1.6% of SLE patients followed in our Unit. The age at DAH attack was 42.75 ± 18.9 years. The average time between diagnosis of SLE and the onset of DAH was 7.1 years. Three patients had the diagnosis of SLE and the DAH attack at the same time. Disease activity according to SLEDAI was high, ranging from 15 to 41. All patients were treated with methylprednisolone, 37.5% cyclophosphamide and 28.6% plasmapheresis. The overall mortality rate was 28.6%.

  13. T Cell Transcriptomes Describe Patient Subtypes in Systemic Lupus Erythematosus

    PubMed Central

    Bradley, Sean J.; Suarez-Fueyo, Abel; Moss, David R.; Kyttaris, Vasileios C.; Tsokos, George C.

    2015-01-01

    Background T cells regulate the adaptive immune response and have altered function in autoimmunity. Systemic Lupus Erythematosus (SLE) has great diversity of presentation and treatment response. Peripheral blood component gene expression affords an efficient platform to investigate SLE immune dysfunction and help guide diagnostic biomarker development for patient stratification. Methods Gene expression in peripheral blood T cell samples for 14 SLE patients and 4 controls was analyzed by high depth sequencing. Unbiased clustering of genes and samples revealed novel patterns related to disease etiology. Functional annotation of these genes highlights pathways and protein domains involved in SLE manifestation. Results We found transcripts for hundreds of genes consistently altered in SLE T cell samples, for which DAVID analysis highlights induction of pathways related to mitochondria, nucleotide metabolism and DNA replication. Fewer genes had reduced mRNA expression, and these were linked to signaling, splicing and transcriptional activity. Gene signatures associated with the presence of dsDNA antibodies, low complement levels and nephritis were detected. T cell gene expression also indicates the presence of several patient subtypes, such as having only a minimal expression phenotype, male type, or severe with or without induction of genes related to membrane protein production. Conclusions Unbiased transcriptome analysis of a peripheral blood component provides insight on autoimmune pathophysiology and patient variability. We present an open source workflow and richly annotated dataset to support investigation of T cell biology, develop biomarkers for patient stratification and perhaps help indicate a source of SLE immune dysfunction. PMID:26544975

  14. The Association of the GABRP Polymorphisms with Systemic Lupus Erythematosus

    PubMed Central

    Kim, Hun-Soo; Jin, Eun-Heui; Mo, Ji-Su; Shim, Hyeok; Lee, Shin-Seok; Chae, Soo-Cheon

    2015-01-01

    Gamma-aminobutyric acid receptor subunit pi (GABRP) is involved in inhibitory synaptic transmission in the central nervous system. This gene encodes multisubunit chloride channels and is also expressed in numerous nonneuronal tissues such as the uterus and the ovaries. This study was aimed to validate whether the polymorphisms in the GABRP gene are associated with the susceptibility to systematic lupus erythematosus (SLE). The genotype frequencies of the rs929763, rs732157, and rs3805455 of the GABRP gene in SLE patients were significantly different from those of the control group (P < 0.0001, P = 0.05 and 0.002, resp.). Additional analysis showed that the genotype of the rs929763 and rs3805455 of the GABRP gene were also significantly associated with female SLE patients (P < 0.0001, P = 0.005, resp.). Two haplotype frequencies including a major haplotype of GABRP SNPs were more significantly different between the SLE patients and the healthy controls (P = 0.038 and 4.2E − 24, resp.). These results suggest that the polymorphisms in the GABRP gene might be associated with the susceptibility to SLE and the haplotype of GABRP SNPs is useful genetic marker for SLE. PMID:26634217

  15. Liver abnormalities and liver membrane autoantibodies in systemic lupus erythematosus.

    PubMed Central

    Kushimoto, K; Nagasawa, K; Ueda, A; Mayumi, T; Ishii, Y; Yamauchi, Y; Tada, Y; Tsukamoto, H; Kusaba, T; Niho, Y

    1989-01-01

    The hepatic involvement of 57 patients with systemic lupus erythematosus (SLE) was studied with special reference to liver membrane autoantibody (LMA). Liver abnormalities were found predominantly in patients with active SLE (27/48 (56%) in active SLE v 3/20 (15%) in inactive SLE). They were, however, rather mild or moderate and tended to disappear as the disease activity of SLE decreased. In this respect the liver abnormalities observed in this study differed from those in patients with lupoid hepatitis. The incidence of LMA in active SLE (8/11 (73%] was significantly greater than that in inactive SLE (4/12 (33%)). The mean LMA index value in active SLE was 8.3, which was also greater than the 2.9 in inactive SLE. Furthermore, in active SLE the mean LMA titre was significantly higher in patients with liver abnormalities than in those without. These results suggest that LMA may be associated with the activity of SLE and may be one of the factors which cause transient liver abnormalities. Images PMID:2596885

  16. [Aseptic necrosis of the temporomandibular joint in systemic lupus erythematosus].

    PubMed

    Grinin, V M; Maksimovskiĭ, Iu M; Nasonova, V A

    1999-01-01

    A total of 285 patients with verified systemic lupus erythematosus (SLE) were examined. They complained of painful temporo-mandibular joint (TMJ) and/or limited mobility of the mandible. Eight patients with chronic arthritis of the TMJ and a shortened (by 3-4 mm) mandibular branch were detected. In 4 female patients aged 25-38 with SLE lasting for about 5.5 years, unilateral signs of myoarticular dysfunction of the TMJ, flattening and decrease of the articular head without erosion and destruction, subcortical round foci with uneven internal contours were found in the central or median part of the head, with the compact bone above the foci thinned and the articular surface flattened; this prompted us to regard these changes as avascular necrosis (AN) of the joint. Shortening of the mandibular branch was caused by deformation of the neck of the articular process and its declination backwards. The detected changes in the TMJ were not accompanied by involvement of other joints. All these 4 patients with SLE and TMJ AN suffered from cerebropathy with epileptic attacks (frequent in 2 patients), Raynaud's syndrome, and bright capillaries of the palms and soles; 2 patients developed clinical picture of the antiphospholipid syndrome. Computer tomography and magnetic resonance findings validating the development of TMJ AN in?

  17. Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology

    PubMed Central

    Berntsson, Shala Ghaderi; Katsarogiannis, Evangelos; Lourenço, Filipa; Moraes-Fontes, Maria Francisca

    2016-01-01

    Progressive multifocal leukoencephalopathy (PML) caused by reactivation of the JC virus (JCV), a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE). We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine) since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions) and JCV isolation from the cerebrospinal fluid (700 copies/ml). IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need. PMID:27065427

  18. Taxonomy for Systemic Lupus Erythematosus with Onset before Adulthood

    PubMed Central

    Silva, Clovis A; Avcin, Tadej; Brunner, Hermine I

    2012-01-01

    Objective To propose a common nomenclature to refer to individuals who fulfill the American College of Rheumatology Classification Criteria for systemic lupus erythematosus (SLE) during childhood or adolescence. Methods The medical literature was reviewed for studies conducted in the target population between 1960 and December of 2011 to obtain information about the terms used to refer to such children and adolescents. We reviewed the threshold ages used and disease features considered to discriminate these individuals from patients with onset of SLE during adulthood. Furthermore, the nomenclature used in other chronic diseases with onset during both childhood and adulthood was assessed. Results There was an astonishing variability in the age cut-offs used to define SLE-onset prior to adulthood, ranging from 14 to 21 years but most studies used 18 years of age. The principal synonyms in the medical literature were SLE without reference to the age at onset of disease, childhood-onset SLE, juvenile SLE, and pediatric (or paediatric) SLE. Conclusions Based on the definition of childhood, in analogy with other complex chronic disease commencing prior to adulthood, and given the current absence of definite genetic variations that discriminate adults from children, the term childhood-onset SLE is proposed when referring to individuals with onset of SLE prior to age 18 years. PMID:22730317

  19. Undiagnosed Systemic Lupus Erythematosus Presenting as Hemophagocytic Lymphohistiocytosis

    PubMed Central

    Rahal, Ahmad K.; Fernandez, Justin; Dakhil, Christopher

    2015-01-01

    Hemophagocytic Lymphohistiocytosis (HLH) is rarely diagnosed in adults. Incidence is reported as one case per million persons per year. It can be triggered by conditions that affect immune homeostasis as infections, malignancies, and rheumatologic disorders. The following case demonstrates a rare instance in which undiagnosed systemic lupus erythematosus (SLE) presented as HLH. A 28-year-old male presented with progressive weakness and recurrent fevers for 2 months. Vital signs were within normal limits except for temperature of 100.3°F. His exam was unremarkable except for a left cervical scar and malar rash. His labs showed pancytopenia with neutropenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Hemophagocytosis was present on bone marrow biopsy. All workup for a source of infection was negative. A tentative diagnosis of HLH was made based on clinical presentation and laboratory data. The patient was treated with an HLH protocol. Later, it was determined that his HLH was actually secondary to a primary diagnosis of SLE. The patient was treated for SLE with an immunosuppressive regimen of cyclosporine and dexamethasone, and he improved dramatically. HLH rarely presents due to a rheumatologic condition such as SLE. Physicians should consider testing for SLE in patients diagnosed with HLH. PMID:26236531

  20. Interaction between glutathione and Apoptosis in Systemic Lupus Erythematosus

    PubMed Central

    Shah, Dilip; Sah, Sangita; Nath, Swapan K.

    2013-01-01

    Systemic lupus erythematosus (SLE) is characterized by imbalance redox state and increased apoptosis. The activation, proliferation and cell death of lymphocytes are dependent on intracellular levels of glutathione and controlled production of reactive oxygen species (ROS). Changes in the intracellular redox environment of cells, through oxygen-derived free radical production known as oxidative stress, have been reported to be critical for cellular immune dysfunction, activation of apoptotic enzymes and apoptosis. The shift in the cellular GSH-to-GSSG redox balance in favor of the oxidized species, GSSG, constitutes an important signal that can decide the fate of the abnormal apoptosis in the disease. The current review will focus on four main areas: (1) general description of oxidative stress markers in SLE, (2) alteration of redox state and complication of disease (3) role of redox mechanisms in the initiation and execution phases of apoptosis, and (4) intracellular glutathione and its checkpoints with lymphocyte apoptosis represent novel targets for pharmacological intervention in SLE. PMID:23279845

  1. The complement system in systemic lupus erythematosus: an update.

    PubMed

    Leffler, Jonatan; Bengtsson, Anders A; Blom, Anna M

    2014-09-01

    The complement system plays a major role in the autoimmune disease, systemic lupus erythematosus (SLE). However, the role of complement in SLE is complex since it may both prevent and exacerbate the disease. In this review, we explore the latest findings in complement-focused research in SLE. C1q deficiency is the strongest genetic risk factor for SLE, although such deficiency is very rare. Various recently discovered genetic associations include mutations in the complement receptors 2 and 3 as well as complement inhibitors, the latter related to earlier onset of nephritis. Further, autoantibodies are a distinct feature of SLE that are produced as the result of an adaptive immune response and how complement can affect that response is also being reviewed. SLE generates numerous disease manifestations involving contributions from complement such as glomerulonephritis and the increased risk of thrombosis. Furthermore, since most of the complement system is present in plasma, complement is very accessible and may be suitable as biomarker for diagnosis or monitoring of disease activity. This review highlights the many roles of complement for SLE pathogenesis and how research has progressed during recent years.

  2. Renal histology and pregnancy performance in systemic lupus erythematosus.

    PubMed

    Devoe, L D; Loy, G L; Spargo, B H

    1983-01-01

    Previous reports indicate that maternal and fetal outcome in pregnancies complicated by systemic lupus erythematosus (SLE) may be strongly influenced by the presence of renal disease. As the relationship between renal histology and clinical function in SLE is not consistent, prospective data on the outcomes of such pregnancies would aid patient counselling. Fifteen women with SLE had 18 pregnancies subsequent to renal biopsies, performed from 3 months to 8 years prior to conception. Their renal function was evaluated before, during and after pregnancy. Fourteen of 15 patients had evidence of renal involvement, based on by light and electron microscopic sections: 7 had mesangial involvement (WHO Class II); 5 had active focal or diffuse glomerulonephritis (Classes III and IV); two had membranous involvement (Class V); 1, no evident disease. Perinatal outcome was similar whether lesions were milder (8 continuing pregnancies, 4 term deliveries) or more severe (6 continuing pregnancies, 3 term deliveries). Clinical renal function was normal in all but 3 cases at the beginning of pregnancy; 2 additional patients experienced moderate deteriorations in renal function during pregnancy but recovered normal function in the puerperium. Fetal outcome was abnormal (3 premature deliveries, 1 neonatal death, 1 spontaneous abortion) in all cases where renal function was decreased, while 10 of 13 pregnancies in patients with normal renal function ended in term deliveries. The data suggest that currently preconceptual renal histology provides a less accurate basis for perinatal counselling than does the assessment of clinical renal function.

  3. Viruses as potential pathogenic agents in systemic lupus erythematosus.

    PubMed

    Nelson, P; Rylance, P; Roden, D; Trela, M; Tugnet, N

    2014-05-01

    Genetic and environmental factors appear to contribute to the pathogenesis of systemic lupus erythematosus (SLE). Viral infections have been reported to be associated with the disease. A number of exogenous viruses have been linked to the pathogenesis of SLE, of which Epstein-Barr virus (EBV) has the most evidence of an aetiological candidate. In addition, human endogenous retroviruses (HERV), HRES-1, ERV-3, HERV-E 4-1, HERV-K10 and HERV-K18 have also been implicated in SLE. HERVs are incorporated into human DNA, and thus can be inherited. HERVs may trigger an autoimmune reaction through molecular mimicry, since homology of amino acid sequences between HERV proteins and SLE autoantigens has been demonstrated. These viruses can also be influenced by oestrogen, DNA hypomethylation, and ultraviolet light (UVB) exposure which have been shown to enhance HERV activation or expression. Viral infection, or other environmental factors, could induce defective apoptosis, resulting in loss of immune tolerance. Further studies in SLE and other autoimmune diseases are needed to elucidate the contribution of both exogenous and endogenous viruses in the development of autoimmunity. If key peptide sequences could be identified as molecular mimics between viruses and autoantigens, then this might offer the possibility of the development of blocking peptides or antibodies as therapeutic agents in SLE and other autoimmune conditions.

  4. Senescent profile of angiogenic T cells from systemic lupus erythematosus patients.

    PubMed

    López, Patricia; Rodríguez-Carrio, Javier; Martínez-Zapico, Aleida; Caminal-Montero, Luis; Suarez, Ana

    2016-03-01

    The chronic inflammatory environment associated with systemic lupus erythematosus can lead to an accelerated immunosenescence responsible for the endothelial damage and increased cardiovascular risk observed in these patients. The present study analyzed two populations with opposite effects on vascular endothelium, angiogenic T cells and the senescent CD4(+)CD28(null) subset, in 84 systemic lupus erythematosus patients and 46 healthy controls. Also, 48 rheumatoid arthritis patients and 72 individuals with traditional cardiovascular risk factors participated as disease controls. Phenotypic characterization of CD28(+) and CD28(null) cells was performed by analyzing markers of senescence (CCR7, CD27, CD57) and cytotoxicity (CD56, perforin, granzyme B, IFN-γ). IL-1β, IL-6, IL-8, IL-10, IL-12, IL-17A, IFN-α, IFN-γ, TNF-α, B lymphocyte stimulator, and GM-CSF serum levels were analyzed in systemic lupus erythematosus patients and healthy controls. CD4(+)CD28(null) cells were notably increased in the systemic lupus erythematosus patients and disease controls compared with healthy controls. In contrast, angiogenic T cells were only reduced in the disease controls (those with rheumatoid arthritis or traditional cardiovascular risk factors). Nevertheless, an anomalous presence of CD28(null)-angiogenic T cells, with cytotoxic and senescent characteristics, was noted in systemic lupus erythematosus patients in association with anti-dsDNA titer, anti-SSA/Ro antibodies and circulating TNF-α, IL-8, IFN-α, and B lymphocyte stimulator amounts. This subset was also detected in those with traditional cardiovascular risk factors but not in the rheumatoid arthritis patients. In contrast, CD28(+)-angiogenic T cells were reduced in the systemic lupus erythematosus patients with cardiovascular disorders. In conclusion, CD28 expression must be used to redefine the angiogenic T cell population, because in pathologic conditions, a senescent CD28(null)-angiogenic T cell subset with

  5. Autoimmunity induced by human cytomegalovirus in patients with systemic lupus erythematosus

    PubMed Central

    2012-01-01

    Human cytomegalovirus is a common herpesvirus that is linked to autoimmunity, especially in genetically predisposed persons. The article by Hsieh and colleagues in a previous issue of Arthritis Research & Therapy suggests that a C-terminal peptide of the human cytomegalovirus protein pp65 is highly immunogenic in patients with systemic lupus erythematosus and that antibodies against this peptide cross-react with nuclear proteins and double-stranded DNA, which are highly frequent autoantibodies in systemic lupus erythematosus patients. These observations highlight the fact that immunization with one small cytomegalovirus-specific peptide results in multiple autoreactive antibodies, probably through molecular mimicry and epitope spreading, in genetically predisposed persons. PMID:22277352

  6. Is there an association between systemic lupus erythematosus and periodontal disease?

    PubMed

    Calderaro, Débora Cerqueira; Ferreira, Gilda Aparecida; de Mendonça, Santuza Maria Souza; Corrêa, Jôice Dias; Santos, Fabrícia Xavier; Sanção, João Guilherme Capinam; da Silva, Tarcília Aparecida; Teixeira, Antônio Lúcio

    2016-01-01

    Periodontal disease results from the interaction between pathogenic bacteria that colonize supragingival and subgingival biofilms and the host, triggering an inflammatory response, with systemic effects leading to immune-mediated destruction of the attachment apparatus and loss of supporting alveolar bone. Immunological pathways and predisposing genetic factors common to periodontal disease and rheumatic diseases, including systemic lupus erythematosus, have been described. Case reports have suggested greater severity of periodontal disease in patients with systemic lupus erythematosus. However, studies evaluating the influence of the treatment of one disease on the clinical and laboratory manifestations of the other have yielded conflicting results.

  7. An unusual case of Streptococcus agalactiae meningitis in a patient with sys-temic lupus erythematosus

    PubMed Central

    Protonotariou, E; Arampatzi, A; Ourailoglou, V; Diza, E; Skoura, L

    2015-01-01

    Background: Streptococcus agalactiae (group B Streptococcus) is a major cause of sepsis and meningitis in neonates and an important cause of invasive disease in adults. Case description: We describe an unusual case of fatal bacterial meningitis caused by Streptococcus agalactiae in a young man suffering from systemic lupus erythematosus for over 20 years. The young man was transferred intubated in AHEPA University Hospital in a coma; twenty-four hours upon arrival and despite intense invasive treatment, he died from multiple organ failure. Conclusion: The risk of serious infections in patients with systemic lupus erythematosus even under treatment with moderate doses of corticosteroids is high. Hippokratia 2015; 19 (4):372-373.

  8. Sub-acute cutaneous lupus erythematosus following nitrofurantoin: causative or coincidental?

    PubMed

    Murie, Jill; Agarwal, Monica

    2014-11-01

    A 70-year-old woman presented with progressive skin lesions on the face, limbs and trunk in the absence of systemic illness. Three months earlier, she had been prescribed six months prophylactic nitrofurantoin for recurrent urinary tract infections, treated with nitrofurantoin and trimethoprim. Positive immunology and histological inflammatory changes in a skin biopsy were consistent with a diagnosis of sub-acute cutaneous lupus erythematosus. Following treatment with topical steroids, the skin lesions regressed, but alopecia followed and required hydroxychloroquine. One year later, there are no new skin lesions and no evidence of systemic lupus erythematosus. Nitrofurantoin is associated with many side effects and hypersensitivity reactions. Possible drug-induced lupus reactions due to nitrofurantoin include pneumonitis, blood disorders and hepatotoxicity. This is the only published case of isolated sub-acute cutaneous lupus following maintenance nitrofurantoin.

  9. Salmonella septic arthritis of the knees in a patient with systemic lupus erythematosus.

    PubMed

    Kim, S S; Perino, G; Boettner, F; Miller, A; Goodman, S

    2013-06-01

    Hematogenous Salmonella osteomyelitis is uncommon in immunocompetent hosts, but occurs with some regularity in immunosuppressed patients affected by systemic lupus erythematosus (SLE). Surgical debridement with resection of compromised tissue is central to the surgical management of osteomyelitis. Persistence of septic arthropathy may result from inadequate debridement, areas of osteonecrosis (ON), and an abnormal cellular and humoral dysregulation characteristic of SLE. We describe a 53-year-old Hispanic female with SLE on immunosuppressive therapy, who developed acute salmonella-induced septic arthritis and osteomyelitis of both knees associated with ON and recurrent SLE synovitis. She received prolonged antibiotic therapy and an extensive surgical debridement as part of a successful two-stage bilateral total knee replacement. This report illustrates the significance of Salmonella enterica infection in SLE patients, and the role of underlying bone and joint pathology such as bone infarcts, sub-acute osteomyelitis, and SLE synovitis.

  10. Rituximab: an emerging treatment for recurrent diffuse alveolar hemorrhage in systemic lupus erythematosus.

    PubMed

    Tse, J R; Schwab, K E; McMahon, M; Simon, W

    2015-06-01

    Diffuse alveolar hemorrhage (DAH) is a rare manifestation of systemic lupus erythematosus (SLE) and is associated with high mortality rates. Treatment typically consists of aggressive immunosuppression with pulse-dose steroids, cyclophosphamide, and plasma exchange therapy. Mortality rates remain high despite use of multiple medical therapies. We present a case of recurrent DAH in a 52-year-old female with SLE after a deceased donor renal transplant who was successfully treated with rituximab. Our report highlights the pathophysiologic importance of B-cell-mediated immunosuppression in SLE-associated DAH and suggests that rituximab may represent a viable alternative to cyclophosphamide in the treatment of this disease. We also review eight other reported cases of rituximab use in SLE-associated DAH.

  11. Complement-fixing properties of antinuclear antibodies distinguish drug-induced lupus from systemic lupus erythematosus.

    PubMed

    Rubin, R L; Teodorescu, M; Beutner, E H; Plunkett, R W

    2004-01-01

    The immunofluorescence antinuclear antibody (ANA) test has been widely used to monitor autoimmune disease, but its value for diagnostic purposes is compromised by low specificity and high prevalence in disease-free individuals. The capacity of autoantibodies to fix serum complement proteins when bound to antigen is an important effector function because this property is associated with acute and chronic inflammatory processes. The current study evaluates the complement-fixing properties of antinuclear antibodies (CANA) in three well-defined and clinically-related patient groups: systemic lupus erythematosus (SLE), drug-induced lupus (DIL) and drug-induced autoimmunity (DIA). Of 20 patients diagnosed with SLE, 90% displayed complement-fixing ANA while this feature was present in only two of 18 patients with DIL and no patients with DIA without associated disease even though the mean ANA titres were similar among these patient groups. CANA was significantly correlated with anti-Sm activity. Because SLE but not DIL or DIA can be a life-threatening disease associated with complement consumption in vivo, these results demonstrate that measurement of CANA is a diagnostically useful tool and may have immunopathologic implications.

  12. Acute lupus pneumonitis followed by intestinal pseudo-obstruction in systemic lupus erythematosus: A case report

    PubMed Central

    JI, CAIHONG; YU, XING; WANG, YONG; SHI, LUFENG

    2016-01-01

    Intestinal pseudo-obstruction (IpsO) and acute lupus pneumonitis (ALP) are uncommon severe complications of systemic lupus erythematosus (SLE). The present study reports the case of a 26-year-old female who presented with abdominal pain, nausea and vomiting as initial symptoms. Computed tomography (CT) scanning revealed the jejunal wall was thickened and streaky, mimicking the presentation of intestinal obstruction. Following emergency surgery, the patient's general condition was aggravated, with evident limb erythematous rashes. A series of laboratory examinations revealed SLE, and combined with patient's medical history IpsO was diagnosed, with a disease Activity Index score of 10. During the therapeutic period, high fever, dyspnea and oxygen saturation (SaO2) reductions were detected, and CT scans indicated lung infiltration, excluding other causes through a comprehensive infectious work-up and a bronchoalveolar lavage examination. ALP was confirmed and treated with high-dose methylprednisolone and gamma globulin supplement. The patient responded well and was discharged in 2 weeks. In the one-year tapering period and after stopping corticosteroids, the patient recovered well with no relapse detected. In conclusion, the manifestation of IpsO in SLE is rare and represents a challenge for the surgeon to establish the correct diagnosis and avoid inappropriate surgical intervention. ALP may be the consequence of emergency surgery, and immediate high-dose glucocorticoid therapy is recommended. PMID:27347044

  13. Satisfaction with control of systemic lupus erythematosus and lupus nephritis: physician and patient perspectives

    PubMed Central

    Mozaffarian, Neelufar; Lobosco, Steve; Lu, Peng; Roughley, Adam; Alperovich, Gabriela

    2016-01-01

    Purpose Patient satisfaction with disease control of systemic lupus erythematosus (SLE) is an important component of medical management. This analysis evaluated patient and physician satisfaction with disease control of SLE, factors associated with satisfaction/dissatisfaction, and the degree of physician–patient concordance of these parameters. Patients and methods Data were extracted from the US Adelphi Real World Lupus Disease Specific Programme®, a cross-sectional survey of 50 rheumatologists, 25 nephrologists, and their patients with non-nephritis SLE (NNSLE) or lupus nephritis (LN). Results Physicians reported moderate or severe disease activity in 25.0% of patients with NNSLE and in 50.5% of patients with LN, and were satisfied with disease control in 78.6% (132/168) and 73.8% (152/206) of patients, respectively. For patients, 75.8% (75/99) with NNSLE were satisfied with their current treatment, compared with 65.5% (74/113) with LN. Physician–patient agreement (70.7%) on the level of satisfaction was “slight” (kappa =0.1445) for NNSLE; patients were more frequently dissatisfied than physicians with regard to joint tenderness, fatigue, anxiety, pain on movement, malar rash, and photosensitivity. Physician–patient agreement (71.4%) on the level of satisfaction was “fair” (kappa =0.3695) for LN; patients expressed greater dissatisfaction than physicians for headache, photosensitivity, and anxiety, whereas physicians were more dissatisfied with regard to joint swelling, kidney function, and blood pressure control. In general, patients with NNSLE or LN who were dissatisfied (or whose physicians were dissatisfied) were more likely to have joint swelling, joint stiffness, malar rash, hair loss, depression, and fatigue, have moderate or severe disease, or to be currently experiencing disease flare. Conclusion These data highlight the patient and physician dissatisfaction with real-world disease control of SLE. PMID:27784995

  14. Low molecular weight C1q in systemic lupus erythematosus.

    PubMed

    Hoekzema, R; Hannema, A J; Swaak, T J; Paardekooper, J; Hack, C E

    1985-07-01

    In sera of patients suffering from an exacerbation of systemic lupus erythematosus (SLE), increased amounts of abnormal C1q were detected, contrasting with decreased or even undetectable levels of normal C1q in these sera. When analyzed immunochemically by double immunodiffusion, this low m.w. C1q (LMW-C1q) appeared to be identical with the defective C1q in serum of individuals with an inherited, homozygous inability to produce functional plasma C1q. These persons show a tendency to develop SLE-like syndromes. Like the genetically defective C1q, the abnormal C1q molecule in SLE sera was hemolytically inactive, did not incorporate in C1, was found in the supernatant of euglobulin-precipitated serum, and appeared in the break-through fraction of a cation-exchange column. Sucrose gradients and gel filtration analyses supported the putative identity of the molecules. SDS-PAGE and immunoblots revealed the presence of subunits that reacted with antibodies against C1q and confirmed the C1q-like nature of LMW-C1q. Low levels of LMW-C1q were also detected in serum and plasma of normal individuals. A radial immunodiffusion technique was used to measure LMW-C1q in the serum of 54 patients. Although these patients were not selected for parameters of disease activity, their levels of LMW-C1q were significantly higher than those of normal individuals and children with decreased C3 levels due to acute glomerulonephritis.

  15. Osteoporosis in murine systemic lupus erythematosus--a laboratory model.

    PubMed

    Schapira, D; Kabala, A; Raz, B; Israeli, E

    2001-01-01

    The aim of this study was to assess the skeletal metabolism in a murine model of systemic lupus erythematosus (SLE). MRL/n and MRL/l mice (respectively representing a benign and a malignant form of the disease) were observed from 1.5 to 6.5 months of life. The monthly follow-up included: biochemical and histomorphometrical studies of the femoral bone, serum biochemistry, immunoglobulins and osteocalcin, and histological evaluation of the kidney tissue. The results showed a higher femoral weight (+11.5%), calcium (+4.4%) and protein bone content (+11.4%) and a significantly higher (+77%) phosphorus bone content in the MRL/n group; significantly lower (-48.9%) bone alkaline phosphatase enzymatic activity, lower bone alkaline/acid phosphatase enzymatic activities ratio (-40.8%) and lower (-38.4%) serum osteocalcin values in the MRL/l group (which might suggest reduced bone formation in these animals); markedly smaller trabecular bone volume (BV/TV) in the femoral head (-36.2%) and femoral neck (-39.8%), and smaller cortical and femoral areas in the mid-femoral shaft (-38.8% and -38.1% respectively) in the MRL/l group; higher serum immunoglobulins, increased serum blood urea nitrogen (BUN) and creatinine and a higher index of activity in the kidney histology in the MRL/l group, indicating increased activity of the disease in this substrain. The MRL mice, through their two substrains, may serve as a valuable laboratory animal model for study of the skeletal changes in SLE and of the influence of the disease activity on the skeletal metabolism.

  16. Fc-rosette inhibition by hypocomplementaemic systemic lupus erythematosus sera.

    PubMed

    Morito, T; Tanimoto, K; Hashimoto, Y; Horiuchi, Y; Juji, T

    1975-10-01

    Human red cells sensitized with one of the Rh antisera (Ripley) form rosettes (Fc-rosette) with human B lymphocytes and the rosettes are well inhibited by aggregated human IgG. Since sera of hypocomplementaemic patients with systemic lupus erythematosus (SLE) have frequently been reported to contain immune complexes, they were used for the inhibition of Fc-rosette formation in this study. The results of Fc-rosette inhibition rates of the sera were inversely correlated with the serum CH50 levels. When the sera were separated into top, middle, and bottom fractions by ultracentrifugation, the bottom fractions showed more effective inhititions than the others. Similarly, the strongest inhibition was found in the void volume of the serum separated by Sephadex G200 gel filtration. Reduction and alkylation of IgG resulted in the loss of reactivity with Fc receptor of B lymphocytes, and the rosette inhibiting activities of the SLE sera were markedly reduced after reduction and alkylation. Some of anti-HLA sera were inhibitory for the Fc-rosette formation, while the tested sera did not contain anti-HLA activity assessed by the microcytotoxicity test. These results indicated that circulating immune complexes contained in the sera inhibit the rosette formation, and that the Fc-rosette inhibition test is a simple and relatively sensitive method for the detection of circulating immune complexes. Antinuclear antibody activities of the sera were tested by the indirect immunofluorescent method; however, clear correlations were not obtained between Fc-rosette inhibition rates and staining patterns of antinuclear antibodies. On the other hand, the positive groups of LE-test exhibited slightly greater inhibition rates of the rosette than the negative groups.

  17. Aging and Systemic Lupus Erythematosus - Immunosenescence and Beyond.

    PubMed

    van den Hoogen, Lucas Laurens; Sims, Gary Patrick; van Roon, Joel Adrianus Gijsbert; Fritsch-Stork, Ruth Dorothea Elisabeth

    2015-01-01

    The lifespan of humans has increased drastically over the last decades; considerable effort has been applied to delineate the mechanisms behind aging in order to find strategies for longevity. As the benefits of the gained knowledge might extend to diseases, where accelerated aging is suspected, the role of aging in the systemic autoimmune disease Systemic Lupus Erythematosus (SLE) is of particular interest. In this review the immunological similarities of SLE and aging are analyzed on three levels: the clinical, the cellular and the molecular, in order to find possible common pathological mechanisms. Common clinical features (e.g. increased infection rates, incidence of tumors and cardiovascular diseases) of SLE-patients and elderly individuals and shared characteristics of immuno-senescence and SLE are identified. These similarities are strongest in the adaptive immune system, where terminally differentiated T-cells and an immunological risk profile are found in both conditions. Also the aging innate immune system has overlapping features with SLE, exemplified by a generally lowered phagocytic capacity. However, great disparities between the aging immune system and SLE become apparent on a closer look, affecting numbers, phenotype and function of most immune cells, ranging from NETosis by granulocytes to the mechanisms underlying abnormal IL-2 production by T-cells. On the molecular level, also the increased presence of aging mechanisms like telomere attrition, DNA damage, autophagy and the characteristics of the mTOR pathway in SLE, possibly contributing to the shared changes on the cellular and clinical level are elaborated. The possible implications thereof concern existing (hydroxychloroquine, rapamycine, Glucocorticoids) as well as novel therapeutic strategies targeting more specific pathways which might rapidly reach the clinical arena. Overall a differential view on the similarities of aging and SLE and possible consequences is presented. PMID:26212055

  18. Work Dynamics Among Persons With Systemic Lupus Erythematosus

    PubMed Central

    YELIN, EDWARD; TRUPIN, LAURA; KATZ, PATRICIA; CRISWELL, LINDSEY; YAZDANY, JINOOS; GILLIS, JOANN; PANOPALIS, PETER

    2010-01-01

    Objective To track changes in the proportion of persons ages 18–64 with systemic lupus erythematosus (SLE) who were employed from diagnosis through 2004, to estimate changes in annual work hours during this time, and to describe risk factors for work loss among those employed at diagnosis. Methods A structured telephone survey was administered to a cohort of 982 persons with SLE, which was assembled between 2002 and 2004. Of the 900 enrolled in 2002–2003, 832 (92%) were re-interviewed in 2004. We tabulated the proportion employed at diagnosis, at baseline interview, and at followup in 2004. Among individuals employed at each time frame, we estimated the hours of work per year. We then used the Kaplan-Meier method to estimate time until work loss among individuals employed at diagnosis and Cox proportional hazards regression to describe the risk factors for such work loss. Results Between diagnosis and followup interview, the proportion employed declined from 74% to 54%. Over the same period, hours of work per year declined by 32.2% among all individuals with a work history, but by only 1% among those continuously employed. Among individuals working at diagnosis, the proportion employed declined by 15% and 63% after 5 and 20 years, respectively. Demographics (age, sex, and education) and work characteristics (physical and psychological demands of jobs and level of control) were the principal determinants of work loss. Conclusion Total cessation of employment, rather than reduced hours among employed persons, accounts for most of the decline in annual work hours among persons with SLE. PMID:17266065

  19. Tuberculosis among Filipino patients with systemic lupus erythematosus.

    PubMed

    Victorio-Navarra, S T; Dy, E E; Arroyo, C G; Torralba, T P

    1996-12-01

    A retrospective review of the clinical records of 54 patients with systemic lupus erythematosus (SLE) and documented tuberculosis (TB) infection seen at the University of Santo Tomas Hospital was accomplished. There were 53 women and one man, with a mean age of 32.2 +/- 10 years and a total of 57 TB occurrences. Pulmonary involvement was recorded in 42 (74%): upper lungfield in 25, mid to lower lungfield in 7, and miliary pattern or diffuse infiltrates in 10. TB arthritis was noted in 8, osteomyelitis in 4, and soft tissue abscesses in 4. Central nervous system involvement consisted of brain abscesses (tuberculomas) in two and meningitis in one. Two patients each had TB lymphadenitis, genitourinary TB, ileocecal TB, and TB peritonitis. Hepatobiliary and cutaneous TB occurred in one patient each. Eight of 10 patients with disseminated or miliary TB died primarily of respiratory failure; six of these eight patients also had some form of extrapulmonary involvement. Using the Wilcoxon rank-sum test, there were significant differences in the mean SLE Disease Activity Index (SLEDAI) and Severity of Disease Index (SDI) scores between those with limited TB (SLEDAI 24 +/- 7 SD; SDI 19 +/- 18 SD) versus those with extensive TB (SLEDAI 41 +/- 16 SD; SDI 36 +/- 21 SD), P < .05. There was no significant difference in the average daily prednisone dose (mg) between those with limited TB (25 +/- 17 SD) versus those with extensive TB (31 +/- 16 SD). The contributory role of tuberculous infection in the morbidity and mortality of patients with SLE must be emphasized, especially in areas endemic for TB.

  20. Resilience and Treatment Adhesion in Patients with Systemic Lupus Erythematosus

    PubMed Central

    Faria, Daniella Antunes Pousa; Revoredo, Luciana Silva; Vilar, Maria José; Eulália Maria Chaves, Maia

    2014-01-01

    Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune, rheumatic inflammatory disease that can cause significant morbidity with evident psychological impacts and obvious harm to quality-of-life that require the patient to adapt treatment. Objective: Assessment of resilience and the self-reported treatment adhesion behaviors of patients with SLE, investigating which of these factors are associated to resilience. Method: Cross-sectional study of 40 women with SLE. A questionnaire with social demographic data, health history and the Wagnild Young Resilience Scale were used. Results: 62.5% followed the medical treatment properly but 55% found it difficult. 27.5% of the patients presented low resilience, 57.5% medium and 15% high resilience. Resilience was associated in the chi-square test (p-value < 0.05) with the variables work, understanding SLE, trying to find out about SLE, following the treatment correctly, difficulty in following the treatment and stopping some activity because of the disease. In the correlation analysis, resilience was associated with age (-0.3960), number of working hours (0.5533), specialized treatment duration (-0.8103) and disease duration from diagnosis (-0.8014). Conclusion: Patients with high resilience tended to follow treatment correctly, tried to understand the disease and adhered more to the treatment to avoid risks and promote protection factors. Therefore knowledge of resilience in patients with SLE is necessary. It is important that the state takes necessary actions to facilitate access to treatment, to educational programs and to medical support. Awareness and counselling sessions must be initiated to develop and promote individual capacities to learn how to tackle with the disease for which psychological support of family and doctors can play a significant role. PMID:24665352

  1. Molecular therapies for systemic lupus erythematosus: clinical trials and future prospects.

    PubMed

    Monneaux, Fanny; Muller, Sylviane

    2009-01-01

    The prognosis of patients with systemic lupus erythematosus has greatly improved since treatment regimens combining corticosteroids and immunosuppressive medications have been widely adopted in therapeutic strategies given to these patients. Immune suppression is evidently efficient but also leads to higher susceptibility to infectious and malignant diseases. Toxic effects and sometimes unexpectedly dramatic complications of current therapies have been progressively reported. Identifying novel molecular targets therefore remains an important issue in the treatment of lupus. The aim of this review article is to highlight emerging pharmacological options and new therapeutic avenues for lupus with a particular focus on non-antibody molecular strategies.

  2. [Hemorrhagic pericarditis and cardiac tamponade in systemic lupus erythematosus. A case report].

    PubMed

    Barrera-Ramírez, Carlos Felipe; Pineda-Pompa, Luis R; Melo, Mario; Valdez Castro, Ricardo; Medina-Gómez, Héctor; Godina-Alonso, Gustavo; Guzmán, Carlos E

    2005-01-01

    Systemic lupus erythematosus is a chronic inflammatory autoimmune disorder that can affect any organ or system. Although pericarditis is the most frequent cardiac manifestation of this entity, usually is not a life threatening situation. Pericardial effusion causing cardiac tamponade is a very rare complication in lupus, with an incidence less than 2%. We report a case of pericardial tamponade due to SLE with severe hemodynamic involvement in a 21-year-old woman associated to rapidly progressive glomerulonephritis, acute pancreatitis, acute acalculous cholecystitis, pleural effusion, hematologic, cutaneous and neurologic lupus activity. Recognition of this rare manifestation of SLE may be life saving. PMID:16366174

  3. Pure cutaneous lupus erythematosus in a population of African descent in French Guiana: a retrospective population-based description.

    PubMed

    Deligny, C; Marie, D Sainte; Clyti, E; Arfi, S; Couppié, P

    2012-11-01

    The objective of this study was to examine the characteristics of cutaneous lupus erythematosus, excluding systemic lupus erythematosus (SLE), in patients of African descent. Indeed, since the description of subacute cutaneous lupus erythematosus (SCLE), which had been included in chronic cutaneous lupus erythematosus (CCLE), there has been no description of the disease in black patients. In 2000, we performed a retrospective epidemiological study by querying multiple sources to identify all patients with lupus in French Guiana--a part of France in South America having western living conditions, free healthcare and 157,000 inhabitants, most of whom are of African origin. We found 45 patients with pure cutaneous lupus, which included CCLE (mostly discoid), SCLE and bullous lupus. The disease characteristics of these patients exhibited few differences compared with those of the Caucasian patients cited in the literature. However, the age of onset for our patients of African descent was younger than that of Caucasian patients. In contrast to the race-related differences reported for SLE, we found no major differences in terms of demographic, clinical and biological presentation between this cohort of pure cutaneous lupus erythematosus patients of African origin and Caucasian patients with similar forms of lupus.

  4. Hair dye treatment use and clinical course in patients with systemic lupus erythematosus and cutaneous lupus.

    PubMed

    Jiménez-Alonso, J; Sabio, J M; Pérez-Alvarez, F; Reche, I; Hidalgo, C; Jáimez, L

    2002-01-01

    The etiological role of hair dye treatment (HDT), some of them such as permanent hair dyes containing aromatic amines, in the development of SLE has been previously ruled out. However, the possible influence of HDT use on the course and prognosis of lupus patients has been assessed only in one short-term study. Since HDT is very extensive among the population, the knowledge of this possible negative effect may be very important. Thus, the aim of this study was to assess the long-term influence of several HDTs on the course and clinical severity of patients with both systemic lupus erythematosus (SLE) and cutaneous lupus (CL). In this longitudinal case series study, 91 SLE patients and 22 CL patients were prospectively studied from October 1988 to May 2000. They were divided into three groups: (a) non-HDT users--patients who have never used HDT (n = 65); (b) P-HDT users--HDT permanent type users, alone or in combination with other types of HDT (n = 28); (c) non P-HDT--users of other treatments different from permanent tinting (bleach, lowlights, etc; n = 20). In each patient we determined: (1) number of flares/year in SLE patients and worsening of cutaneous lesions for CL; (2) Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage index; (3) predominant damaged organs/systems according to the HDT use and type of HDT; and (4) subjective impression about the disease evolution in relation to HDT use. No significant differences were found with respect to flares/year and SLICC/ACR damage index between the study groups. Non-HDT group presented more renal involvement and serositis than both HDT-user groups. No patient related the HDT use to the worsening of his disease. Therefore, in this study no evidence of an association between the long-term use of several types of HDT and the clinical activity and course of SLE and CL was found.

  5. Hair dye treatment use and clinical course in patients with systemic lupus erythematosus and cutaneous lupus.

    PubMed

    Jiménez-Alonso, J; Sabio, J M; Pérez-Alvarez, F; Reche, I; Hidalgo, C; Jáimez, L

    2002-01-01

    The etiological role of hair dye treatment (HDT), some of them such as permanent hair dyes containing aromatic amines, in the development of SLE has been previously ruled out. However, the possible influence of HDT use on the course and prognosis of lupus patients has been assessed only in one short-term study. Since HDT is very extensive among the population, the knowledge of this possible negative effect may be very important. Thus, the aim of this study was to assess the long-term influence of several HDTs on the course and clinical severity of patients with both systemic lupus erythematosus (SLE) and cutaneous lupus (CL). In this longitudinal case series study, 91 SLE patients and 22 CL patients were prospectively studied from October 1988 to May 2000. They were divided into three groups: (a) non-HDT users--patients who have never used HDT (n = 65); (b) P-HDT users--HDT permanent type users, alone or in combination with other types of HDT (n = 28); (c) non P-HDT--users of other treatments different from permanent tinting (bleach, lowlights, etc; n = 20). In each patient we determined: (1) number of flares/year in SLE patients and worsening of cutaneous lesions for CL; (2) Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage index; (3) predominant damaged organs/systems according to the HDT use and type of HDT; and (4) subjective impression about the disease evolution in relation to HDT use. No significant differences were found with respect to flares/year and SLICC/ACR damage index between the study groups. Non-HDT group presented more renal involvement and serositis than both HDT-user groups. No patient related the HDT use to the worsening of his disease. Therefore, in this study no evidence of an association between the long-term use of several types of HDT and the clinical activity and course of SLE and CL was found. PMID:12195784

  6. Normal pressure hydrocephalus in the spectrum of neurological complications of systemic lupus erythematosus.

    PubMed

    de Oliveira, Fabricio Ferreira; Cardoso, Tania Aparecida Marchiori; Sampaio-Barros, Percival Degrava; Damasceno, Benito Pereira

    2013-06-01

    Normal pressure hydrocephalus is an unusual manifestation of systemic lupus erythematosus and its pathogenesis is still unclear. We report the case of a 39-year-old white woman with systemic lupus erythematosus who developed magnetic gait, speech difficulties, progressive memory impairment, urinary incontinence and episodes of involuntary closure of the eyelids. Signs and symptoms, associated with ventriculomegaly and normal cerebrospinal fluid pressure, suggested normal pressure hydrocephalus, which as a complication of systemic lupus erythematosus believably develops due to the insidious inflammatory process that occurs in the meningeal tissues or to the vasculitis itself. Normal pressure hydrocephalus tends to develop secondary to trauma, infection or subarachnoid haemorrhage, but in 50 % of patients no aetiology is found. Shunt surgery is the only effective treatment, specifically for the gait disorder, which usually improves more than the cognitive symptoms. Since the tap-test showed a strongly positive result, a medium pressure ventriculoperitoneal shunt was inserted, further replaced by a high pressure one in view of the complications, with less than expected improvement. Subdural hematomas and empyemas developed, requiring surgery and antibiotic therapy. A new tap-test was positive, and the patient improved only after a programmable valve was finally placed. However, pressure regulation shall be continuously required, and shunt dysfunction might still develop in the long term. The few similar case reports in the literature are reviewed, confirming the rarity of this neurological complication of systemic lupus erythematosus.

  7. Treatment of chronic discoid lupus erythematosus with an oral gold compound (auranofin).

    PubMed

    Dalziel, K; Going, G; Cartwright, P H; Marks, R; Beveridge, G W; Rowell, N R

    1986-08-01

    Twenty-three patients with severe longstanding discoid lupus erythematosus, unresponsive to conventional treatments, were treated with oral gold in a multicentre open study. Nineteen patients showed clinical improvement and in four of these there was complete resolution of lesions. Adverse reactions were generally mild and self limiting.

  8. Pulmonary manifestations of Sjögren syndrome, systemic lupus erythematosus, and mixed connective tissue disease.

    PubMed

    Mira-Avendano, Isabel C; Abril, Andy

    2015-05-01

    Interstitial lung disease is a common and often life-threatening manifestation of different connective tissue disorders, often affecting its overall prognosis. Systemic lupus erythematosus, Sjögren syndrome, and mixed connective tissue disease, although all unique diseases, can have lung manifestations as an important part of these conditions. This article reviews the different pulmonary manifestations seen in these 3 systemic rheumatologic conditions.

  9. Acute Bilateral Tuberculous Pneumonia in a Patient with Systemic Lupus Erythematosus

    PubMed Central

    Bhat, Rama; Bhat, Nitin; D’Souza, Savio; Chenchaiah, Venkata

    2016-01-01

    Pulmonary tuberculosis is a common infection associated with immunocompromised state. It usually presents with fibrosis or fibrocavitary lesions in the lung. We report a case of bilateral tuberculous pneumonia of acute presentation in a young lady who was being treated for systemic lupus erythematosus. PMID:27437280

  10. Immune Response Modulation by Vitamin D: Role in Systemic Lupus Erythematosus

    PubMed Central

    Iruretagoyena, Mirentxu; Hirigoyen, Daniela; Naves, Rodrigo; Burgos, Paula Isabel

    2015-01-01

    Vitamin D plays key roles as a natural immune modulator and has been implicated in the pathophysiology of autoimmune diseases, including systemic lupus erythematosus (SLE). This review presents a summary and analysis of the recent literature regarding immunoregulatory effects of vitamin D as well as its importance in SLE development, clinical severity, and possible effects of supplementation in disease treatment. PMID:26528285

  11. Specific Psychosocial and Behavioral Outcomes from the Systemic Lupus Erythematosus Self-Help Course.

    ERIC Educational Resources Information Center

    Braden, Carrie Jo; And Others

    1993-01-01

    Data from 104 participants in the Systemic Lupus Erythematosus Self-Help Course showed that patients had significant increases in enabling skills and use of relaxation/exercise and decreases in depression. Amount of time spent in class was correlated with significant changes over time. (SK)

  12. Effect of vitamin A treatment on the immune reactivity of patients with systemic lupus erythematosus.

    PubMed

    Vien, C V; González-Cabello, R; Bodó, I; Gergely, P

    1988-05-01

    Treatment of patients suffering from systemic lupus erythematosus with vitamin A (100,000 U daily for 2 weeks) resulted in an enhancement of antibody-dependent cell-mediated cytotoxicity, natural killer cell activity and blastogenic response to plant mitogens and interleukin-2 (IL-2). PMID:3184159

  13. Immune Response Modulation by Vitamin D: Role in Systemic Lupus Erythematosus.

    PubMed

    Iruretagoyena, Mirentxu; Hirigoyen, Daniela; Naves, Rodrigo; Burgos, Paula Isabel

    2015-01-01

    Vitamin D plays key roles as a natural immune modulator and has been implicated in the pathophysiology of autoimmune diseases, including systemic lupus erythematosus (SLE). This review presents a summary and analysis of the recent literature regarding immunoregulatory effects of vitamin D as well as its importance in SLE development, clinical severity, and possible effects of supplementation in disease treatment.

  14. [Neonatal lupus erythematosus: complete atrioventricular block and SSA/Ro antibodies].

    PubMed

    Prados, R; Maroto, E; López Longo, J; Monteagudo, I; Carreño, L; García, E J

    1987-06-01

    A newborn boy with complete A-V block and positive anti-SSA/Ro antibodies is reported. Authors comment on pathological findings of neonatal lupus erythematosus. They also review prognosis and clinical course and point out management of these patients before and after birth.

  15. Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus.

    PubMed

    Peñacoba Toribio, Patricia; Córica Albani, María Emilia; Mayos Pérez, Mercedes; Rodríguez de la Serna, Arturo

    2014-01-01

    Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. We report the case of a patient with non-responding SLS (neither to glucocorticoids nor immunosupresors), who showed remarkable improvement after the onset of treatment with rituximab. Although there is a little evidence, treatment with rituximab could be proposed in SLS when classical treatment fails.

  16. Myopericarditis and severe myocardial dysfunction as the initial manifestation of systemic lupus erythematosus

    PubMed Central

    Peñataro, Joaquín S; De Mingo, Ana; Del Río, Ana; Martínez, José A; Heras, Magda

    2012-01-01

    Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE). However, a large pericardial effusion as the initial manifestation of the disease is infrequent, especially when it is associated with myocardial damage. We describe an unusual case of a young female with pleuropericarditis and severe myocardial dysfunction as the initial manifestation of SLE. PMID:24062915

  17. Spontaneous retroperitoneal bleeding from renal microaneurysms and pancreatic pseudocyst in a patient with systemic lupus erythematosus.

    PubMed

    Melamed, N; Molad, Y

    2006-01-01

    Visceral vasculitis and pancreatic pseudocyst are rare manifestations of systemic lupus erythematosus (SLE). We describe a patient with SLE who presented with spontaneous bilateral perinephric and retroperitoneal haematoma secondary to polyarteritis nodosa (PAN)-like vasculitis of the renal arteries, which subsequently evolved into systemic vasculitis with pancreatic pseudocyst formation.

  18. Breakdown of Immune Tolerance in Systemic Lupus Erythematosus by Dendritic Cells.

    PubMed

    Liao, Xiaofeng; Reihl, Alec M; Luo, Xin M

    2016-01-01

    Dendritic cells (DC) play an important role in the pathogenesis of systemic lupus erythematosus (SLE), an autoimmune disease with multiple tissue manifestations. In this review, we summarize recent studies on the roles of conventional DC and plasmacytoid DC in the development of both murine lupus and human SLE. In the past decade, studies using selective DC depletions have demonstrated critical roles of DC in lupus progression. Comprehensive in vitro and in vivo studies suggest activation of DC by self-antigens in lupus pathogenesis, followed by breakdown of immune tolerance to self. Potential treatment strategies targeting DC have been developed. However, many questions remain regarding the mechanisms by which DC modulate lupus pathogenesis that require further investigations. PMID:27034965

  19. Cryptococcal meningitis in systemic lupus erythematosus patients: pooled analysis and systematic review.

    PubMed

    Fang, Wenjie; Chen, Min; Liu, Jia; Hagen, Ferry; Ms, Abdullah; Al-Hatmi; Zhang, Peilian; Guo, Yun; Boekhout, Teun; Deng, Danqi; Xu, Jianping; Pan, Weihua; Liao, Wanqing

    2016-01-01

    Cryptococcal meningitis is an important fungal infection among systemic lupus erythematosus patients. We conducted a pooled analysis and systematic review to describe the epidemiological and clinical profile of cryptococcal meningitis in systemic lupus erythematosus patients. From two hospitals in China and nine literature databases, cases and prevalence data were collected for pooled analysis and meta-analysis, respectively. Categorical variables of cases were compared using a χ(2)-test on the statistical program of SAS. A multiple regression analysis was performed to ascertain independent predictors significantly correlated with prognosis. Meta-analysis was conducted by the statistical program of R. The prevalence of cryptococcal meningitis in systemic lupus erythematosus patients was 0.5%. Patients were predominantly females and adults. A prednisone equivalent of more than 30 mg/day before infection was associated with higher mortality (odds ratio (OR)=9.69 (1.54, 60.73)). In all, 36.8-38.9% patients showed low lupus activity when they developed the crytococcal infection. Moreover, 38.2% of the patients were misdiagnosed. The estimated case-fatality rate was 23.6%. Our results suggest that more emphasis should be placed to further understand lupus-related cryptococcal meningitis and to develop better prophylaxis and management strategies to combat this condition. PMID:27599471

  20. Critical illness in systemic lupus erythematosus and the antiphospholipid syndrome

    PubMed Central

    Williams, F; Chinn, S; Hughes, G; Leach, R

    2002-01-01

    Objectives: To investigate the causes, course, and outcome of critical illness requiring emergency admission to the intensive care unit (ICU) in patients with systemic lupus erythematosus (SLE) or the antiphospholipid syndrome (APS), or both. Methods: Critically ill patients with SLE or APS, or both, admitted to a London teaching hospital ICU over a 15 year period were studied. Demographic, diagnostic, physiological, laboratory, and survival data were analysed. Kaplan-Meier survival curves were constructed by age, time from first diagnosis of SLE, and time from first ICU admission. The log rank test and a backwards stepwise Cox regression were used to identify factors associated with reduced survival. Results: Sixty one patients with SLE alone (39%) and/or APS (61%) required 76 emergency admissions to the ICU. Patients had high severity of illness scores (median APACHE II 22 (range 8–45)) and multiorgan dysfunction. The primary diagnoses for patients admitted were infection in 31/76 (41%), renal disease in 16/76 (21%), cardiovascular disease in 12/76 (16%), and coagulopathies in 11/76 (14%). The commonest secondary diagnosis was renal dysfunction (49%). Factors associated with an increased risk of death were cyclophosphamide before admission, low white cell count, and high severity of illness score. Before adjustment for these factors renal disease had a strong adverse effect on long term survival (analysis by age at diagnosis p=0.005, analysis by time since first ICU admission, p=0.07). After adjustment, infection at admission to ICU was associated with an increased ICU mortality (p=0.02) and was the cause of death in 13/17 patients who died in the ICU. Similarly, after adjustment, APS was associated with reduced ICU survival (p=0.1) and reduced long term (p=0.03) survival. Seventeen patients (28%) died in the ICU, and 31 patients (51%) had died by the last follow up. Median time from ICU admission to death was four years. Overall five year survival from the

  1. Markers of acute neuropsychiatric systemic lupus erythematosus: a multidisciplinary evaluation.

    PubMed

    Abda, Essam A; Selim, Zahraa I; Radwan, Moustafa E M; Mahmoud, Nagham M; Herdan, Omar M; Mohamad, Khalid A; Hamed, Sherifa A

    2013-05-01

    This study was aimed to assess: (1) the additive diagnostic utility of diffusion-weighted imaging (DWI) and magnetic resonance angiography (MRA) over conventional MRI in detecting brain lesions in patients with acute primary neuropsychiatric systemic lupus erythematosus (NPSLE), and (2) the relevance of their findings to the associated NP manifestations. Included were 34 patients with acute NPSLE with mean age of 33.26 ± 10.14 years and duration of illness of 3.33 ± 1.71 years. Clinical interviewing and psychiatric and cognitive evaluations were performed by applying the criteria of the diagnostic and statistical manual of mental health disorders criteria (DSM-IV), Stanford Binet Subset Testing, Mini-Mental State Examination and Wechsler Memory Scale-Revised. Serologic tests included looking for antinuclear antibodies, anti-double strand DNA, anti-phospholipid antibodies. Radiologic evaluation included conventional MRI, DWI and MRA. One or more NP manifestations were diagnosed in 28 patients, in which cognitive deficits were reported with headache, psychosis and CVS. Anti-phospholipid antibodies were reported in patients with CVS. Twenty patients (71.43 %) with primary NPSLE (n = 28) had MRI abnormalities in which hyperintense signals at subcortical and periventricular white matter and at the junction between the gray and white matter represented 75 % (n = 15) and with headache (n = 6), psychosis (n = 6) and acute confusional state (n = 3) with and without cognitive deficits, respectively. Moderate-sized infarctions with restricted diffusion in the distribution of middle cerebral arteries were represented in 35 % (n = 7) and with CVS, of them, 71.43 % (n = 5) had beading and focal narrowing of carotid arteries were consistent with vasculitis. Brain atrophy represented 20 % (n = 4) and with psychosis. Compared to those with normal MRI, patients with MRI abnormalities were older (P < 0.050) and had longer duration of illness (P < 0.050). To conclude, although DWI

  2. Cardiovascular Disease in Systemic Lupus Erythematosus: The Role of Traditional and Lupus Related Risk Factors

    PubMed Central

    Zeller, Carlos Borelli; Appenzeller, Simone

    2008-01-01

    Atherosclerosis is a chronic inflammatory disorder characterized by immune cell activation, inflammation driven plaque formation and subsequent destabilization. In other disorders of an inflammatory nature, the chronic inflammatory state per se has been linked to acceleration of the atherosclerotic process which is underlined by an increased incidence of cardiovascular disease (CVD) in disorders such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and antiphopholipid (Hughes) syndrome (APS). SLE is an autoimmune disease that may affect any organ. Premature coronary heart disease has emerged as a major cause of morbidity and mortality in SLE. In addition to mortality, cardiovascular morbidity is also markedly increased in these patients, compared with the general population. The increased cardiovascular risk can be explained only partially by an increased prevalence of classical risk factors for cardiovascular disease; it also appears to be related to inflammation. Inflammation is increasingly being considered central to the pathogenesis of atherosclerosis and an important risk factor for vascular disease. Recent epidemiologic and pathogenesis studies have suggested a great deal in common between the pathogenesis of prototypic autoimmune disease such as SLE and that of atherosclerosis. We will review traditional risk factors for CVD in SLE. We will also discuss the role of inflammation in atherosclerosis, as well as possible treatment strategies in these patients. PMID:19936286

  3. Serology of Lupus Erythematosus: Correlation between Immunopathological Features and Clinical Aspects

    PubMed Central

    Cozzani, Emanuele; Drosera, Massimo; Parodi, Aurora

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the aberrant production of a broad and heterogenous group of autoantibodies. Even though the presence of autoantibodies in SLE has been known, for more than 60 years, still nowadays a great effort is being made to understand the pathogenetic, diagnostic, and prognostic meaning of such autoantibodies. Antibodies to ds-DNA are useful for the diagnosis of SLE, to monitor the disease activity, and correlate with renal and central nervous involvements. Anti-Sm antibodies are highly specific for SLE. Anti-nucleosome antibodies are an excellent marker for SLE and good predictors of flares in quiescent lupus. Anti-histone antibodies characterize drug-induced lupus, while anti-SSA/Ro and anti-SSB/La antibodies are associated with neonatal lupus erythematosus and photosensitivity. Anti-ribosomal P antibodies play a role in neuropsychiatric lupus, but their association with clinical manifestations is still unclear. Anti-phospholipid antibodies are associated with the anti-phospholipid syndrome, cerebral vascular disease, and neuropsychiatric lupus. Anti-C1q antibodies amplify glomerular injury, and the elevation of their titers may predict renal flares. Anti-RNP antibodies are a marker of Sharp's syndrome but can be found in SLE as well. Anti-PCNA antibodies are present in 5–10% of SLE patients especially those with arthritis and hypocomplementemia. PMID:24649358

  4. Indices to assess patients with systemic lupus erythematosus in clinical trials, long-term observational studies, and clinical care.

    PubMed

    Castrejón, I; Tani, C; Jolly, M; Huang, A; Mosca, M

    2014-01-01

    This review summarises most currently used indices to assess and monitor patients with systemic lupus erythematosus (SLE) in clinical trials, long-term observational studies, and clinical care. Six SLE disease activity indices include the British Isles Lupus Assessment Group Index (BILAG), European Consensus Lupus Activity Measurement (ECLAM), Systemic Lupus Activity Measure (SLAM), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Lupus Activity Index (LAI), and Systemic Lupus Erythematosus Activity Questionnaire (SLAQ). Three SLE responder indices include Responder Index for Lupus Erythematosus (RIFLE), SLE Responder Index (SRI), and BILAG Based Combined Lupus Assessment (BICLA). Three SLE damage indices include the Systemic Lupus International Collaborating Clinics/American College of Rheumatology-Damage Index (SLICC/ACE-DI), Lupus Damage Index Questionnaire (LDIQ), and Brief Index of Lupus Damage (BILD). The SLAQ, LDIQ and the BILD are patient self-report questionnaires, which appear to give similar information to physician-completed indices, but are pragmatically more easily completed as patients do almost all the work. Additional self-report indices which have been used to assess and monitor patients with in SLE include a generic general health short form 36 (SF36), a SLE-specific Lupus Patient Reported Outcome (LupusPRO), and a generic rheumatology index, Routine Assessment of Patient Index Data 3 (RAPID3). These activity, response, damage and patient self-report indices have been validated at different levels with no consensus about what it is the most appropriate for every setting. Sensitive and feasible assessment of SLE in clinical trials, observational studies, and busy clinical settings remains a challenge to the rheumatology community.

  5. Current Perspectives on Arthroplasty in Systemic Lupus Erythematosus: Rates, Outcomes, and Adverse Events.

    PubMed

    Kasturi, Shanthini; Goodman, Susan

    2016-09-01

    Systemic lupus erythematosus (SLE) is a chronic debilitating condition with significant impact on the musculoskeletal system. Arthroplasty may be indicated for damage related to active lupus or its treatment. As therapies for SLE have advanced, morbidity and mortality have declined, while the rate of joint replacement has increased. The age of SLE patients undergoing arthroplasty is increasing, and the indication for surgery is evolving-while avascular necrosis was previously the predominant indication for arthroplasty, osteoarthritis now accounts for a larger proportion of surgeries. Pain and functional outcomes of arthroplasty in SLE patients are comparable to those of the general population with osteoarthritis, but lupus remains an independent risk factor for post-hip arthroplasty complications and mortality. Further research is needed to characterize the impact of lupus disease activity and severity on arthroplasty outcomes. PMID:27443850

  6. Sensitivity to Change and Minimal Important Differences of the LupusQoL in Patients With Systemic Lupus Erythematosus

    PubMed Central

    McElhone, Kathleen; Abbott, Janice; Sutton, Chris; Mullen, Montana; Lanyon, Peter; Rahman, Anisur; Yee, Chee‐Seng; Akil, Mohammed; Bruce, Ian N.; Ahmad, Yasmeen; Gordon, Caroline

    2016-01-01

    Objective As a health‐related quality of life (HRQOL) measure, the LupusQoL is a reliable and valid measure for adults with systemic lupus erythematosus (SLE). This study evaluates the responsiveness and minimal important differences (MIDs) for the 8 LupusQoL domains. Methods Patients experiencing a flare were recruited from 9 UK centers. At each of the 10 monthly visits, HRQOL (LupusQoL, Short Form 36 health survey [SF‐36]), global rating of change (GRC), and disease activity using the British Isles Lupus Assessment Group 2004 index were assessed. The responsiveness of the LupusQoL and the SF‐36 was evaluated primarily when patients reported an improvement or deterioration on the GRC scale and additionally with changes in physician‐reported disease activity. MIDs were estimated as mean changes when minimal change was reported on the GRC scale. Results A total of 101 patients were recruited. For all LupusQoL domains, mean HRQOL worsened when patients reported deterioration and improved when patients reported an improvement in GRC; SF‐36 domains showed comparable responsiveness. Improvement in some domains of the LupusQoL/SF‐36 was observed with a decrease in disease activity, but when disease activity worsened, there was no significant change. LupusQoL MID estimates for deterioration ranged from −2.4 to −8.7, and for improvement from 3.5 to 7.3; for the SF‐36, the same MID estimates were −2.0 to −11.1 and 2.8 to 10.9, respectively. Conclusion All LupusQoL domains are sensitive to change with patient‐reported deterioration or improvement in health status. For disease activity, some LupusQoL domains showed responsiveness when there was improvement but none for deterioration. LupusQoL items were derived from SLE patients and provide the advantage of disease‐specific domains, important to the patients, not captured by the SF‐36. PMID:26816223

  7. Critical peripheral ischemia precipitated by severe episode of Raynaud's phenomenon in a patient with aPL-positive systemic lupus erythematosus, upon high titer anti-RNP seroconversion.

    PubMed

    Levy, O; Maslakov, I; Vosco, S; Markov, A; Amit-Vazina, M; Tishler, M

    2015-03-01

    A 35-year-old female with long standing aPL-positive lupus without history of thromboembolic events, who has developed critical peripheral ischemia (CPI) is described. An episode of severe Raynaud's phenomenon rapidly progressed to an extensive digit-threatening ischemia, involving bilateral hands and feet. She was successfully treated with corticosteroids, anticoagulation, iloprost, sildenafil, and nifedipine. Her serological studies were remarkable for the emergence of high titer anti-RNP seroconversion and an increase in aPL titer, suggesting that these autoantibodies played a role in the pathogenesis of CPI. It is important to note that such observation should herald this potentially devastating complication of systemic lupus erythematosus.

  8. [In vitro fertilization and systemic lupus erythematosus or antiphospholipid syndrome: An update].

    PubMed

    Orquevaux, P; Masseau, A; Le Guern, V; Gayet, V; Vauthier, D; Boutin, D; Wechsler, B; Morel, N; Guettrot-Imbert, G; Pennaforte, J-L; Piette, J-C; Costedoat-Chalumeau, N

    2015-03-01

    Fertility is not impaired in systemic lupus erythematosus or antiphospholipid syndrome, but, similarly to the general population, these patients may undergo in vitro fertilization. This type of treatment increases the risk of lupus flare, thrombosis, and ovarian hyperstimulation syndrome. This review will focus on in vitro fertilization in systemic lupus erythematosus or antiphospholipid syndrome. Literature data are relatively scant with only 3 reported studies. The first one included 17 patients and 63 cycles of induction ovulation/in vitro fertilization leading to 25 % of lupus flare, no thrombosis, and 3 % of ovarian hyperstimulation syndrome. The second study included 10 patients and 40 cycles of in vitro fertilization showing 31 % of lupus flare, no thrombosis and no ovarian hyperstimulation syndrome. The last one included 34 patients and 83 procedures of in vitro fertilization leading to 8 % of flares, 5 % of thrombosis and no ovarian hyperstimulation syndrome. Interestingly, in this last study, half of the complications were explained by poor adherence to treatment. These data are reassuring but it is important to remember that in vitro fertilization should be scheduled and carefully supervised in the same way as the high-risk pregnancies occurring in these patients.

  9. Immunological aspects of biopsy-proven lupus nephritis in Bahraini patients with systemic lupus erythematosus.

    PubMed

    Farid, Eman M; Hassan, Adla B; Abalkhail, Ali A; El-Agroudy, Amgad E; Arrayed, Sameer Al-M; Al-Ghareeb, Sumaya M

    2013-11-01

    Lupus nephritis (LN) is a frequent and potentially serious complication of systemic lupus erythematosus (SLE) that may influence morbidity and mortality. Immunological investigations are aiding tools to the kidney biopsy findings in early diagnosis, in addition to monitoring the effect of therapy. The aim of the present study is to highlight the role of these investigations in a group of Bahraini patients and to determine whether there is any positive association between these findings and the outcome of LN. The current study is a retrospective case-control study of randomly selected 88 SLE patients, 44 with biopsy-proven LN and 44 without, acting as controls. All renal biopsies performed during the period from 1996 to 2012 were classified according to the World Health Organization classification. Immunological investigations analyzed are: Antinuclear antibodies (ANA), anti-ds DNA, anti-ENA, anti-cardiolipin antibodies (abs) and complement components C3, C4. Human leukocyte antigen (HLA) typing class II was performed on selected cases. All patients had positive ANA (100%). A significantly high frequency of anti-Smith abs among the non-LN group (43.18%) compared with the LN group (18.18%) was found (P <0.001). On the other hand, the anti-Ro/SSA abs in the non-LN group was also found at a statistically higher frequency (20.45%) compared with that in the LN group (4.54%) (P <0.01). Anti-ds-DNA abs were found to be higher in the LN group (84.09%) compared with the non-LN group (70.45%), but the difference was not statistically significant (P = 0.082). There was a positive association of ANA positivity and low C3 and or C4 in the studied group. In our study, 88.2% of the HLA typed patients had HLADR2, DR3 or both. In conclusion, in our Arabic Bahraini SLE patients, the presence of anti-Smith, anti-Ro/SSA and anti-RNP antibodies and the absence of anti-dsDNA antibodies are independent predictive markers for renal involvement. However, more prospective studies with a

  10. Protein-losing enteropathy associated with refractory systemic lupus erythematosus with a good response to rituximab.

    PubMed

    Sansinanea, Pierina; Carrica, Sebastián Augusto; Marcos, Josefina; García, Mercedes Argentina

    2016-01-01

    A case is presented of a protein-losing enteropathy (PLE) as the initial manifestation of systemic lupus erythematosus (SLE) in a 17 year-old female patient, who presented with ascites, edema and hypoalbuminemia. The diagnosis of SLE was based on the presence of: malar rash, oral ulcers, thrombocytopenia, antinuclear antibodies, IgM anticardiolipin antibody, and lupus anticoagulant. Renal and liver diseases were ruled out. The PLE diagnosis was confirmed with fecal alpha 1-antitrypsin clearance. The PLE was refractory to different lines of immunosuppressive agents like glucocorticoids, cyclophosphamide, azathioprine, and cyclosporine, showing a satisfactory and sustained response with rituximab, allowing steroid sparing and long term remission. PMID:25818375

  11. Review: Male systemic lupus erythematosus: a review of sex disparities in this disease.

    PubMed

    Lu, L-J; Wallace, D J; Ishimori, M L; Scofield, R H; Weisman, M H

    2010-02-01

    Although males with systemic lupus erythematosus (SLE) represent 4-22% of all SLE patients, it may not be appropriate that these cases should be subordinated to females with SLE in terms of most health-related issues. Over the past few decades, some distinctive features of male lupus have been observed with regard to genetic and environmental aspects of sex differences, clinical features, and outcome. In addition, recent insights into sex disparities in this disease have brought forth a few plausible and novel pathogenetic hypotheses. This review discusses these findings and sex disparities in SLE that appear to be especially noteworthy and pertinent to our understanding of male SLE.

  12. Current and novel therapeutics in the treatment of systemic lupus erythematosus.

    PubMed

    Yildirim-Toruner, Cagri; Diamond, Betty

    2011-02-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease with significant clinical heterogeneity. Recent advances in our understanding of the genetic, molecular, and cellular bases of autoimmune diseases and especially SLE have led to the application of novel and targeted treatments. Although many treatment modalities are effective in lupus-prone mice, the situation is more complex in human subjects. This article reviews the general approach to the therapy of SLE, focusing on current approved therapies and novel approaches that might be used in the future.

  13. Protein-losing enteropathy associated with refractory systemic lupus erythematosus with a good response to rituximab.

    PubMed

    Sansinanea, Pierina; Carrica, Sebastián Augusto; Marcos, Josefina; García, Mercedes Argentina

    2016-01-01

    A case is presented of a protein-losing enteropathy (PLE) as the initial manifestation of systemic lupus erythematosus (SLE) in a 17 year-old female patient, who presented with ascites, edema and hypoalbuminemia. The diagnosis of SLE was based on the presence of: malar rash, oral ulcers, thrombocytopenia, antinuclear antibodies, IgM anticardiolipin antibody, and lupus anticoagulant. Renal and liver diseases were ruled out. The PLE diagnosis was confirmed with fecal alpha 1-antitrypsin clearance. The PLE was refractory to different lines of immunosuppressive agents like glucocorticoids, cyclophosphamide, azathioprine, and cyclosporine, showing a satisfactory and sustained response with rituximab, allowing steroid sparing and long term remission.

  14. Carbon monoxide inhibits T cell activation in target organs during systemic lupus erythematosus

    PubMed Central

    Mackern-Oberti, J P; Obreque, J; Méndez, G P; Llanos, C; Kalergis, A M

    2015-01-01

    Systemic lupus erythematosus is characterized by the presence of circulating anti-nuclear antibodies (ANA) and systemic damage that includes nephritis, haematological manifestations and pulmonary compromise, among others. Although major progress has been made in elucidating the molecular mechanisms responsible for autoimmunity, current therapies for lupus have not improved considerably. Because the exposure of carbon monoxide (CO) has been shown to display beneficial immunoregulatory properties in different immune-mediated diseases, we investigated whether CO therapy improves lupus-related kidney injury in lupus mice. MRL-Faslpr lupus mice were exposed to CO and disease progression was evaluated. ANA, leucocyte-infiltrating populations in spleen, kidney and lung and kidney lesions, were measured. CO therapy significantly decreased the frequency of activated B220+ CD4− CD8− T cells in kidneys and lungs, as well as serum levels of ANA. Furthermore, we observed that CO therapy reduced kidney injury by decreasing proliferative glomerular damage and immune complexes deposition, decreased proinflammatory cytokine production and finally delayed the impairment of kidney function. CO exposure ameliorates kidney and lung leucocyte infiltration and delays kidney disease in MRL-Faslpr lupus mice. Our data support the notion that CO could be explored as a potential new therapy for lupus nephritis. PMID:26095291

  15. Carbon monoxide inhibits T cell activation in target organs during systemic lupus erythematosus.

    PubMed

    Mackern-Oberti, J P; Obreque, J; Méndez, G P; Llanos, C; Kalergis, A M

    2015-10-01

    Systemic lupus erythematosus is characterized by the presence of circulating anti-nuclear antibodies (ANA) and systemic damage that includes nephritis, haematological manifestations and pulmonary compromise, among others. Although major progress has been made in elucidating the molecular mechanisms responsible for autoimmunity, current therapies for lupus have not improved considerably. Because the exposure of carbon monoxide (CO) has been shown to display beneficial immunoregulatory properties in different immune-mediated diseases, we investigated whether CO therapy improves lupus-related kidney injury in lupus mice. MRL-Fas(lpr) lupus mice were exposed to CO and disease progression was evaluated. ANA, leucocyte-infiltrating populations in spleen, kidney and lung and kidney lesions, were measured. CO therapy significantly decreased the frequency of activated B220(+) CD4(-) CD8(-) T cells in kidneys and lungs, as well as serum levels of ANA. Furthermore, we observed that CO therapy reduced kidney injury by decreasing proliferative glomerular damage and immune complexes deposition, decreased proinflammatory cytokine production and finally delayed the impairment of kidney function. CO exposure ameliorates kidney and lung leucocyte infiltration and delays kidney disease in MRL-Fas(lpr) lupus mice. Our data support the notion that CO could be explored as a potential new therapy for lupus nephritis.

  16. Toxic Epidermal Necrolysis-Like Lesions and Systemic Lupus Erythematosus Possibly Triggered by Sulfasalazine

    PubMed Central

    Gül, Cigdem; Andersen, Bjarne; Tvede, Niels

    2016-01-01

    This case report describes a patient with arthritis of the large joints, bilateral sacroiliitis, and positive anti-SSA and anti-dsDNA antibody, who received sulfasalazine and shortly thereafter became critically ill. He developed toxic epidermal necrolysis, hemolytic anemia, lymphopenia, markedly elevated ferritin, and muscle wasting. A diagnosis of systemic lupus erythematosus was made, and mycophenolate mofetil and systemic glucocorticoids brought this severe disease under control. Toxic epidermal necrolysis-like lesions and hemophagocytic syndrome have been reported as manifestations of systemic lupus erythematosus. This patient possibly had spondyloarthritis or an undifferentiated connective tissue disease at presentation, and we suggest, based on the timing of events, that sulfasalazine may have acted as a trigger of the severe disease manifestations. PMID:27478675

  17. Prevalence of hyposalivation in patients with systemic lupus erythematosus in a brazilian subpopulation.

    PubMed

    Leite, Cristhiane Almeida; Galera, Marcial Francis; Espinosa, Mariano Martínez; de Lima, Paulo Ricardo Teles; Fernandes, Vander; Borges, Álvaro Henrique; Dias, Eliane Pedra

    2015-01-01

    Background. Systemic lupus erythematosus (SLE) is a chronic inflammatory, multisystem, and autoimmune disease. Objective. The aim of this study was to describe the prevalence of hyposalivation in SLE patients and evaluate factors associated. Methods. This is a cross-sectional study developed at the Cuiaba University General Hospital (UNIC-HGU), Mato Grosso, Brazil. The study population consisted of female SLE patients treated at this hospital from 06/2010 to 12/2012. Unstimulated salivary flow rates (SFRs) were measured. Descriptive and inferential analyses were performed in all cases using a significance level P < 0.05. Results. The results showed that 79% of patients with systemic lupus erythematosus suffered from hyposalivation and that the disease activity and age in years were the factors that resulted in statistically significant differences. Conclusion. The activity of the disease, age >27 years, and the drugs used were factors associated with hyposalivation, resulting in a statistically significant decrease in saliva production.

  18. B-cell-targeted therapies in systemic lupus erythematosus and ANCA-associated vasculitis: current progress.

    PubMed

    Md Yusof, Md Yuzaiful; Vital, Edward M J; Emery, Paul

    2013-08-01

    B cells play a central role in the pathogenesis of systemic lupus erythematosus and anti-neutrophil cytoplasmic antibody-associated vasculitis. There are various strategies for targeting B cells including depletion, inhibition of survival factors, activation and inhibition of co-stimulatory molecules. Controlled trials in systemic lupus erythematosus have shown positive results for belimumab, promising results for epratuzumab and negative results for rituximab. The failure of rituximab in controlled trials has been attributed to trial design, sample size and outcome measures rather than true inefficacy. In anti-neutrophil cytoplasmic antibody-associated vasculitis, rituximab is effective for remission induction and in relapsing disease. However, the optimal long-term re-treatment strategy remains to be determined. Over the next 5 years, evidence will be available regarding the clinical efficacy of these novel therapies, biomarkers and their long-term safety.

  19. Evaluation of epratuzumab as a biologic therapy in systemic lupus erythematosus.

    PubMed

    Rao, Vijay; Gordon, Caroline

    2014-01-01

    B cells play a key role in the pathogenesis of systemic lupus erythematosus. Some of the current biologic therapies target B cells or B-cell activating factors. Epratuzumab is a humanized monoclonal antibody, which targets CD22 on B cells. This review focuses on the safety and efficacy of epratuzumab in systemic lupus erythematosus based on the information from various published clinical trials and presentations at international meetings. Epratuzumab acts as a B-cell modulator through inhibition of B-cell receptor signaling. It has been shown to be efficacious in open-label and Phase I and Phase II randomized controlled trials. The drug has steroid-sparing properties and treatment is associated with significant improvements in Health Related Quality of Life and its safety profile is comparable to placebo.

  20. A Comprehensive Rehabilitation Approach in a Patient With Serious Neuropsychiatric Systemic Lupus Erythematosus

    PubMed Central

    2016-01-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system in patients with systemic lupus erythematosus (SLE). It is essential to specify the problems faced by patients with NPSLE because it causes diverse disabilities and impairs quality of life. After performing a comprehensive evaluation, tailored management should be provided for the patient's specific problems. We report here the case of a 30-year-old female with SLE who experienced serious neuropsychiatric symptoms cerebral infarction followed by posterior reversible encephalopathy syndrome and peripheral polyneuropathy. We systemically assessed the patient using the International Classification of Functioning, Disability and Health model as a clinical problem-solving tool and provided comprehensive rehabilitation by focusing on her problems. PMID:27606283

  1. A Comprehensive Rehabilitation Approach in a Patient With Serious Neuropsychiatric Systemic Lupus Erythematosus.

    PubMed

    Ko, Yong Jae; Lee, Yang Gyun; Park, Ji Woong; Ahn, Sung Ho; Kwak, Jin Myoung; Choi, Yoon-Hee

    2016-08-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system in patients with systemic lupus erythematosus (SLE). It is essential to specify the problems faced by patients with NPSLE because it causes diverse disabilities and impairs quality of life. After performing a comprehensive evaluation, tailored management should be provided for the patient's specific problems. We report here the case of a 30-year-old female with SLE who experienced serious neuropsychiatric symptoms cerebral infarction followed by posterior reversible encephalopathy syndrome and peripheral polyneuropathy. We systemically assessed the patient using the International Classification of Functioning, Disability and Health model as a clinical problem-solving tool and provided comprehensive rehabilitation by focusing on her problems. PMID:27606283

  2. Prevalence of Hyposalivation in Patients with Systemic Lupus Erythematosus in a Brazilian Subpopulation

    PubMed Central

    Leite, Cristhiane Almeida; Galera, Marcial Francis; Espinosa, Mariano Martínez; de Lima, Paulo Ricardo Teles; Fernandes, Vander; Borges, Álvaro Henrique; Dias, Eliane Pedra

    2015-01-01

    Background. Systemic lupus erythematosus (SLE) is a chronic inflammatory, multisystem, and autoimmune disease. Objective. The aim of this study was to describe the prevalence of hyposalivation in SLE patients and evaluate factors associated. Methods. This is a cross-sectional study developed at the Cuiaba University General Hospital (UNIC-HGU), Mato Grosso, Brazil. The study population consisted of female SLE patients treated at this hospital from 06/2010 to 12/2012. Unstimulated salivary flow rates (SFRs) were measured. Descriptive and inferential analyses were performed in all cases using a significance level P < 0.05. Results. The results showed that 79% of patients with systemic lupus erythematosus suffered from hyposalivation and that the disease activity and age in years were the factors that resulted in statistically significant differences. Conclusion. The activity of the disease, age >27 years, and the drugs used were factors associated with hyposalivation, resulting in a statistically significant decrease in saliva production. PMID:25649631

  3. Cutaneous horn arising from an area of discoid lupus erythematosus on the scalp.

    PubMed

    Fatani, Mohammad Ibrahim; Hussain, Waleed Mohd; Baltow, Badee; Alsharif, Sahar

    2014-04-03

    A cutaneous horn is a rare clinical condition characterised by a conical projection of hyperkeratotic epidermis. Cutaneous horns most commonly arise from sun-exposed skin in elderly men, but may arise from any part of the body at any age in men and women. When a cutaneous horn forms, it is important to determine the underlying cause. Various skin diseases may present with cutaneous horns including viral warts, actinic keratosis, keratoacanthoma, seborrhoeic keratosis, pyogenic granuloma, discoid lupus erythematosus, verruca vulgaris, Bowen's disease, basal cell carcinoma and squamous cell carcinoma. The underlying pathology is benign in 61.1% of cases, premalignant in 23.2% of cases and malignant in 15.7% of cases. We report a patient with a cutaneous horn arising from an area of discoid lupus erythematosus on the scalp.

  4. Mesial temporal lobe epilepsy as a neuropsychiatric syndrome of systemic lupus erythematosus.

    PubMed

    Toyota, Tomoko; Akamatsu, Naoki; Tanaka, Akihiro; Shouzaki, Taisaku; Tsuji, Sadatoshi; Saito, Kazuyoshi; Tanaka, Yoshiya

    2013-03-01

    In this study, we aimed to investigate the types of seizures and epilepsy associated with systemic lupus erythematosus (SLE). We searched the medical records at a tertiary referral center to identify a cohort of epilepsy patients with SLE who were treated between January 2000 and August 2011. We analyzed the clinical and immunologic profiles of these patients, their seizure and epilepsy classifications, electroencephalography (EEG) and magnetic resonance imaging (MRI) assessments, and the treatment administered for epilepsy and SLE. As the result, 17 patients with SLE and epilepsy were identified. Seven patients had mesial temporal lobe epilepsy (MTLE), eight had epilepsy secondary to stroke, and two had generalized epilepsy. Of the seven patients with MTLE, anteriotemporal spikes were noted in all patients with EEG, and MRI findings suggesting hippocampal sclerosis were noted in four patients. Clobazam and levetiracetam were effective in treating three patients, and one patient underwent amygdalohippocampectomy. In conclusion, MTLE may be a characteristic manifestation of neuropsychiatric syndrome of systemic lupus erythematosus.

  5. A Comprehensive Rehabilitation Approach in a Patient With Serious Neuropsychiatric Systemic Lupus Erythematosus

    PubMed Central

    2016-01-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) involves the central and peripheral nervous system in patients with systemic lupus erythematosus (SLE). It is essential to specify the problems faced by patients with NPSLE because it causes diverse disabilities and impairs quality of life. After performing a comprehensive evaluation, tailored management should be provided for the patient's specific problems. We report here the case of a 30-year-old female with SLE who experienced serious neuropsychiatric symptoms cerebral infarction followed by posterior reversible encephalopathy syndrome and peripheral polyneuropathy. We systemically assessed the patient using the International Classification of Functioning, Disability and Health model as a clinical problem-solving tool and provided comprehensive rehabilitation by focusing on her problems.

  6. Lupus - resources

    MedlinePlus

    Resources - lupus ... The following organizations are good resources for information on systemic lupus erythematosus : The Lupus Foundation of America -- www.lupus.org The National Institute of Arthritis and Musculoskeletal ...

  7. [Lipid profile in patients with systemic lupus erythematosus, with special focus on lipoprotein(a) in lupus nephritis].

    PubMed

    Kiss, Emese; Fazekas, Brigitta; Tarr, Tünde; Muszbek, László; Zeher, Margit; Szegedi, Gyula

    2004-02-01

    Systemic lupus erythematosus (SLE) is a multifactorial polysystemic autoimmune disorder. Although life expectance in SLE has been improved by adequate immune suppressive therapy, the importance of chronic renal failure has not been reduced. Among late complications of the disease accelerated atherosclerosis attempts increasing attention. Dyslipoproteinemia and increased concentration of lipoproteins are important risk factors of atherosclerotic cardiovascular complication in SLE. Serum lipid parameters of 50 patients with lupus were examined in the present work. Thirty patients had histologically proven lupus nephritis (LN+), while the other group did not have renal involvement (LN-). Serum triglyceride, total cholesterol, LDL-C and apolipoprotein B (apoB) concentrations were significantly higher in the lupus nephritis (LN+) group. On the other hand, HDL-C and apoAI levels were also elevated in patients with LN. As a consequence of that, LDL-C/HDL-C and the apoB/apoAI ratios did not differ between patients with or without kidney involvement. This concluded the authors to measure the concentration of lipoprotein (a) in SLE patients, as Lp(a) is known to be an independent risk factor of atherosclerosis. Results indicated a significantly increased Lp(a) concentration in patients with lupus nephritis as compared to the LN- group. All but 2 patients without kidney involvement had lower than 100 mg/L Lp(a) concentration, while 27% of patients with lupus nephritis has an Lp(a) level between 100-300 mg/L. Further more, Lp(a) concentration was higher than 300 mg/L in 13% of the LN+ group. In a good correlation of these observations patients with nephritis suffered more frequently from deep venous thrombosis and ischaemic heart disease. The frequencies of hypertension and non-insulin dependent diabetes mellitus were slightly elevated in patients with nephritis. Present results suggest the importance of elevated lipoprotein (a) concentration in patients with lupus nephritis

  8. Ocular Complications in Cutaneous Lupus Erythematosus: A Systematic Review with a Meta-Analysis of Reported Cases.

    PubMed

    Arrico, L; Abbouda, A; Abicca, I; Malagola, R

    2015-01-01

    Ocular complications associated with cutaneous lupus erythematosus (CLE) are less studied compared with those ones associated with systemic lupus erythematosus (SLE). The main ocular sites involved in patients affected by discoid lupus erythematosus (DLE) are eyelids followed by orbit and periorbit, the least being cornea. The most common complications are blepharitis usually affecting the lower lid and associated with some type of lid lesion such as plaque or erythematosus patches and madarosis. Few cases with LE profundus (LEP) and ocular complications are reported, but they are associated with orbital inflammatory syndrome and severe complications. The main treatment prescribed is hydroxychloroquine with a dose of 200 mg twice a day for 6 to 8 weeks. Corticosteroids are also used. Intervals between the correct diagnosis and the beginning of the ocular symptoms are commonly delayed. Ophthalmologist should be aware of the ocular manifestation of this autoimmune disease. PMID:26171240

  9. Disseminated Nocardia farcinica infection in a patient with systemic lupus erythematosus.

    PubMed

    Hara, Hiroyuki; Wakui, Fuminori; Ochiai, Toyoko

    2011-06-01

    Here, we describe a patient with disseminated systemic nocardiosis. He had a history of systemic lupus erythematosus and had received oral prednisolone for 7 months. Nocardia farcinica was isolated from the pus. There were neither clinical nor radiologic features of pulmonary nocardiosis. The patient was treated with oral trimethoprim/sulfamethoxazole, intravenous imipenem and surgical drainage with a good clinical response, and there has been no recurrence of the infection. PMID:21372184

  10. Systemic lupus erythematosus with membranous glomerulonephritis and transverse myelitis associated with anabolic steroid use.

    PubMed

    Radis, C D; Callis, K P

    1997-10-01

    This report describes a 29-year-old bodybuilder taking anabolic steroids who presented with urinary retention, arthralgias, and peripheral edema, subsequently developed acute lower-extremity paralysis, and was diagnosed as having transverse myelitis and membranous glomerulonephritis secondary to systemic lupus erythematosus (SLE). The association of anabolic steroid use and hyperprolactinemia, and their possible link to the development of SLE, are reviewed. PMID:9336429

  11. Characterization of the inflammatory infiltrate and expression of endothelial cell adhesion molecules in lupus erythematosus tumidus.

    PubMed

    Kuhn, Annegret; Sonntag, Monika; Lehmann, Percy; Megahed, Mosaad; Vestweber, Dietmar; Ruzicka, Thomas

    2002-03-01

    Lupus erythematosus tumidus (LET) is a disease with characteristic clinical and histopathologic features that has not always been considered a subset of cutaneous lupus erythematosus (CLE). Although LET was first mentioned in the literature in 1930, it has rarely been documented, and immunohistochemical studies have never been performed. The aim of the present study was to characterize the inflammatory infiltrate and to analyze the expression of endothelial cell adhesion molecules in skin specimens from patients with LET and to compare the results with those from patients with other variants of CLE, such as discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). Cryostat sections of lesional skin specimens from ten patients with LET demonstrated an infiltrate composed of more than 75% CD4+, CD8+, and HLA-DR+ cells. Interestingly, CD45RO+ cells, in contrast to CD45RA+ cells, were the prevailing inflammatory cell population. Compared with skin specimens from patients with DLE and SCLE, the mean expression of CD4+ and CD8+ cells was higher (but not significantly so) in LET, and no differences were observed with the other three antibodies. Furthermore, in contrast to controls, intercellular adhesion molecule-1, vascular adhesion molecule-1, E-selectin, and P-selectin showed the same expression pattern in skin specimens from patients with DLE, SCLE, and LET. In conclusion, the inflammatory infiltrate of LET primarily consists of CD4+/CD8+ lymphocytes. Furthermore, expression of endothelial cell adhesion molecules was equally upregulated in LET compared with the expression in DLE and SCLE, suggesting a similar immunopathomechanism of these subtypes of CLE. PMID:12071156

  12. Pneumatosis cystoides intestinalis in neuropsychiatric systemic lupus erythematosus with diabetes mellitus: case report and literature review.

    PubMed

    Shimojima, Yasuhiro; Ishii, Wataru; Matsuda, Masayuki; Tojo, Kana; Watanabe, Rie; Ikeda, Shu-Ichi

    2011-08-01

    We report a patient with neuropsychiatric systemic lupus erythematosus (NPSLE) complicated by diabetes mellitus (DM) who showed pneumatosis cystoides intestinalis (PCI) while being treated with prednisolone (PSL) and an alpha-glucosidase inhibitor (αGI). The PCI was ameliorated with the cessation of the αGI and tapering of PSL in addition to transient fasting. Multiple factors, including NPSLE, DM, and medications, may have been involved in the pathogenesis of PCI in this patient.

  13. Disseminated Cryptococcosis Presenting as Cutaneous Cellulitis in an Adolescent With Systemic Lupus Erythematosus.

    PubMed

    Valente, Ellen Simionato; Lazzarin, Mauricio Costa; Koech, Bruno Lopes; da Rosa, Ralph Vighi; de Almeida, Rafael; de Oliveira, Umberto Lopes; Neugebauer, Maria Gertrudes Fernandes Pereira; Sacco, Alexander Gonüalves

    2015-04-15

    We report here the case of a 17-year-old girl from Pelotas, Brazil, with systemic lupus erythematosus and disseminated cryptococcal infection. Prior to diagnosis, she was a chronic user of corticosteroids and other immunosuppressive drugs. Her first symptoms were skin lesions that simulated bacterial cellulitis. Upon suspicion, we performed a biopsy and fungal infection was confirmed. Appropriate therapy was established, and the patient was discharged after 42 days of treatment in complete remission.

  14. Disseminated Cryptococcosis Presenting as Cutaneous Cellulitis in an Adolescent With Systemic Lupus Erythematosus

    PubMed Central

    Valente, Ellen Simionato; Lazzarin, Mauricio Costa; Koech, Bruno Lopes; da Rosa, Ralph Vighi; de Almeida, Rafael; de Oliveira, Umberto Lopes; Neugebauer, Maria Gertrudes Fernandes Pereira; Sacco, Alexander Gonüalves

    2015-01-01

    We report here the case of a 17-year-old girl from Pelotas, Brazil, with systemic lupus erythematosus and disseminated cryptococcal infection. Prior to diagnosis, she was a chronic user of corticosteroids and other immunosuppressive drugs. Her first symptoms were skin lesions that simulated bacterial cellulitis. Upon suspicion, we performed a biopsy and fungal infection was confirmed. Appropriate therapy was established, and the patient was discharged after 42 days of treatment in complete remission. PMID:26294948

  15. Cat scratch disease in an immunosuppressed patient with systemic lupus erythematosus.

    PubMed

    Vargas-Hitos, J A; Sabio, J M; Navarrete-Navarrete, N; Arenas-Miras, M del M; Zamora-Pasadas, M; Jiménez-Alonso, J

    2016-03-01

    Cat scratch disease is an infectious disorder transmitted by cats that typically affects children and young adults. Immunosuppression is a well-known risk factor for the development of severe and atypical forms of the disease; hence it is under-diagnosed in patients with compromised immunity. We are reporting the first case of cat scratch disease, which presented as fever and fatigue, in a patient with systemic lupus erythematosus while receiving immunosuppressant therapy after a kidney transplant.

  16. Myelopathy in systemic lupus erythematosus: a case report and a review of the literature.

    PubMed

    Hamming, L; van der Meulen, R; Vergouwen, A; Siegert, C

    2015-07-01

    Myelopathy, a severe condition characterised by paraparesis, sensory deficits and sphincter dysfunction, is one of the neuropsychiatric manifestations that have been described in patients with systemic lupus erythematosus (SLE). SLE-associated myelopathy may confront clinicians with a challenging decision-making process due to the broad differential diagnosis, the lack of disease-specific findings, and the urgency to initiate immunosuppressive therapy early in the course of the disease to favourably affect outcome. PMID:26228194

  17. Catatonia in systemic lupus erythematosus: a case report and review of literature.

    PubMed

    Grover, S; Parakh, P; Sharma, A; Rao, P; Modi, M; Kumar, A

    2013-05-01

    Although, neuropsychiatric morbidity is quite high in patients with systemic lupus erythematosus (SLE), catatonia has been rarely reported. We report a case of a 22-year-old female who presented with catatonic symptoms at the time of relapse of SLE and have discussed the presentation in the context of existing literature with regard to phenomenology of catatonia, psychiatric co-morbidity and treatment of catatonia in patients with SLE.

  18. Systemic Lupus Erythematosus Presenting with Massive Ascites: A Case of Pseudo-Pseudo Meigs Syndrome

    PubMed Central

    Song, J.; Abrudescu-Opran, A.

    2016-01-01

    The case presented is consistent with the phenomenon known as Pseudo-Pseudo Meigs Syndrome (PPMS). In it, we describe a young woman with newly diagnosed Systemic Lupus Erythematosus presenting with ascites, pleural effusions, and an elevated CA-125 level. Although rare, and of uncertain etiology, PPMS is becoming increasingly recognized in the literature. It should be considered as a differential diagnosis in such patients, along with the search for malignancy. PMID:27366341

  19. Cheilitis granulomatosa associated with lupus erythematosus discoid and treated with methotrexate: report of a case.

    PubMed

    Nazzaro, Gianluca; Muratori, Simona; Carrera, Carlo Giovanni; Coggi, Antonella; Gianotti, Raffaele

    2015-01-01

    We present the rare case of a 47-year-old patient, suffering from cheilitis granulomatosa and lupus erythematosus discoid: this association is really exceptional because only once reported in English literature. In addition, the treatment of cheilitis granulomatosa is a challenge for the dermatologist: the gold standard, represented by steroids, is in fact designed as a short-time option. Our report confirms the good efficacy of methotrexate as a steroid-sparing agent. PMID:26312716

  20. Maternal systemic lupus erythematosus associated with fetal congenital heart block. A case report.

    PubMed

    Moore, P J

    1981-08-15

    Congenital heart block (CHB) is a rare cause of fetal bradycardia and, if misinterpreted as fetal distress, may lead to detrimental obstetric intervention. There is a definite association between systemic lupus erythematosus (SLE), as well as other connective tissue disorders in the mother and CHB in her offspring. A case of CHB in the child of a mother with asymptomatic SLE is reported and a useful diagnostic investigation, the sonar-atropine test, is described.

  1. Invasive aspergillosis associated with systemic lupus erythematosus and cardiac postoperative complication

    PubMed Central

    Macêdo, Danielle Patrícia Cerqueira; Silva-Júnior, Heraldo Maia; de Souza-Motta, Cristina Maria; Milan, Eveline Pípolo; Neves, Rejane Pereira

    2009-01-01

    Aspergillus is a ubiquitous fungus which can cause a variety of clinical syndromes. This fungus has emerged as agent of systemic infections and has therefore gained considerable public health importance. This paper describes two cases of invasive aspergillosis caused by A. fumigatus in immuno-suppressed patients and underscores the importance of early identification of Aspergillus infection associated with systemic lupus erythematosus and cardiac postoperative complications. PMID:24031340

  2. Blisters and Loss of Epidermis in Patients With Lupus Erythematosus: A Clinicopathological Study of 22 Patients.

    PubMed

    Merklen-Djafri, Carine; Bessis, Didier; Frances, Camille; Poulalhon, Nicolas; Debarbieux, Sébastien; Cordel, Nadège; Lipsker, Dan

    2015-11-01

    The nosology of bullous lesions or equivalents (vesicles, erosions, and crusts) in patients with lupus erythematosus (LE) is rarely addressed.The primary aim of this study was to draw up a precise phenotypic inventory of such skin lesions; the secondary objective was to assess a potential relationship between the different types of loss of epidermis and extracutaneous lupus manifestations.We conducted a retrospective multicenter study including 22 patients with definite LE and bullous lesions or equivalents. All biopsies were reviewed. Patients were recruited in the dermatology departments of 6 centers. Patients were included if they met the diagnosis of systemic LE according to American College of Rheumatology and/or Systemic Lupus International Collaborating Clinics criteria or diagnosis of cutaneous LE based on classic clinical criteria and/or histological ascertainment of LE. Patients were recruited through clinician's memory and photographic collections.Three clinico-pathological patterns could be individualized. First, toxic epidermal necrolysis (TEN)-like, sheet-like, skin detachment; sun-exposure, mild mucosal involvement, and dermal mucin deposition allow differential diagnosis with classical Lyell syndrome. Second, vesiculo-bullae and/or crusting occurring on typical lesions of subacute cutaneous lupus erythematosus or chronic cutaneous lupus erythematosus. Third, tense vesicles and/or blisters with an underlying neutrophilic dermatosis and a usual response to dapsone.A careful analysis of 22 LE patients with epidermal detachment reveals 2 main pathomechanisms: a classic LE interface dermatitis, which can be hyperacute and lead to TEN-like skin detachment; and a neutrophilic dermatosis, with tense vesicles and/or blisters, including classic bullous LE. PMID:26579826

  3. Factors associated with metabolic syndrome in patients with systemic lupus erythematosus from Puerto Rico.

    PubMed

    Negrón, A M; Molina, M J; Mayor, A M; Rodríguez, V E; Vilá, L M

    2008-04-01

    The aim of this study was to determine the factors associated with metabolic syndrome in patients with systemic lupus erythematosus from Puerto Rico. A total of 204 patients with systemic lupus erythematosus (per the American College of Rheumatology classification criteria) were evaluated. Metabolic syndrome was assessed using the American Heart Association and the National Heart, Lung, and Blood Institute classification. Socioeconomic-demographic parameters, health-related behaviours, clinical manifestations, autoantibodies, pharmacological treatments, disease activity (per the Systemic Lupus Activity Measure--Revised), and damage accrual (per the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index) were determined at study visit. Factors associated with metabolic syndrome were examined by univariable analyses and multivariable logistic regression models. A total of 196 (96.2%) were women. The mean age at study visit was 43.6 +/- 13.0 years, and the mean disease duration was 8.7 +/- 7.7 years. Seventy-eight patients (38.2%) had metabolic syndrome. In the multivariable analysis, age (odds ratio [OR] = 1.05; 95% confidence interval [CI] 1.02-1.09), government health insurance (OR = 2.06; 95% CI 1.07-4.22), exercise (OR = 0.33; 95% CI 0.14-0.92), thrombocytopenia (OR = 4.19; 95% CI 1.54-11.37), erythrocyte sedimentation rate (OR = 1.64; 95% CI 1.03-2.63), disease activity (OR = 1.14; 95% CI 1.00-1.30), and prednisone >10 mg/day (OR = 3.69; 95% CI 1.22-11.11) were associated with metabolic syndrome. In conclusion, older age, low socioeconomic status, lack of exercise, thrombocytopenia, increased erythrocyte sedimentation rate , higher disease activity, and prednisone >10 mg/day were independently associated with metabolic syndrome in patients with systemic lupus erythematosus from Puerto Rico.

  4. Methods for detecting lupus anticoagulants and their relation to thrombosis and miscarriage in patients with systemic lupus erythematosus.

    PubMed Central

    Ferro, D.; Saliola, M.; Quintarelli, C.; Valesini, G.; Basili, S.; Grandilli, A. M.; Bonavita, M. S.; Violi, F.

    1992-01-01

    AIMS: To examine the sensitivity and specificity to past thrombotic events of four different coagulation tests, which screen for lupus anticoagulant (LA), and of anticardiolipin antibodies in patients with systemic lupus erythematosus. METHODS: Fifty three consecutive patients with systemic lupus erythematosus were studied of whom three males and 21 females, aged 21-60 years, had a history of venous and arterial thrombosis, or miscarriage, or both. Activated partial thromboplastin time (aPTT), dilute Russell's viper venom time (dRVVT), kaolin clotting time (KCT), dilute aPTT and the circulating titre of anticardiolipin antibodies were investigated in the two groups of patients and in 20 healthy control subjects. RESULTS: The prolonged dilute aPTT was more sensitive to thromboses or miscarriages, or both than dRVVT (p less than 0.05), KCT (p less than 0.01), and aPTT (p less than 0.001). No significant differences in specificity were found among aPTT (100%), dRVVT (93%), KCT (93%) and dilute aPTT (86.2%); but aPTT and dRVVT were significantly more specific (p less than 0.01, p less than 0.05, respectively) than anticardiolipin antibodies. CONCLUSIONS: The study shows a strong association between lupus anticoagulant and thrombosis when a very sensitive test such as the dilute aPTT is used. The combination of this assay with a very specific test such as dRVVT might enable patients with SLE at high risk of thrombosis to be identified. PMID:1577971

  5. Magnetic Resonance Imaging And Brain Histopathology In Neuropsychiatric Systemic Lupus Erythematosus

    PubMed Central

    Sibbitt, Wilmer L.; Brooks, William M.; Kornfeld, Mario; Hart, Blaine L.; Bankhurst, Arthur D.; Roldan, Carlos A.

    2013-01-01

    Objective Magnetic resonance imaging (MRI) often demonstrates brain lesions in neuropsychiatric systemic lupus erythematosus (NPSL). The present study compared post-mortem histopathology with pre-mortem MRI in NPSL. Methods 200 subjects with NPSLE were studied prospectively with MRI over a 10-year period during which 22 subjects died. In 14 subjects, a brain autopsy with histopathology that permitted direct comparison with pre mortem MRI was successfully obtained. Surface anatomy was used to determine the approximate location of individual lesions. Results Pre mortem MRI findings in fatal NPSLE were small focal white matter lesions (100%), cortical atrophy (64%), ventricular dilation (57%), cerebral edema (50%), diffuse white matter abnormalities (43%), focal atrophy (36%), cerebral infarction (29%), acute leukoencephalopathy (25%), intracranial hemorrhage (21%), and calcifications (7%). Microscopic findings in fatal NPSLE included global ischemic changes (57%), parenchymal edema (50%), microhemorrhages (43%), glial hyperplasia (43%), diffuse neuronal/axonal loss (36%), resolved cerebral infarction (33%), microthomboemboli (29%), blood vessel remodeling (29%), acute cerebral infarction (14%), acute macrohemorrhages (14%), and resolved intracranial hemorrhages (7%). Cortical atrophy and ventricular dilation seen by MRI predicted brain mass at autopsy (r = -0.72, p = 0.01, and r = -0.77, p =0.01, respectively). Cerebral autopsy findings, including infarction, cerebral edema, intracranial hemorrhage, calcifications, cysts, and focal atrophy were also predicted accurately by pre mortem MRI. Conclusion Brain lesions in NPSLE detected by MRI accurately represent serious underlying cerebrovascular and parenchymal brain injury on pathology. PMID:19880162

  6. Pregnancy Outcomes in Chinese Patients with Systemic Lupus Erythematosus (SLE): A Retrospective Study of 109 Pregnancies.

    PubMed

    Ku, Ming; Guo, Shuiming; Shang, Weifeng; Li, Qing; Zeng, Rui; Han, Min; Ge, Shuwang; Xu, Gang

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients' characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records. After exclusion of elective abortions, the live birth rate was 61.5%. Significantly, APS (antiphospholipid syndrome), disease activity, hypertension, hypocomplementemia, thrombocytopenia, and anemia during pregnancy were more commonly observed in fetal loss pregnancies than in live birth pregnancies. Compared to the 64 women with a history of SLE, 19 women with new-onset lupus during pregnancy had worse pregnancy outcome. Furthermore, the 64 patients with a history of SLE were divided into lupus nephritis group and SLE group (non-renal involvement). We found that the lupus nephritis group had worse maternal outcome than the SLE group. We conclude that new-onset lupus during pregnancy predicts both adverse maternal and fetal outcomes, while a history of lupus nephritis predicts adverse maternal outcomes. It is essential to provide SLE women with progestational counseling and regular multispecialty care during pregnancy. PMID:27442513

  7. Pregnancy Outcomes in Chinese Patients with Systemic Lupus Erythematosus (SLE): A Retrospective Study of 109 Pregnancies

    PubMed Central

    Ku, Ming; Guo, Shuiming; Shang, Weifeng; Li, Qing; Zeng, Rui; Han, Min; Ge, Shuwang; Xu, Gang

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients’ characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records. After exclusion of elective abortions, the live birth rate was 61.5%. Significantly, APS (antiphospholipid syndrome), disease activity, hypertension, hypocomplementemia, thrombocytopenia, and anemia during pregnancy were more commonly observed in fetal loss pregnancies than in live birth pregnancies. Compared to the 64 women with a history of SLE, 19 women with new-onset lupus during pregnancy had worse pregnancy outcome. Furthermore, the 64 patients with a history of SLE were divided into lupus nephritis group and SLE group (non-renal involvement). We found that the lupus nephritis group had worse maternal outcome than the SLE group. We conclude that new-onset lupus during pregnancy predicts both adverse maternal and fetal outcomes, while a history of lupus nephritis predicts adverse maternal outcomes. It is essential to provide SLE women with progestational counseling and regular multispecialty care during pregnancy. PMID:27442513

  8. Apoptotic Debris Accumulates on Hematopoietic Cells and Promotes Disease in Murine and Human Systemic Lupus Erythematosus.

    PubMed

    Kang, SunAh; Rogers, Jennifer L; Monteith, Andrew J; Jiang, Chuancang; Schmitz, John; Clarke, Stephen H; Tarrant, Teresa K; Truong, Young K; Diaz, Marilyn; Fedoriw, Yuri; Vilen, Barbara J

    2016-05-15

    Apoptotic debris, autoantibody, and IgG-immune complexes (ICs) have long been implicated in the inflammation associated with systemic lupus erythematosus (SLE); however, it remains unclear whether they initiate immune-mediated events that promote disease. In this study, we show that PBMCs from SLE patients experiencing active disease, and hematopoietic cells from lupus-prone MRL/lpr and NZM2410 mice accumulate markedly elevated levels of surface-bound nuclear self-antigens. On dendritic cells (DCs) and macrophages (MFs), the self-antigens are part of IgG-ICs that promote FcγRI-mediated signal transduction. Accumulation of IgG-ICs is evident on ex vivo myeloid cells from MRL/lpr mice by 10 wk of age and steadily increases prior to lupus nephritis. IgG and FcγRI play a critical role in disease pathology. Passive transfer of pathogenic IgG into IgG-deficient MRL/lpr mice promotes the accumulation of IgG-ICs prior to significant B cell expansion, BAFF secretion, and lupus nephritis. In contrast, diminishing the burden IgG-ICs in MRL/lpr mice through deficiency in FcγRI markedly improves these lupus pathologies. Taken together, our findings reveal a previously unappreciated role for the cell surface accumulation of IgG-ICs in human and murine lupus. PMID:27059595

  9. Immune dysregulation accelerates atherosclerosis and modulates plaque composition in systemic lupus erythematosus

    PubMed Central

    Stanic, Aleksandar K.; Stein, Charles M.; Morgan, Adam C.; Fazio, Sergio; Linton, MacRae F.; Wakeland, Edward K.; Olsen, Nancy J.; Major, Amy S.

    2006-01-01

    Patients with systemic lupus erythematosus (SLE) have accelerated atherosclerosis. The underlying mechanisms are poorly understood, and investigations have been hampered by the absence of animal models that reflect the human condition of generalized atherosclerosis and lupus. We addressed this problem by transferring lupus susceptibility to low-density lipoprotein (LDL) receptor-deficient (LDLr−/−) mice, an established model of atherosclerosis, creating radiation chimeras with NZM2410-derived, lupus-susceptible, B6.Sle1.2.3 congenic or C57BL/6 control donors (LDLr.Sle and LDLr.B6, respectively). LDLr.Sle mice developed a lupus-like disease characterized by production of double-stranded DNA autoantibodies and renal disease. When fed a Western-type diet, LDLr.Sle chimeras had increased mortality and atherosclerotic lesions. The plaques of LDLr.Sle mice were highly inflammatory and contained more CD3+ T cells than controls. LDLr.Sle mice also had increased activation of CD4+ T and B cells and significantly higher antibody to oxidized LDL and cardiolipin. Collectively, these studies demonstrate that the lupus-susceptible immune system enhances atherogenesis and modulates plaque composition. PMID:16636270

  10. Inhibition of SHP2 ameliorates the pathogenesis of systemic lupus erythematosus

    PubMed Central

    Wang, Jianxun; Zeng, Li-Fan; Bronson, Roderick; Finnell, Michele; Terhorst, Cox; Kyttaris, Vasileios C.; Zhang, Zhong-Yin; Kontaridis, Maria I.

    2016-01-01

    Systemic lupus erythematosus (SLE) is a devastating multisystemic autoimmune disorder. However, the molecular mechanisms underlying its pathogenesis remain elusive. Some patients with Noonan syndrome, a congenital disorder predominantly caused by gain-of-function mutations in the protein tyrosine phosphatase SH2 domain–containing PTP (SHP2), have been shown to develop SLE, suggesting a functional correlation between phosphatase activity and systemic autoimmunity. To test this directly, we measured SHP2 activity in spleen lysates isolated from lupus-prone MRL/lpr mice and found it was markedly increased compared with that in control mice. Similar increases in SHP2 activity were seen in peripheral blood mononuclear cells isolated from lupus patients relative to healthy patients. To determine whether SHP2 alters autoimmunity and related immunopathology, we treated MRL/lpr mice with an SHP2 inhibitor and found increased life span, suppressed crescentic glomerulonephritis, reduced spleen size, and diminished skin lesions. SHP2 inhibition also reduced numbers of double-negative T cells, normalized ERK/MAPK signaling, and decreased production of IFN-γ and IL-17A/F, 2 cytokines involved in SLE-associated organ damage. Moreover, in cultured human lupus T cells, SHP2 inhibition reduced proliferation and decreased production of IFN-γ and IL-17A/F, further implicating SHP2 in lupus-associated immunopathology. Taken together, these data identify SHP2 as a critical regulator of SLE pathogenesis and suggest targeting of its activity as a potent treatment for lupus patients. PMID:27183387

  11. Premature vascular damage in systemic lupus erythematosus: an imbalance of damage and repair?

    PubMed

    Kaplan, Mariana J

    2009-08-01

    Systemic lupus erythematosus (SLE) is associated with an increase in the risk of premature cardiovascular complications caused by accelerated atherosclerosis, which significantly contributes to morbidity and mortality. Standard Framingham risk factors seem to be less important predictors of cardiovascular events than the presence of active SLE, and the immune dysregulation characteristic of lupus seems to play a dominant role in atherogenesis. Although both SLE-specific and nonspecific mechanisms have been proposed to play a prominent role in the induction of premature vascular damage in this disease, the exact etiology remains unclear. This review summarizes some of the proposed mechanisms that may promote accelerated vascular damage in lupus and explores potential targets for cardiovascular risk prevention in this patient population.

  12. Rituximab in systemic lupus erythematosus: an updated systematic review and meta-analysis.

    PubMed

    Duxbury, B; Combescure, C; Chizzolini, C

    2013-12-01

    The wide spectrum of clinical manifestations and high relapse rate represent a therapeutic challenge in systemic lupus erythematosus (SLE). Observational studies suggested efficacy of rituximab (RTX), a B-cell-targeting antibody, to control the activity of SLE. Two randomized trials controlled by placebo did not prove the superiority of RTX when used in addition to conventional treatment in nonrenal (EXPLORER) and renal (LUNAR) lupus. A systematic review of studies exploring the efficacy of RTX in SLE patients was conducted. The pooled percentages of response were assessed. Thirty studies with 1243 patients were analyzed. In studies using the British Isles Lupus Assessment Group (BILAG), the complete response (CR) rate was 46.7% (95% CI 36.8%-56.8%) and the partial response (PR) was 37.9% (95% CI 30.6%-45.8%). With the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), the CR was 56.6% (95% CI 32.4%-78.1%) and the PR was 30.9% (95% CI 8.9%-46%). In renal lupus the CR was 36.1% (95% CI 25.2%-48.6%); PR was 37.4% (95% CI 28.5%-47.3%). In EXPLORER, CR was 12.4% and PR was 17.2%; in LUNAR CR was 26.4% and PR was 30.6%, in both cases not different from controls. Assessment and standardization of SLE response to treatment remain a challenge. The discrepancy in the perceived efficacy of RTX between controlled and observational studies reflects the heterogeneity of lupus and stringency in criteria of response. Further randomized trials focusing on selected SLE manifestations and using composite response indices are warranted.

  13. B-cell-targeted therapy for systemic lupus erythematosus.

    PubMed

    Sabahi, Ramin; Anolik, Jennifer H

    2006-01-01

    Systemic lupus erythematosus (SLE) is a complex disease characterised by numerous autoantibodies and clinical involvement in multiple organ systems. The immunological events triggering the onset of clinical manifestations have not yet been fully defined, but a central role for B cells in the pathogenesis of this disease has more recently gained prominence as a result of research in both mice and humans. Both antibody-dependent and -independent mechanisms of B cells are important in SLE. Autoantibodies contribute to autoimmunity by multiple mechanisms, including immune complex-mediated type III hypersensitivity reactions, type II antibody-dependent cytotoxicity, and by instructing innate immune cells to produce pathogenic cytokines such as interferon-alpha, tumour necrosis factor and interleukin-1. Suggested autoantibody-independent B-cell functions include antigen presentation, T-cell activation and polarisation, and dendritic-cell modulation. Several of these functions are mediated by the ability of B cells to produce immunoregulatory cytokines, chemokines and lymphangiogenic growth factors, and by their critical contribution to lymphoid tissue development and organisation, including the development of ectopic tertiary lymphoid tissue. Given the large body of evidence implicating abnormalities in the B-cell compartment in SLE, a recent therapeutic focus has been to develop interventions that target the B-cell compartment by multiple mechanisms.Rituximab, a mouse-human chimeric monoclonal antibody against CD20 that specifically depletes B cells, has been studied the most extensively. Although promising open-label data await confirmation in ongoing multicentre placebo-controlled trials, a number of preliminary conclusions can be drawn. The adequacy of peripheral B-cell depletion depends on achieving high and sustained serum rituximab concentrations, pharmacokinetics that can be varied with treatment dose and factors that may affect drug clearance, such as human anti

  14. Systemic lupus erythematosus in three ethnic groups. XII. Risk factors for lupus nephritis after diagnosis.

    PubMed

    Bastian, H M; Roseman, J M; McGwin, G; Alarcón, G S; Friedman, A W; Fessler, B J; Baethge, B A; Reveille, J D

    2002-01-01

    The purpose of this study was to determine the cumulative incidence of lupus nephritis (LN) and the factors predictive of its occurrence in a multiethnic systemic lupus erythematosus (SLE) cohort. We studied 353 SLE patients as defined by the American College of Rheumatology (ACR) criteria (65 Hispanics, 93 African-Americans and 91 Caucasians). First, we determined the cumulative incidence of LN in all patients. Next, we determined the predictors for LN in those with nephritis occurring after diagnosis. The dependent variable, LN, was defined by: (1) A renal biopsy demonstrating World Health Organization (WHO), class II-V histopathology; and/or (2) proteinuria > or = 0.5 g/24 h or 3+ proteinuria attributable to SLE; and/or (3) one of the following features also attributable to SLE and present on two or more visits, which were performed at least 6 months apart--proteinuria > or = 2+, serum creatinine > or = 1.4 mg/dl, creatinine clearance < or = 79 ml/min, > or = 10 RBCs or WBCs per high power field (hpf), or > or = 3 granular or cellular casts per hpf. Independent variables assessed at diagnosis, and if absent, at baseline, were from four domains: sociodemographic, clinical, immunologic and immunogenetic (including the complete antibody profile and MHC class II alleles), and health habits. Variables with P < 0.05 by chi square analyses were entered into domain-specific stepwise logistic regression analyses controlling for disease duration, with LN as the dependent variable. Significant domain-specific regression variables (P < or = 0.1) were then entered into an overall model. The cumulative incidence of LN was 54.3% in all patients, and 35.3% for those developing LN after diagnosis. LN after diagnosis occurred in 43.1% of 65 Hispanics, 50.5% of 93 African-Americans, and 14.3% of 91 Caucasians, P < 0.0001. The duration of follow-up for those with LN after diagnosis was 5.5+/-2.4 vs 4.0+/-2.9 years for those without LN. Hispanic (odds ratio (OR) = 2.71, 95

  15. Systemic lupus erythematosus in three ethnic groups. XII. Risk factors for lupus nephritis after diagnosis.

    PubMed

    Bastian, H M; Roseman, J M; McGwin, G; Alarcón, G S; Friedman, A W; Fessler, B J; Baethge, B A; Reveille, J D

    2002-01-01

    The purpose of this study was to determine the cumulative incidence of lupus nephritis (LN) and the factors predictive of its occurrence in a multiethnic systemic lupus erythematosus (SLE) cohort. We studied 353 SLE patients as defined by the American College of Rheumatology (ACR) criteria (65 Hispanics, 93 African-Americans and 91 Caucasians). First, we determined the cumulative incidence of LN in all patients. Next, we determined the predictors for LN in those with nephritis occurring after diagnosis. The dependent variable, LN, was defined by: (1) A renal biopsy demonstrating World Health Organization (WHO), class II-V histopathology; and/or (2) proteinuria > or = 0.5 g/24 h or 3+ proteinuria attributable to SLE; and/or (3) one of the following features also attributable to SLE and present on two or more visits, which were performed at least 6 months apart--proteinuria > or = 2+, serum creatinine > or = 1.4 mg/dl, creatinine clearance < or = 79 ml/min, > or = 10 RBCs or WBCs per high power field (hpf), or > or = 3 granular or cellular casts per hpf. Independent variables assessed at diagnosis, and if absent, at baseline, were from four domains: sociodemographic, clinical, immunologic and immunogenetic (including the complete antibody profile and MHC class II alleles), and health habits. Variables with P < 0.05 by chi square analyses were entered into domain-specific stepwise logistic regression analyses controlling for disease duration, with LN as the dependent variable. Significant domain-specific regression variables (P < or = 0.1) were then entered into an overall model. The cumulative incidence of LN was 54.3% in all patients, and 35.3% for those developing LN after diagnosis. LN after diagnosis occurred in 43.1% of 65 Hispanics, 50.5% of 93 African-Americans, and 14.3% of 91 Caucasians, P < 0.0001. The duration of follow-up for those with LN after diagnosis was 5.5+/-2.4 vs 4.0+/-2.9 years for those without LN. Hispanic (odds ratio (OR) = 2.71, 95

  16. Patient-reported outcome measures in a population of medically indigent patients with systemic lupus erythematosus in Puerto Rico

    PubMed Central

    Rodríguez-Rivera, Diana V; Rodríguez-Navedo, Yerania; Nieves-Plaza, Mariely; Vilá, Luis M

    2016-01-01

    Objective: To determine patient-reported outcomes measures in indigent patients with systemic lupus erythematosus receiving their healthcare through the Puerto Rico government managed care system and compare these measures with non-indigent patients treated in a private fee-for-service setting. Methods: A cross-sectional study was conducted in a cohort of 98 Puerto Ricans with systemic lupus erythematosus. Patients from the public group (n = 40) were treated in a university-based specialized systemic lupus erythematosus clinic and the private group (n = 58) in a community-based rheumatology practice. Demographic and clinical features and patient-reported outcomes measures per LupusPRO instrument were determined. LupusPRO captures quality-of-life measures in 12 domains. Differences among study groups were examined using chi-square, Fisher’s exact, t-tests, and the Wilcoxon signed-rank test. Results: The mean (standard deviation) age of the study population was 44.9 (12.0) years; 94 (95.9%) were women. Patients in the public setting were younger and were more likely to have renal disease and elevated anti-double-stranded DNA antibodies, and being treated with azathioprine and cyclophosphamide. Patients from the public sector were more likely to have better quality-of-life measures in the LupusPRO domains of pain/vitality and coping. No significant differences were observed for the domains of lupus symptoms, physical health, emotional health, body image, cognition, procreation, lupus medications, desires/goals, social support, and satisfaction with medical care. Conclusion: Despite having a lower socioeconomic status and worse clinical status, systemic lupus erythematosus patients from the public sector had equal or better patient-reported outcomes measures than those treated in the private setting. This favorable outcome may be associated with the comprehensive healthcare received by these patients in a specialized lupus clinic. PMID:27721978

  17. Three cases of spondyloenchondrodysplasia (SPENCD) with systemic lupus erythematosus: a case series and review of the literature.

    PubMed

    Bilginer, Y; Düzova, A; Topaloğlu, R; Batu, E D; Boduroğlu, K; Güçer, Ş; Bodur, I; Alanay, Y

    2016-06-01

    Spondyloenchondrodysplasia (SPENCD) is a rare autosomal recessive skeletal dysplasia caused by recessive mutations in the ACP5 gene, and it is characterized by the persistence of chondroid tissue islands within the bone. The clinical spectrum of SPENCD includes neurological involvement and immune dysfunction, such as systemic lupus erythematosus (SLE). To date, there are only 12 reported cases of SPENCD associated with SLE in the literature; however, detailed clinical follow-up data is absent for this comorbidity. This report presents clinical and laboratory data of three patients diagnosed with SPENCD-associated SLE. All three patients had short stature, arthralgia/arthritis, lupus nephritis, hypocomplementemia, and positive autoantibodies, including anti-nuclear and anti-dsDNA antibodies. Two patients exhibited class IV and one patient exhibited class V lupus nephritis. The early recognition of SPENCD is imperative, and this condition should be considered in patients with SLE, particularly in individuals with short stature and skeletal abnormalities. The cases presented here demonstrate that timely diagnosis and follow-up are key factors for the successful management of these conditions.

  18. Three cases of spondyloenchondrodysplasia (SPENCD) with systemic lupus erythematosus: a case series and review of the literature.

    PubMed

    Bilginer, Y; Düzova, A; Topaloğlu, R; Batu, E D; Boduroğlu, K; Güçer, Ş; Bodur, I; Alanay, Y

    2016-06-01

    Spondyloenchondrodysplasia (SPENCD) is a rare autosomal recessive skeletal dysplasia caused by recessive mutations in the ACP5 gene, and it is characterized by the persistence of chondroid tissue islands within the bone. The clinical spectrum of SPENCD includes neurological involvement and immune dysfunction, such as systemic lupus erythematosus (SLE). To date, there are only 12 reported cases of SPENCD associated with SLE in the literature; however, detailed clinical follow-up data is absent for this comorbidity. This report presents clinical and laboratory data of three patients diagnosed with SPENCD-associated SLE. All three patients had short stature, arthralgia/arthritis, lupus nephritis, hypocomplementemia, and positive autoantibodies, including anti-nuclear and anti-dsDNA antibodies. Two patients exhibited class IV and one patient exhibited class V lupus nephritis. The early recognition of SPENCD is imperative, and this condition should be considered in patients with SLE, particularly in individuals with short stature and skeletal abnormalities. The cases presented here demonstrate that timely diagnosis and follow-up are key factors for the successful management of these conditions. PMID:26854080

  19. The economic burden of systemic lupus erythematosus in Asia: the current state.

    PubMed

    Mak, A

    2010-10-01

    Systemic lupus erythematosus (SLE) is a chronic systemic inflammatory disorder which imposes considerable negative impact on patients' function and quality of life, and it appears to entail substantial loss of work productivity and healthcare cost. Although much has been studied regarding the epidemiology, pathogenesis, disease activity, disease damage and pharmacological treatment of SLE, publications on the economic burden of lupus are scarce. As the majority of lupus patients are residing in the Asia Pacific region where many are financially and socially deprived, and, from what we know from the current literature, work disability of lupus patients in Asia is substantial, cost-of-illness studies on SLE are thus particularly relevant in countries around the region. Reliable data from properly conducted prospective SLE cost studies are imperative for policymakers to efficiently distribute healthcare resources, especially in Asia where limited resources are unable to cope with the huge population. In this paper, we review the current state of cost-of-illness research on lupus in Asia and analyze the reasons why such studies are urgently required in the Asia Pacific region.

  20. Genetically Determined Amerindian Ancestry Correlates with Increased Frequency of Risk Alleles for Systemic Lupus Erythematosus

    PubMed Central

    Sanchez, E; Webb, R; Rasmussen, A.; Kelly, J.A; Riba, L.; Kaufman, K.M.; Garcia-de la Torre, I.; Moctezuma, J.F.; Maradiaga-Ceceña, M.A.; Cardiel, M.; Acevedo, E.; Cucho-Venegas, M.; Garcia, M.A.; Gamron, S.; Pons-Estel, B.A.; Vasconcelos, C.; Martin, J.; Tusié-Luna, T.; Harley, J.B.; Richardson, B.; Sawalha, A.H.; Alarcón-Riquelme, M.E.

    2011-01-01

    Objectives To analyze if genetically determined Amerindian ancestry predicts the increased presence of risk alleles of known susceptibility genes for systemic lupus erythematosus. Methods Single nucleotide polymorphisms within 16 confirmed genetic susceptibility loci for SLE were genotyped in a set of 804 Mestizo lupus patients and 667 Mestizo normal healthy controls. In addition, 347 admixture informative markers were genotyped. Individual ancestry proportions were determined using STRUCTURE. Association analysis was performed using PLINK, and correlation of the presence of risk alleles with ancestry was done using linear regression. Results A meta-analysis of the genetic association of the 16 SNPs across populations showed that TNFSF4, STAT4, PDCD1, ITGAM, and IRF5 were associated with lupus in a Hispanic-Mestizo cohort enriched for European and Amerindian ancestry. In addition, two SNPs within the MHC region, previously associated in a genome-wide association study in Europeans, were also associated in Mestizos. Using linear regression we predict an average increase of 2.34 risk alleles when comparing a lupus patient with 100% Amerindian ancestry to an SLE patient with 0% American Indian Ancestry (p<0.0001). SLE patients with 43% more Amerindian ancestry are predicted to carry one additional risk allele. Conclusion Amerindian ancestry increased the number of risk alleles for lupus. PMID:20848568

  1. [Systemic lupus erythematosus and the central nervous system].

    PubMed

    Rojas, E; Orrea Solano, M

    1993-01-01

    The central nervous system (CNS) manifestations of the chronic autoimmune disease systemic lupus erythematous (SLE) are reviewed. SLE-CNS dysfunction is broadly divided into neurologic and psychiatric clinical categories. The distinct clinical entities within these broad categories are fully described. Diagnostic criteria employed to verify the presence of SLE-CNS dysfunction, including laboratory serum and cerebral spinal fluid analyses as well as radiologic and other multimodality diagnostic tools, are compared and contrasted with respect to sensitivity and specificity.

  2. An uncommon presentation of an uncommon disease: relapsing polychondritis overlap with systemic lupus erythematosus.

    PubMed

    Nguyen, Michelle A; Rahnama-Moghadam, Sahand; Gilson, Robert T

    2016-01-01

    Relapsing polychondritis (RP) is a rare rheumatologic disorder in which recurrent episodes of inflammation result in destruction of cartilage of the ears and nose. The joints, eyes, audio-vestibular system, heart valves, respiratory tract, kidneys, and skin can also be involved. Skin involvement is most frequently linked to concomitant myelodysplastic syndrome and has rarely been associated with systemic lupus erythematosus. A 47-year-old woman presented with violaceous, indurated, tender plaques on the bilateral cartilaginous ears with sparing of the lobes, consistent with RP. Further investigations revealed positive ANA and anti-Smith antibody, oral ulcers, a photo-distributed skin eruption, and biopsy-proven lupus nephritis, leading to a second concomitant diagnosis of systemic lupus erythematosus (SLE). The diagnosis of SLE associated with RP was made and the patient was started on oral prednisone and hydroxychloroquine. This is a rare report of SLE associated with RP. It is unclear whether RP occurring in patients with SLE represents another clinical manifestation of SLE or a coexisting disease. However, a significant ANA titer in a patient with RP strongly suggests the presence of an associated autoimmune disorder. If immunologic abnormalities usually found in SLE are detected in patients with RP, it is important to monitor patients for the development of other manifestations of SLE. PMID:27267190

  3. Subacute lupus erythematosus during treatment with golimumab for seronegative rheumatoid arthritis.

    PubMed

    Brunasso, A M G; Aberer, W; Massone, C

    2014-02-01

    We report on a 52-year-old woman with a history of severe seronegative rheumatoid arthritis. Several conventional therapies and biological therapy with etanercept and infliximab had been unsuccessful. In 2010 she was given golimumab subcutaneously at a monthly dose of 50 mg. She had a negative ANA titre. After 16 months of uninterrupted therapy and sustained response, she developed skin lesions on the upper trunk, back and upper extremities, which worsened on exposure to the sun. The skin biopsy was compatible with subacute lupus erythematosus. Laboratory findings included an ANA titre 1:640, negative anti-Ro/SSA and anti-DNA antibodies. Topical corticosteroid therapy proved inadequate. The patient's condition improved only after discontinuation of golimumab. The causal relationship between subacute cutaneous lupus erythematosus and golimumab is not dose-related and occurs with some delay (a typical feature of immunological adverse reactions). The association is likely, but not confirmed (because re-challenge was not performed). However, a clear improvement was noted after withdrawal. Based on this case, we hypothesized the aetiological role of golimumab-associated immunogenicity. TNF-α antagonist-induced lupus-like syndrome (TAILS) is a well-known side effect of this class of substances. The British Society of Rheumatology recommends discontinuation of the causal anti-TNF-α treatment in patients with TAILS.

  4. IL2/IL21 region polymorphism influences response to rituximab in systemic lupus erythematosus patients.

    PubMed

    Márquez, Ana; Dávila-Fajardo, Cristina Lucía; Robledo, Gema; Rubio, José Luis Callejas; de Ramón Garrido, Enrique; García-Hernández, Francisco J; González-León, Rocío; Ríos-Fernández, Raquel; Barrera, José Cabeza; González-Escribano, Ma Francisca; García, Ma Teresa Camps; Palma, Ma Jesús Castillo; del Mar Ayala, Ma; Ortego-Centeno, Norberto; Martín, Javier

    2013-08-01

    To determine whether the IL2/IL21 region, a general autoimmunity locus, contributes to the observed variation in response to rituximab in patients with systemic lupus erythematosus as well as to analyze its influence in a cohort including other autoimmune diseases. rs6822844 G/T polymorphism at the IL2-IL21 region was analyzed by TaqMan assay in 84 systemic lupus erythematosus (SLE) and 60 different systemic autoimmune diseases Spanish patients receiving rituximab. Six months after the first infusion patients were classified, according to the EULAR criteria, as good responders, partial responders and non-responders. A statistically significant difference was observed in GG genotype frequency between responder (total and partial response) (83.56%) and non-responder (45.45%) SLE patients (p=0.010, odds ratio (OR)=6.10 [1.28-29.06]). No association with the response was evident in the group of patients with autoimmune diseases other than lupus. Furthermore, when both groups of patients were pooled in a meta-analysis, a reduced statistical significance of the association was observed (p=0.024, OR=3.53 [1.06-11.64]). Our results show for a first time that IL2-IL21 region seems to play a role in the response to rituximab in SLE patients but not in other autoimmune diseases.

  5. Long-term cardiac changes in patients with systemic lupus erythematosus

    PubMed Central

    2013-01-01

    Background The aim of this study was evaluate the late-onset repercussions of heart alterations of patients with systemic lupus erythematosus (SLE) after a 13-year follow up. Methods A historical prospective study was carried out involving the analysis of data from the charts of patients with a confirmed diagnosis of lupus in follow up since 1998. The 13-year evolution was systematically reviewed and tabulated to facilitate the interpretation of the data. Results Forty-eight patient charts were analyzed. Mean patient age was 34.5 ± 10.8 years at the time of diagnosis and 41.0 ± 10.3 years at the time of the study (45 women and 3 men). Eight deaths occurred in the follow-up period (two due to heart problems). Among the alterations found on the complementary exams, 46.2% of cases demonstrated worsening at reevaluation and four patients required a heart catheterization. In these cases, coronary angioplasty was performed due to the severity of the obstructions and one case required a further catheterization, culminating in the need for surgical myocardial revascularization. Conclusion The analysis demonstrated progressive heart impairment, with high rates of alterations on conventional complementary exams, including the need for angioplasty or revascularization surgery in four patients. These findings indicate the need for rigorous cardiac follow up in patients with systemic lupus erythematosus. PMID:23635330

  6. Transient Life-Threatening Cerebral Edema in a Patient with Systemic Lupus Erythematosus

    PubMed Central

    Bianchi, Matt T.; Lavigne, Catherine; Sorond, Farzaneh; Bermas, Bonnie

    2015-01-01

    Central nervous system symptoms occur in a substantial portion of patients with systemic lupus erythematosus. However, coma is a rare presentation and is usually secondary to complications such as subarachnoid hemorrhage, seizure, or ischemia. Here, we present a 49-year-old woman with lupus erythematosus and a history of recurrent aseptic meningitis and mild subarachnoid hemorrhage who presented with altered mental status and lethargy that progressed rapidly over hours to the herniation syndrome of coma, extensor posturing, and unilateral pupillary dilation. Spinal fluid showed massive protein elevation (>1600), and head computed tomography revealed global cerebral edema. The clinical and radiologic findings rapidly reversed with intravenous corticosteroids and mannitol within 24 hours, and her mental status improved to baseline. Her course was complicated by 2 episodes of recurrent encephalopathy when corticosteroids were tapered; these resolved after resuming high dosing. Because of ongoing pancytopenia, chemotherapy immunosuppression was delayed, and instead she received intravenous immunoglobulin with improvement in the pancytopenia. She remained cognitively intact during subsequent corticosteroid tapering. Rapid development of coma in lupus patients may be due to a primary process of the disease impacting blood brain barrier integrity. Although rare, this potentially fatal complication may be reversible with acute corticosteroid administration. PMID:19455059

  7. Effect of chloroquine phosphate treatment on serum MMP-9 and TIMP-1 levels in patients with systemic lupus erythematosus.

    PubMed

    Lesiak, A; Narbutt, J; Sysa-Jedrzejowska, A; Lukamowicz, J; McCauliffe, D P; Wózniacka, A

    2010-05-01

    Antimalarials are widely used for the treatment of systemic lupus erythematosus. However, their mechanisms of action have not been fully elucidated. Literature data indicate that matrix metalloproteinases may play a role in the immune response and tissue damage that occur in autoimmune skin diseases. The aim of this study was to determine the effect of 3 months of chloroquine treatment on serum levels of MMP-9 and TIMP-1 in patients with systemic lupus erythematosus. The study group consisted of 25 patients with systemic lupus erythematosus and 25 sex- and age-matched healthy volunteers. Before drug administration, serum levels of MMP-9 and TIMP-1 were determined by enzyme-linked immunosorbent assay. The same procedure was performed after chloroquine treatment. We found significantly higher median serum levels of MMP-9 in patients with systemic lupus erythematosus before therapy (57.20 ng/ml) when compared with controls (44.50 ng/ml) (p < 0.001). After chloroquine therapy the median MMP-9 serum level of systemic lupus erythematosus patients decreased significantly (43 ng/ml; p < 0.001). Before treatment the median TIMP-1 serum level in the patients with systemic lupus erythematosus was significantly higher than in the control group (500 vs. 200 ng/ml; p < 0.001), and after therapy it increased significantly (750 ng/ml TIMP-1; p < 0.001). The results suggest that chloroquine treatment may affect the matrix metalloproteinase network, and this effect may contribute to the immunoregulatory and anti-inflammatory properties of antimalarials.

  8. Reprint of: B cell elimination in systemic lupus erythematosus. Clin. Immunol. 146(2) 90-103.

    PubMed

    Furtado, João; Isenberg, David A

    2013-09-01

    Systemic lupus erythematosus (SLE) is an autoimmune disorder with a worldwide distribution, potentially life-threatening with considerable morbidity. The elimination of pathogenic B cells has emerged as a rational therapeutic option. Many open label studies have reported encouraging results in which clinical and serological remission have invariably been described, often enabling the reduction of steroid and immunosuppressive treatment. However, the results from randomized controlled studies have been disappointing and several questions remain to be answered. In this review we will focus on results of B cell direct depletion in the treatment of patients with systemic lupus erythematosus.

  9. Reprint of: B cell elimination in systemic lupus erythematosus. Clin. Immunol. 146(2) 90-103.

    PubMed

    Furtado, João; Isenberg, David A

    2013-09-01

    Systemic lupus erythematosus (SLE) is an autoimmune disorder with a worldwide distribution, potentially life-threatening with considerable morbidity. The elimination of pathogenic B cells has emerged as a rational therapeutic option. Many open label studies have reported encouraging results in which clinical and serological remission have invariably been described, often enabling the reduction of steroid and immunosuppressive treatment. However, the results from randomized controlled studies have been disappointing and several questions remain to be answered. In this review we will focus on results of B cell direct depletion in the treatment of patients with systemic lupus erythematosus. PMID:23642318

  10. Mesenchymal stem cells for the treatment of systemic lupus erythematosus: is the cure for connective tissue diseases within connective tissue?

    PubMed

    Carrion, Flavio A; Figueroa, Fernando E

    2011-05-11

    Mesenchymal stem cells (MSCs) are now known to display not only adult stem cell multipotency but also robust anti-inflammatory and regenerative properties. After widespread in vitro and in vivo preclinical testing in several autoimmune disease models, allogenic MSCs have been successfully applied in patients with severe treatment-refractory systemic lupus erythematosus. The impressive results of these uncontrolled phase I and II trials - mostly in patients with non-responding renal disease - point to the need to perform controlled multicentric trials. In addition, they suggest that there is much to be learned from the basic and clinical science of MSCs in order to reap the full potential of these multifaceted progenitor cells in the treatment of autoimmune diseases.

  11. Severe pulmonary hypertension as the initial manifestation of systemic lupus erythematosus: a case report and review of the literature.

    PubMed

    Prete, M; Fatone, M C; Vacca, A; Racanelli, V; Perosa, F

    2014-01-01

    Severe pulmonary arterial hypertension (PAH) is rarely observed as the initial manifestation of systemic lupus erythematosus (SLE), and the diagnosis is often delayed. Here we present the case of a 32-year-old woman with severe PAH as the initial manifestation of SLE, who was successfully treated with mycophenolate mofetil and cyclosporine. This case offered the opportunity to critically review the epidemiology data, predictive markers, and pathogenic pathways of SLE-associated PAH (SLE-PAH) in relation to the currently available therapeutic options and to the main clinical trials of the last 10 years focused on the treatment of SLE-PAH. Mycophenolate mofetil and cyclosporine - currently used in the maintenance phase of the disease in certain clinical settings - should be considered, as an alternative to cyclophosphamide, in future clinical trials aimed at evaluating the most effective treatment of SLE-PAH at presentation.

  12. Occupational exposures and risk of systemic lupus erythematosus.

    PubMed

    Parks, Christine G; Cooper, Glinda S

    2005-11-01

    This review summarizes the growing body of epidemiologic and experimental research pertaining to the relationship between SLE and occupational exposures, such as crystalline silica, solvents, and pesticides. Epidemiologic studies, using different designs in different settings, have demonstrated moderate to strong associations between occupational silica exposure and SLE. Recent experimental studies of silica in lupus-prone mice provide support for the idea that, in addition to its known adjuvant effect, silica exposure increases the generation of apoptotic material, an important source of self-antigen. Despite compelling experimental studies of the organic solvent trichloroethylene (TCE) in lupus-prone mice, there is little evidence of an overall association of SLE and occupational exposure to a broad classification of solvents in humans. However, there is a lack of data on SLE in occupational cohorts with exposures to TCE or other specific solvents. One epidemiologic study reported an association of pesticide mixing and SLE, while a recent experimental study reported accelerated disease in pesticide-treated lupus-prone mice. Other occupational exposures worth investigating include asbestos, metals, and UV radiation. Attention should also be given to the role of gene-environment interactions, which may require large, multi-site studies that collect both genetic material and occupational exposure data. The quality of exposure assessment is an important consideration in designing and evaluating these studies. The use of pre-clinical endpoints (e.g. high-titer autoantibodies) in occupational cohorts with well-characterized exposure histories may reveal occupational risk factors for autoimmunity, and may also provide baseline data for studies of determinants of progression to SLE.

  13. Autoantibodies to evolutionarily conserved epitopes of enolase in a patient with discoid lupus erythematosus.

    PubMed Central

    Gitlits, V M; Sentry, J W; Matthew, M L; Smith, A I; Toh, B H

    1997-01-01

    Although the pathology of discoid lupus erythematosus is well documented the causative agents are not known. Here, we report the identity of the target antigen of an autoantibody present in high titre in the serum of a patient with discoid lupus erythematosus. We have demonstrated that the antigen is enolase; first, because it has properties consistent with this glycolytic enzyme (47,000 MW, cytosolic localization and ubiquitous tissue distribution). Secondly, limited amino acid sequence determination after trypsin digestion shows identity with alpha-enolase. Finally, the autoimmune serum immunoblots rabbit and yeast enolase and predominantly one isoelectric form of enolase (PI approximately 6.1). These results indicate that the reactive autoepitopes are highly conserved from man to yeast. The results also suggest that the autoantibodies are most reactive to the alpha-isoform of enolase, although it is possible that they may also be reactive with gamma-enolase, and have least reactivity to beta-enolase. The anti-enolase autoantibodies belong to the immunoglobulin G1 (IgG1) isotype. This is the first report of IgG1 autoantibodies to evolutionarily conserved autoepitopes of enolase in the serum of a patient with discoid lupus erythematosus. Previous reports of autoantibodies to enolase have suggested associations with autoimmune polyglandular syndrome type I and cancer-associated retinopathy. This report and an earlier report of what is likely to be enolase autoantibodies in two patients without systemic disease suggest that enolase autoantibodies have a broad association and are not restricted to any particular disease. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 PMID:9486109

  14. Mitral valve replacement in systemic lupus erythematosus associated Libman-Sacks endocarditis.

    PubMed

    Akhlaq, Anam; Ali, Taimur A; Fatimi, Saulat H

    2016-04-01

    Libman-Sacks endocarditis, first discovered in 1924, is a cardiac manifestation of systemic lupus erythematosus (SLE). Valvular involvement has been associated with SLE and antiphospholipid syndrome (APS). Mitral valve, especially its posterior leaflet, is most commonly involved. We report a case of a 34 year old woman with antiphospholipid antibody syndrome and SLE, who presented with mitral valve regurgitation. The patient underwent a prosthetic mitral valve replacement, with no followup complications. We suggest mechanical valve replacement employment in the management of mitral regurgitation in Libman-Sacks endocarditis, in view of the recent medical literature and our own case report.

  15. Methyl prednisolone pulse therapy in the treatment of systemic lupus erythematosus.

    PubMed

    Isenberg, D A; Morrow, W J; Snaith, M L

    1982-08-01

    Twenty patients with active systemic lupus erythematosus (SLE) were treated with methyl prednisolone pulse therapy (MPPT) and followed up for up to 24 weeks (mean 18 weeks). Beneficial effects of MPPT were observed principally on arthralgia, pleuritic pain, vasculitic skin rash, pyrexia, and lymphadenopathy. The serological tests showing the most improvement were ds DNA binding and the serum C3 level. MPPT was found to be both safe and easy to administer. It may be of value in treating patients with SLE whose disease is not controlled by moderate doses of corticosteroids and may also enable the dose of maintenance corticosteroids to be reduced appreciably. PMID:7114916

  16. Methyl prednisolone pulse therapy in the treatment of systemic lupus erythematosus.

    PubMed Central

    Isenberg, D A; Morrow, W J; Snaith, M L

    1982-01-01

    Twenty patients with active systemic lupus erythematosus (SLE) were treated with methyl prednisolone pulse therapy (MPPT) and followed up for up to 24 weeks (mean 18 weeks). Beneficial effects of MPPT were observed principally on arthralgia, pleuritic pain, vasculitic skin rash, pyrexia, and lymphadenopathy. The serological tests showing the most improvement were ds DNA binding and the serum C3 level. MPPT was found to be both safe and easy to administer. It may be of value in treating patients with SLE whose disease is not controlled by moderate doses of corticosteroids and may also enable the dose of maintenance corticosteroids to be reduced appreciably. PMID:7114916

  17. Spontaneous coronary artery dissection as the first presentation of systemic lupus erythematosus.

    PubMed

    Reddy, Sravan; Vaid, Tejasvini; Ganiga Sanjeeva, Naveen Chandra; Shetty, Ranjan K

    2016-01-01

    A 33-year-old woman with no premorbidities presented to us with chest pain and worsening dyspnoea since 1 week. Systemic examination was suggestive of acute pulmonary oedema and preliminary investigations revealed evolved anterior wall myocardial infarction (MI). The patient was stabilised and taken up for angiography which revealed spontaneous coronary artery dissection (SCAD) of the left anterior descending (LAD) artery. She underwent percutaneous coronary intervention (PCI) for the same. Further investigation into the cause for the SCAD came strongly positive for systemic lupus erythematosus (SLE). She had no prior symptoms suggestive of SLE and the SCAD was its very first clinical manifestation. PMID:27558190

  18. Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus.

    PubMed

    Bermea, Rene S; Sharma, Niharika; Cohen, Kenneth; Liarski, Vladimir M

    2016-09-01

    Atypical hemolytic uremic syndrome is characterized by the presence of thrombocytopenia, microangiopathic hemolytic anemia, and end-organ injury. In this report, we describe two patients with systemic lupus erythematosus who presented with findings compatible with atypical hemolytic uremic syndrome, complicated by acute kidney injury that was refractory to conventional therapies. Both patients exhibited a response to eculizumab, a monoclonal antibody to complement protein C5, with stabilization of their platelet count. On 1-year follow-up from their initial presentation, their hematologic disease remained in remission without recurrence. PMID:27556240

  19. Pulmonary hypertension in systemic lupus erythematosus: report of four cases and review of the literature

    SciTech Connect

    Perez, H.D.; Kramer, N.

    1981-08-01

    Pulmonary hypertension has been reported rarely in patients with systemic lupus erythematosus (SLE). During the past 31/2 yr we have observed pulmonary hypertension as a major clinical manifestation of their disease in four of 43 patients with well-documented SLE followed at out institution. Pulmonary hypertension could be attributed to underlying lung disease in three and was considered to be primary in the remaining patient. Neither hydralazine nor prednisone administration had any effect on the course of the pulmonary hypertension in these patients. The presence of pulmonary hypertension in the course of active SLE may be more common than previously recognized.

  20. Progress with the use of monoclonal antibodies for the treatment of systemic lupus erythematosus.

    PubMed

    Jordan, Natasha; Lutalo, Pamela Mk; D'Cruz, David P

    2015-01-01

    In recent years, significant progress has been made in the use of monoclonal antibodies in the treatment of systemic lupus erythematosus (SLE). Advances in our understanding of the complexity of SLE immunopathogenesis have led to the testing of several biologic agents in clinical trials. Monoclonal therapies currently emerging or under development include B-cell depletion therapies, agents targeting B-cell survival factors, blockade of T-cell co-stimulation and anticytokine therapies. Issues remain, however, regarding clinical trial design and outcome measures in SLE which need to be addressed to optimize translation of these promising therapies into clinical practice.

  1. The inextricable link between atherosclerosis and prototypical inflammatory diseases rheumatoid arthritis and systemic lupus erythematosus

    PubMed Central

    Full, Louise E; Ruisanchez, Cristina; Monaco, Claudia

    2009-01-01

    The increased burden of cardiovascular disease in patients with rheumatoid arthritis and systemic lupus erythematosus has recently become the focus of intense investigation. Proatherogenic risk factors and dysregulated inflammation are the main culprits, leading to enhanced atherosclerosis in subgroups of patients with inflammatory diseases. Common molecular pathways shared by atherosclerosis and inflammatory disease may be involved. In this review we map the key determinants of the increased incidence of cardiovascular disease in patients with inflammatory diseases at each step of the atherogenesis. PMID:19435478

  2. Development of Systemic Lupus Erythematosus Following Interferon-α Therapy for Hepatitis C Infection.

    PubMed

    Khalil-Ur-Rehman; Khokhar, Nasir

    2016-03-01

    Interferon-alpha (IFN-α) therapy has been associated with de novo development of systemic lupus erythematosus (SLE). We report a 48-year woman with chronic hepatitis C, who developed low grade fever, joint aches and pains, painful mouth ulcers, shortness of breath, dry cough and pleuritic chest pain after 2 months of completion of treatment with pegylated interferon-alpha. These clinical manifestations and the relevant immunologic investigations were in favour of SLE. She responded well to corticosteroids and hydroxychloroquine treatment. PMID:26975957

  3. Peer Support and Psychosocial Pain Management Strategies for Children with Systemic Lupus Erythematosus

    PubMed Central

    Nabors, Laura; Ige, Teminijesu John; Fevrier, Bradley

    2015-01-01

    This paper reviews information on Systemic Lupus Erythematosus (SLE) in children. Children with this chronic illness often experience pain related to their condition. They also can experience social isolation. This paper reviews psychosocial information on peer support and cognitive behavioral pain management strategies. The information presented in this paper provides new insights for health professionals assisting children and families in coping with psychological facets of this disease. Research focusing on ways by which peers and friends can support the child's use of psychological pain management strategies will provide new information for the literature. PMID:26583153

  4. Clinical Features of Neuropsychiatric Syndromes in Systemic Lupus Erythematosus and Other Connective Tissue Diseases

    PubMed Central

    Kasama, Tsuyoshi; Maeoka, Airi; Oguro, Nao

    2016-01-01

    Systemic lupus erythematosus (SLE) and related disorders are chronic inflammatory diseases characterized by abnormalities and, in some cases, even complete failure of immune responses as the underlying pathology. Although almost all connective tissue diseases and related disorders can be complicated by various neuropsychiatric syndromes, SLE is a typical connective tissue disease that can cause neurological and psychiatric syndromes. In this review, neuropsychiatric syndromes complicating connective tissue diseases, especially SLE are outlined, and pathological and other conditions that should be considered in the differential diagnosis are also discussed. PMID:26819561

  5. Villous adenoma of gallbladder in a patient with systemic lupus erythematosus

    PubMed Central

    Xu, Yuyun; Yuan, Jianhua; Chong, Vincent; Ding, Zhongxiang

    2012-01-01

    Villous adenomas occur most frequently in the rectum and colon. These tumors are rarely seen in the gallbladder. We report a case of gallbladder villous adenomas in a 69-year-old patient who has systemic lupus erythematosus (SLE). The patient was admitted for investigation of a gallbladder mass. Ultrasonography, computed tomography, and magnetic resonance imaging showed two well-circumscribed lobulated masses in the gallbladder. Open cholecystectomy was performed and histological examination revealed typical features of villous adenoma. This report describes the first case of villous adenomas of gallbladder with SLE, and documents its imaging findings comprehensively. PMID:23798953

  6. Immunopathological roles of cytokines, chemokines, signaling molecules, and pattern-recognition receptors in systemic lupus erythematosus.

    PubMed

    Yu, Shui-Lian; Kuan, Woon-Pang; Wong, Chun-Kwok; Li, Edmund K; Tam, Lai-Shan

    2012-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with unknown etiology affecting more than one million individuals each year. It is characterized by B- and T-cell hyperactivity and by defects in the clearance of apoptotic cells and immune complexes. Understanding the complex process involved and the interaction between various cytokines, chemokines, signaling molecules, and pattern-recognition receptors (PRRs) in the immune pathways will provide valuable information on the development of novel therapeutic targets for treating SLE. In this paper, we review the immunopathological roles of novel cytokines, chemokines, signaling molecules, PRRs, and their interactions in immunoregulatory networks and suggest how their disturbances may implicate pathological conditions in SLE.

  7. Impact of race and ethnicity in the course and outcome of systemic lupus erythematosus.

    PubMed

    González, Luis Alonso; Toloza, Sergio M A; Alarcón, Graciela S

    2014-08-01

    Genetic factors seem to play a more important role early in the course of systemic lupus erythematosus (SLE), whereas nongenetic factors seem to play a more important role over the course of the disease. SLE is more frequent with less favorable outcomes in nonwhite populations. To overcome these differences and reduce the immediate-term, mediate-term, and long-term impact of SLE among disadvantaged populations, it is essential to increase disease awareness, to improve access to health care and to provide care to these patients in a consistent manner regardless of the severity of their disease.

  8. A case of late-onset systemic lupus erythematosus with severe anemia.

    PubMed

    Matsumoto, Moeko; Kaieda, Shinjiro; Honda, Seiyo; Ida, Hiroaki; Hoshino, Tomoaki; Fukuda, Takaaki

    2013-01-01

    A 59-year-old woman was referred to our hospital because of severe anemia and leucopenia. Although she developed mild arthralgia without the typical symptoms of systemic lupus erythematosus (SLE), positivity for anti-Sm antibodies led us to a diagnosis of late-onset SLE. Autoimmune hemolytic anemia (AIHA) and suppression of reticulocyte production were considered to have been involved in the etiology of severe anemia. Administration of oral prednisolone (PSL) resulted in a marked improvement of the hematological abnormalities. As late-onset SLE is rare and patients tend to show the typical symptoms less frequently, close attention should be focused on latent symptoms and immunological findings.

  9. Genetics of systemic lupus erythematosus: immune responses and end organ resistance to damage

    PubMed Central

    Dai, Chao; Deng, Yun; Quinlan, Aaron; Gaskin, Felicia; Tsao, Betty P; Fu, Shu Man

    2014-01-01

    Systemic lupus erythematosus (SLE) is a prototypic systemic autoimmune disorder. Considerable progress has been made to delineate the genetic control of this complex disorder. In this review, selected aspects of human and mouse genetics related to SLE are reviewed with emphasis on genes that contribute to both innate and adaptive immunity and to genes that contribute directly to susceptibility to end organ damage. It is concluded that the interactions among these two major pathways will provide further insight into the pathogenesis of SLE. An interactive model of the two major pathways is proposed without emphasis on the importance of breaking tolerance to autoantigens. PMID:25458999

  10. Gastrointestinal manifestations as initial presenting features in a 40 years old woman with systemic lupus erythematosus.

    PubMed

    Ahmed, Q M; Arafat, S M; Azad, A K; Chowdhury, M J; Hasan, M K; Ahmed, F; Ananna, M A

    2015-01-01

    Gastrointestinal (GI) symptoms are common in patients with systemic lupus erythematosus (SLE). These symptoms can be due to primary GI disorders like peptic ulcer disease, pancreatitis or intestinal obstruction. But they can be due to SLE itself or complications of treatment of SLE. In this case report, we describe a 40 years old woman who presented initially with GI complaints. Later she was diagnosed as a case of SLE with chronic intestinal pseudo-obstruction (CIPO). The problems related to diagnosis and management is also discussed. PMID:25725689

  11. Diffuse alveolar hemorrhage in patients with systemic lupus erythematosus. Clinical manifestations, treatment, and prognosis.

    PubMed

    Martínez-Martínez, Marco Ulises; Abud-Mendoza, Carlos

    2014-01-01

    Diffuse alveolar hemorrhage (DAH) in patients with systemic lupus erythematosus is a rare but potentially fatal condition. Although the pathogenesis of this condition is unknown, high disease activity is the main characteristic; moreover, histopathology in some studies showed alveolar immune complex deposits and capillaritis. Clinical features of DAH include dyspnea, a drop in hemoglobin, and diffuse radiographic alveolar images, with or without hemoptysis. Factors associated with mortality include mechanical ventilation, renal failure, and infections. Bacterial infections have been reported frequently in patients with DAH, but also invasive fungal infections including aspergillosis. DAH treatment is based on high dose methylprednisolone; other accepted therapies include cyclophosphamide (controversial), plasmapheresis, immunoglobulin and rituximab.

  12. Myelitis transverse in Sjögren's syndrome and systemic lupus erythematosus: presentation of 3 cases.

    PubMed

    Menor Almagro, Raúl; Ruiz Tudela, María del Mar; Girón Úbeda, Juan; Cardiel Rios, Mario H; Pérez Venegas, José Javier; García Guijo, Carmen

    2015-01-01

    Transverse myelitis is a rare focal inflammation of the spinal cord. Multiple etiologies have been identified including autoimmune diseases, mainly systemic lupus erythematosus and Sjögren' syndrome. It can occur in an acute or subacute clinical onset, with the acute presentation having a worse prognosis. An early diagnosis and intensive treatment are important features recommended in these patients. We present three cases with transverse myelitis associated with autoimmune diseases. We discuss different clinical manifestations, association with autoantobodies, radiologic findings, and therapeutic and prognostic issues.

  13. False positive results for antibody to HIV in two men with systemic lupus erythematosus.

    PubMed Central

    Esteva, M H; Blasini, A M; Ogly, D; Rodríguez, M A

    1992-01-01

    False positive results were obtained for HIV tests in two men with active systemic lupus erythematosus (SLE) who were suspected of being infected with HIV because of fever, weight loss, lymphadenopathy, and inflammatory myopathy. Enzyme linked immunosorbent assays (ELISAs) for HIV were twice positive when tested three times over a period of six months. Western blot analysis showed reactivity against the gp41 band in patient 1. False positive results for HIV tests can occur in patients with SLE, potentially leading to an erroneous diagnosis of HIV infection. PMID:1417140

  14. The risk of ultraviolet radiation exposure from indoor lamps in lupus erythematosus

    PubMed Central

    Klein, Rachel S.; Sayre, Robert M.; Dowdy, John C.; Werth, Victoria P.

    2008-01-01

    It is well known that ultraviolet radiation can exacerbate skin disease in patients with lupus erythematosus. While many patients are advised to avoid sunlight and artificial tanning, it is not clear how best to counsel patients regarding the use of indoor lamps. Indeed, many of the light bulbs commonly used in the home and workplace emit low-dose ultraviolet radiation. The irradiance is considerably lower than that of the sun, however the exposure time can last for hours and is typically repeated on a daily basis. Therefore, it is possible that this chronic exposure could ultimately result in a significant accumulation of damage. PMID:18992852

  15. Incidental retinal vascular occlusions on hydroxychloroquine screening in patients with systemic lupus erythematosus

    PubMed Central

    Bajwa, Asima; Khurana, Gitanjali; Kimpel, Donald; Reddy, Ashvini K

    2015-01-01

    Objective The proportion of patients with systemic lupus erythematosus (SLE) who manifest retinal involvement increases many fold in patients with active systemic disease. The objective of this report is to stress upon the significance of comprehensive ophthalmic assessment of all SLE patients to prevent and manage blinding ocular manifestations of the disease. Methods Retrospective case review. Results Incidental retinal vascular complications seen in patients undergoing baseline hydroxychloroquine screening. Conclusion The purpose of comprehensive ophthalmic screening in SLE patients is twofold. It will aid in the diagnosis and treatment of blinding ocular complications of the disease and monitor hydroxychloroquine macular toxicity. PMID:26064074

  16. A severe case of systemic lupus erythematosus with increased pressure communicating hydrocephalus

    PubMed Central

    Özen, Gülşen; Yılmaz-Öner, Sibel; Tuncer, Neşe; Akbaş, Türkay; Tuğlular, Serhan; Direskeneli, Haner

    2015-01-01

    Normal/increased pressure hydrocephaly is an unusual manifestation of systemic lupus erythematosus (SLE), and the pathogenesis is still unclear. We report the case of an 18-year-old white female with severe refractory renal and pulmonary involvement who developed stupor during intensive immunosuppressive treatment. Enlarged ventricles on imaging and increased intracranial pressure with the exclusion of infectious and hemorrhagic/thrombotic processes suggested increased pressure communicating hydrocephalus associated with SLE. Few case reports are reviewed, and potential pathophysiologic mechanisms are discussed. PMID:27708931

  17. Hypercementosis: a rare finding in a patient with systemic lupus erythematosus.

    PubMed

    Shoor, Hitesh; Sujir, Nanditha; Mutalik, Sunil; Pai, Keerthilatha M

    2014-01-01

    Hypercementosis is excessive deposition of non-neoplastic cementum over normal root cementum, which alters root morphology. This cementum may be either hypocellular or cellular in nature. The aetiopathogenesis of hypercementosis is ambiguous. Although most of the cases are idiopathic, several local and systemic factors are also linked to this condition, such as Paget's disease, acromegaly, vitamin A deficiency, etc. We report two rare cases of hypercementosis associated with systemic lupus erythematosus, not previously described in the literature, and also discuss the possible aetiopathogenesis. PMID:25427926

  18. Brodie's abscess of the proximal femoral epiphysis in an adult woman with systemic lupus erythematosus.

    PubMed

    Miyanishi, Keita; Yamamoto, Takuaki; Irisa, Takahiko; Jingushi, Seiya; Noguchi, Yasuo; Iwamoto, Yukihide

    2002-06-01

    We report an unusual case of pathologically proved femoral head Brodie's abscess mimicking avascular necrosis of bone in a 51-year-old woman with a 2-year history of corticosteroid treatment for systemic lupus erythematosus. On plain radiographs, a rounded lucency and thin sclerotic margins together with subchondral collapse and a lytic region were observed in the femoral head. The histopathologic examination revealed a central abscess formation surrounded by fibrous tissue with the aggregation of neutrophils and plasma cells. To our knowledge, this is the first case report describing a Brodie's abscess which had developed within the proximal femoral epiphysis in an adult.

  19. The impact and implications of neuropsychiatric systemic lupus erythematosus in adolescents.

    PubMed

    Klein-Gitelman, Marisa; Brunner, Hermine I

    2009-07-01

    Signs and symptoms of neuropsychiatric systemic lupus erythematosus (NPSLE), as defined by the American College of Rheumatology case definitions, occur commonly in adolescents with lupus. Thought and mood disorders are more difficult to identify than specific central or peripheral nervous system injuries. Furthermore, thought and mood disorders must be differentiated from other causes, including traumatic, metabolic, endocrinologic, toxic, and infectious etiologies. It is also important to recognize that primary mood and thought disorders can present in adolescence. The effects of chronic disease during adolescent development can also trigger alterations in mood and cognitive function. This article reviews proposed etiologies of injury, diagnosis, and differential diagnosis of NPSLE. At this time, no specific test identifies NPSLE as the cause of mood and cognitive changes; however, current efforts to standardize evaluation of cognitive function and develop imaging techniques that identify anatomic brain changes will improve the practice of rheumatology. PMID:19604466

  20. Recurrent stroke and multi-infarct dementia in systemic lupus erythematosus: association with antiphospholipid antibodies.

    PubMed Central

    Asherson, R A; Mercey, D; Phillips, G; Sheehan, N; Gharavi, A E; Harris, E N; Hughes, G R

    1987-01-01

    Four patients with recurrent stroke and multi-infarct dementia are presented in whom the dementia was progressive and severe. Three of the patients developed the dementia during the course of an illness which was punctuated by repeated episodes of cerebral infarction demonstrated by computed tomographic (CT) scans. The fourth patient presented with an illness dominated by progressive and deteriorating higher mental functions, which culminated in a major stroke 18 months later. Three patients fulfilled the American Rheumatism Association (ARA) criteria for the classification of systemic lupus erythematosus, the fourth had a 'lupus-like' disease. All had livedo reticularis, severe migraines, and also demonstrated antibodies to phospholipids. All four patients suffered deep vein thromboses. Images PMID:3116954

  1. Intestinal pseudo-obstruction in patients with systemic lupus erythematosus: A real diagnostic challenge

    PubMed Central

    García López, Carlos Alberto; Laredo-Sánchez, Fernando; Malagón-Rangel, José; Flores-Padilla, Miguel G; Nellen-Hummel, Haiko

    2014-01-01

    Intestinal pseudo-obstruction secondary to systemic lupus erythematosus (SLE) is a rare syndrome described in recent decades. There are slightly over 30 published cases in the English language literature, primarily associated with renal and hematological disease activity. Its presentation and evolution are a diagnostic challenge for the clinician. We present four cases of intestinal pseudo-obstruction due to lupus in young Mexican females. One patient had a previous diagnosis of SLE and all presented with a urinary tract infection of varying degrees of severity during their evolution. We consider that recognition of the disease is of vital importance because it allows for establishing appropriate management, leading to a better prognosis and avoiding unnecessary surgery and complications. PMID:25170234

  2. Discoid lupus erythematosus of the eyelids associated with staphylococcal blepharitis and Meibomian gland dysfunction.

    PubMed

    Ena, P; Pinna, A; Carta, F

    2006-01-01

    Lower eyelid involvement occurs in 6% of patients with discoid lupus erythematosus (DLE). Eyelid lesions are rarely the initial manifestation of DLE. We describe a 25-year-old woman presenting with discoid lesions of the lower eyelids, staphylococcal blepharitis and Meibomian gland dysfunction, who later developed a discoid lesion on the chin. Histopathological and immunofluorescence studies of a biopsy specimen from this lesion established the diagnosis of DLE. We are unaware of any previously reported cases of DLE presenting with discoid eyelid lesions associated with staphylococcal blepharitis and Meibomian gland dysfunction. DLE should be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene. Misdiagnosis may lead to eyelid margin deformities, necessitate a complicated full-thickness biopsy, and delay diagnosis of systemic lupus.

  3. Intestinal pseudo-obstruction in patients with systemic lupus erythematosus: a real diagnostic challenge.

    PubMed

    García López, Carlos Alberto; Laredo-Sánchez, Fernando; Malagón-Rangel, José; Flores-Padilla, Miguel G; Nellen-Hummel, Haiko

    2014-08-28

    Intestinal pseudo-obstruction secondary to systemic lupus erythematosus (SLE) is a rare syndrome described in recent decades. There are slightly over 30 published cases in the English language literature, primarily associated with renal and hematological disease activity. Its presentation and evolution are a diagnostic challenge for the clinician. We present four cases of intestinal pseudo-obstruction due to lupus in young Mexican females. One patient had a previous diagnosis of SLE and all presented with a urinary tract infection of varying degrees of severity during their evolution. We consider that recognition of the disease is of vital importance because it allows for establishing appropriate management, leading to a better prognosis and avoiding unnecessary surgery and complications.

  4. Coincidence of tuberous sclerosis and systemic lupus erythematosus-a case report.

    PubMed

    Carrasco Cubero, Carmen; Bejarano Moguel, Verónica; Fernández Gil, M Ángeles; Álvarez Vega, Jose Luis

    2016-01-01

    Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest. PMID:26526985

  5. Management considerations for childhood-onset systemic lupus erythematosus patients and implications on therapy.

    PubMed

    Silva, Clovis Artur; Aikawa, Nadia Emi; Pereira, Rosa Maria Rodrigues; Campos, Lucia Maria Arruda

    2016-01-01

    Childhood-onset systemic lupus erythematosus (cSLE) is a chronic inflammatory and autoimmune disease that may involve various organs and systems. This narrative review focuses on the recent evidence relating to cSLE management. The general management considerations of cSLE patients require the use of validated classification criteria, disease and health-related quality of life tools evaluation, as well as assessments of lupus nephritis biomarkers and cSLE quality indicators. The drug treatment for cSLE patients includes general supportive care and immunosuppressive therapy. Important implications on cSLE therapy are also updated such as infection, vaccination, infertility, pregnancy, contraception, dyslipidemia, physical activity, cancer, bone health, drug pharmacokinetics, adherence, academic outcomes, transition to adult care and cumulative organ damage.

  6. Dendritic Cells in Systemic Lupus Erythematosus: From Pathogenic Players to Therapeutic Tools.

    PubMed

    Klarquist, Jared; Zhou, Zhenyuan; Shen, Nan; Janssen, Edith M

    2016-01-01

    System lupus erythematosus (SLE) is a multifactorial systemic autoimmune disease with a wide variety of presenting features. SLE is believed to result from dysregulated immune responses, loss of tolerance of CD4 T cells and B cells to ubiquitous self-antigens, and the subsequent production of anti-nuclear and other autoreactive antibodies. Recent research has associated lupus development with changes in the dendritic cell (DC) compartment, including altered DC subset frequency and localization, overactivation of mDCs and pDCs, and functional defects in DCs. Here we discuss the current knowledge on the role of DC dysfunction in SLE pathogenesis, with the focus on DCs as targets for interventional therapies. PMID:27122656

  7. Hydroxychloroquine-induced toxic hepatitis in a patient with systemic lupus erythematosus: a case report.

    PubMed

    Abdel Galil, S M

    2015-05-01

    Increased serum level of liver enzymes is a common finding in patients with systemic lupus erythematosus (SLE). Hepatotoxic drugs, viral hepatitis and fatty liver are thought to be the main causes of hepatic lesion in these patients. Our aim was to determine the cause of strikingly elevated liver enzymes in a case with systemic lupus presenting with acute abdomen. Liver enzyme abnormality was defined as a 10-fold or greater increase in aspartate aminotransferase and alanine aminotransferase. Acute toxic hepatitis was diagnosed, which rapidly returned to normal after cessation of the suspected causative medication, hydroxychloroquine, and subsequent administration of mycophenolate mofetil. Elevated liver enzymes are a major concern and should be well investigated in SLE patients.

  8. Coincidence of tuberous sclerosis and systemic lupus erythematosus-a case report.

    PubMed

    Carrasco Cubero, Carmen; Bejarano Moguel, Verónica; Fernández Gil, M Ángeles; Álvarez Vega, Jose Luis

    2016-01-01

    Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest.

  9. Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus

    PubMed Central

    Shariatpanahi, Shamsa; Pourfarzam, Shahryar; Gheini, Mohammadhosein

    2016-01-01

    Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Iran) in May 2011 because of lower extremities edema and ascites and fever from 1.5 month ago. In physical examinations she had generalized lymphadenopathy, splenomegaly and pleural effusion. In laboratory tests she had pancytopenia, positive ANA and Anti DNA (ds), hypocomplementemia, hypertriglyceridemia and high ferritin level. Gradually she had signs of RPGN and ARDS. The patient had no skin and musculoskeletal signs of SLE and no liver failure nor coagulopathy of MAS. Her lymph node biopsy was reported as Castleman syndrome. Unlike other studies, the patient showed MAS before treatment with cytotoxic for lupus nephritis. PMID:27799976

  10. Inverse Association of Parkinson Disease With Systemic Lupus Erythematosus

    PubMed Central

    Liu, Feng-Cheng; Huang, Wen-Yen; Lin, Te-Yu; Shen, Chih-Hao; Chou, Yu-Ching; Lin, Cheng-Li; Lin, Kuen-Tze; Kao, Chia-Hung

    2015-01-01

    Abstract The effects of the inflammatory mediators involved in systemic lupus erythematous (SLE) on subsequent Parkinson disease have been reported, but no relevant studies have focused on the association between the 2 diseases. This nationwide population-based study evaluated the risk of Parkinson disease in patients with SLE. We identified 12,817 patients in the Taiwan National Health Insurance database diagnosed with SLE between 2000 and 2010 and compared the incidence rate of Parkinson disease among these patients with that among 51,268 randomly selected age and sex-matched non-SLE patients. A Cox multivariable proportional-hazards model was used to evaluate the risk factors of Parkinson disease in the SLE cohort. We observed an inverse association between a diagnosis of SLE and the risk of subsequent Parkinson disease, with the crude hazard ratio (HR) being 0.60 (95% confidence interval 0.45–0.79) and adjusted HR being 0.68 (95% confidence interval 0.51–0.90). The cumulative incidence of Parkinson disease was 0.83% lower in the SLE cohort than in the non-SLE cohort. The adjusted HR of Parkinson disease decreased as the follow-up duration increased and was decreased among older lupus patients with comorbidity. We determined that patients with SLE had a decreased risk of subsequent Parkinson disease. Further research is required to elucidate the underlying mechanism. PMID:26579824

  11. [Kikuchi-Fujimoto disease prior to childhood-systemic lupus erythematosus diagnosis].

    PubMed

    Martins, Sofia S; Buscatti, Izabel M; Freire, Pricilla S; Cavalcante, Erica G; Sallum, Adriana M; Campos, Lucia M A; Silva, Clovis A

    2014-01-01

    Kikuchi-Fujimoto disease (KFD) is a self-limiting histiocytic necrotizing lymphadenitis of unknown origin. Of note, KFD was infrequently reported in adult systemic lupus erythematosus (SLE), with rare occurrence in childhood-SLE (C-SLE) patients. To our knowledge, the prevalence of KFD in the paediatric lupus population was not studied. Therefore, in a period of 29 consecutive years, 5,682 patients were followed at our institution and 289 (5%) met the American College of Rheumatology classification criteria for SLE, one had isolated KFD (0.03) and only one had KFD associated to C-SLE diagnoses, which case was reported herein. A 12 year-old female patient had high fever, fatigue and cervical and axillary lymphadenopathy. The antinuclear antibodies (ANA) were negative, with positive IgM and IgG herpes simplex virus type 1 and type 2 serologies. Fluorine-18-fluoro-deoxy-glucose positron emission tomography/computed tomography (PET/CT) imaging demonstrated diffuse lymphadenopathy. The axillary lymph node biopsy showed necrotizing lymphadenitis with histiocytes, without lymphoproliferative disease, compatible with KFD. After 30 days, she presented spontaneous regression and no therapy was required. Nine months later, she developed malar rash, photosensitivity, oral ulcers, lymphopenia and ANA 1:320 (homogeneous nuclear pattern). At that moment the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 10 and she was treated with prednisone (1.0mg/kg/day) and hidroxychloroquine showing progressive improvement of hers signs and symptoms. In conclusion, KFD is a benign and rare disease in our paediatric lupus population. We also would like to reinforce the relevance of autoimmune diseases diagnosis during the follow-up of patients with KFD.

  12. Survival rates and risk factors for mortality in systemic lupus erythematosus patients in a Chinese center.

    PubMed

    Wu, Ge; Jia, Xiaoyuan; Gao, Dan; Zhao, Zhanzheng

    2014-07-01

    This paper aims to study the survival and risk factors affecting the long-term prognosis of Chinese patients with systemic lupus erythematosus (SLE). We collected clinical data of 1,072 SLE patients at the time of diagnosis. The Kaplan-Meier method was used to estimate the survival rate, and the Cox proportional hazard regression model for the risk factors affecting prognosis. Of the original 1,072 recruited SLE patients, 665 (570 females and 95 males) were successfully followed up. Mean follow-up was 5.47 ± 4.62 years. Mean age of onset was 29.4 ± 13.4 years. Eighty-one patients did not survive during follow-up; infection, followed by cardiovascular disease, renal failure and SLE disease activity were the leading causes of death. The 5- and 10-year survival rates were 91.2 and 79.6 %, respectively. Moreover, the 5-year survival rates of female and male patients were 92.6 and 81.6 % respectively, and the 10-year survival rates were 80.8 and 62.3 %, respectively. Univariate analyses indicated that male gender, older age of onset, hypertension, increased blood creatinine levels, and high-density lipoprotein cholesterol at the time of diagnosis of SLE were risk factors for all-cause mortality. After adjusting for potential confounders by multivariate analysis, male gender, older age of onset, and high SLEDAI scores at the time of diagnosis were independent risk factors for all-cause mortality in SLE patients. The long-term survival of Chinese SLE patients is comparable to that of other countries. Older age of onset, high disease activity, and decline in renal function are independent risk factors for mortality in patients with SLE.

  13. Cellular and molecular pathogenesis of systemic lupus erythematosus: lessons from animal models.

    PubMed

    Pathak, Simanta; Mohan, Chandra

    2011-01-01

    Systemic lupus erythematosus (SLE) is a complex disease characterized by the appearance of autoantibodies against nuclear antigens and the involvement of multiple organ systems, including the kidneys. The precise immunological events that trigger the onset of clinical manifestations of SLE are not yet well understood. However, research using various mouse strains of spontaneous and inducible lupus in the last two decades has provided insights into the role of the immune system in the pathogenesis of this disease. According to our present understanding, the immunological defects resulting in the development of SLE can be categorized into two phases: (a) systemic autoimmunity resulting in increased serum antinuclear and antiglomerular autoantibodies and (b) immunological events that occur within the target organ and result in end organ damage. Aberrations in the innate as well as adaptive arms of the immune system both play an important role in the genesis and progression of lupus. Here, we will review the present understanding--as garnered from studying mouse models--about the roles of various immune cells in lupus pathogenesis.

  14. Follow-up of patients with systemic lupus erythematosus: what is not found in the guidelines.

    PubMed

    Jiménez-Alonso, J; Vargas-Hitos, J A; Navarrete-Navarrete, N; Zamora-Pasadas, M; Aguilar-Huergo, S; Jáimez, L; Sabio, J M

    2013-12-01

    A series of measures in the management of patients with systemic lupus erythematosus (SLE) which usually are not found in the lupus guidelines are discussed. In the lupus patient who has been well-controlled in the long term, the dose of hydroxychloroquine should be progressively reduced, without decreasing more than approximately 600 mg per week. We recommend taking this drug in the morning in patients with insomnia, at night in those with dyspepsia and to separate the intake of the drug from the shower (and the water should be as cool as possible) in those patients with aquagenic pruritus. We do not use prednisone on alternate days and exceptionally divide the dose into ¾ before breakfast and ¼ before dinner. Twenty to 30 min should be used per patient in every scheduled visit to assure a good clinical and human practice. We analyzed the follow-up of 112 consecutive patients from our systemic disease unit and found that 71.4% of them had symptoms that were unexplained by lupus and we only referred 8.9% of them to other specialists, probably because of our general training as internal medicine doctors. We suggest that knowing the views of SLE specialists might be of interest since, well-designed studies that would allow to progress in the understanding of this disease could be performed based on their experience.

  15. Systemic lupus erythematosus induced by anti-tumour necrosis factor alpha therapy: a French national survey

    PubMed Central

    De Bandt, Michel; Sibilia, Jean; Le Loët, Xavier; Prouzeau, Sebastian; Fautrel, Bruno; Marcelli, Christian; Boucquillard, Eric; Siame, Jean Louis; Mariette, Xavier

    2005-01-01

    The development of drug-induced lupus remains a matter of concern in patients treated with anti-tumour necrosis factor (TNF) alpha. The incidence of such adverse effects is unknown. We undertook a retrospective national study to analyse such patients. Between June and October 2003, 866 rheumatology and internal medicine practitioners from all French hospital centres prescribing anti-TNF in rheumatic diseases registered on the website of the 'Club Rhumatismes et Inflammation' were contacted by email to obtain the files of patients with TNF-induced systemic lupus erythematosus. Twenty-two cases were collected, revealing two aspects of these manifestations. Ten patients (six patients receiving infliximab, four patients receiving etanercept) only had anti-DNA antibodies and skin manifestations one could classify as 'limited skin lupus' or 'toxidermia' in a context of autoimmunity, whereas 12 patients (nine patients receiving infliximab, three patients receiving etanercept) had more complete drug-induced lupus with systemic manifestations and at least four American Congress of Rheumatology criteria. One patient had central nervous system manifestations. No patients had lupus nephritis. The signs of lupus occurred within a mean of 9 months (range 3–16 months) in patients treated with infliximab and within a mean of 4 months (range 2–5 months) in patients treated with etanercept. In all cases after diagnosis was determined, anti-TNF was stopped and specific treatment introduced in eight patients: two patients received intravenous methylprednisolone, four patients received oral steroids (15–35 mg/day), and two patients received topical steroids. Lupus manifestations abated within a few weeks (median 8 weeks, standard deviation 3–16) in all patients except one with longer-lasting evolution (6 months). At that time, cautious estimations (unpublished data from Schering Plough Inc. and Wyeth Inc.) indicated that about 7700 patients had been exposed to infliximab and

  16. [Physiopathology of systemic lupus erythematosus: a 2014 update].

    PubMed

    Mathian, A; Arnaud, L; Amoura, Z

    2014-08-01

    Systemic lupus erythematous is a chronic autoimmune disease characterized by the inflammation of several tissues and the production of auto-antibodies directed against nuclear antigens. Complex genetic disorders and environmental factors are at the origin of the disease but the precise cause of the auto-immune process is still unknown. Both innate and adaptive immune systems are involved. Apoptosis seems to be the main source of auto-antigens. The interactions between apoptotic cells, dendritic cells and lymphocytes activate the production of pathogenic antibodies and T lymphocytes. Amplification loops sustain the auto-immune process and the chronic inflammation. Several data point out B-lymphocytes and several cytokines involved in their homeostasis as new promising therapeutic targets.

  17. Cheek and periorbital peculiar discoid lupus erythematosus: rare clinical presentation mimicking tinea faciei, cutaneous granulomatous disease or blepharitis.

    PubMed

    Nakamura, Satoshi; Yamada, Tomoko; Umemoto, Naoka; Nakamura, Toshinobu; Wakatabi, Koji; Iida, Eri; Masaki, Masumi; Kakurai, Maki; Demitsu, Toshio

    2015-01-01

    We present clinically peculiar facial discoid lupus erythematosus (DLE) that mimicked tinea faciei. Although DLE is a chronic autoimmune dermatosis, it has a variety of rare clinical presentations, including periorbital DLE, comedonic DLE and hypertrophic DLE recently. In this case, a scaly, erythematous lesion on the eyelid and the central healed, mildly elevated, annularly distributed facial DLE mimicked tinea faciei, complicating our diagnosis.

  18. Mycobacterium intracellulare infection of the shoulder and spine in a patient with steroid-treated systemic Lupus erythematosus

    SciTech Connect

    Zvetina, J.R.; Rubinstein, H.; Demos, T.C.

    1982-05-01

    Atypical mycobacterial infections of bone are rare. A patient with systemic lupus erythematosus treated with steroids developed an M. intracellulare infection of the shoulder and spine. These infections are insidious and diagnosis is difficult. Marked involvement of one joint, large effusion, or aspirated small synovial fragments suggest an atypical tuberculous joint infection.

  19. Multisystemic diseases and ethnicity: a focus on lupus erythematosus, systemic sclerosis, sarcoidosis and Behçet disease.

    PubMed

    Petit, A; Dadzie, O E

    2013-10-01

    We present an overview of the association between ethnicity and the clinical and epidemiological aspects of four multisystemic diseases: lupus erythematosus, systemic sclerosis, sarcoidosis and Behçet disease. In particular, we highlight observed ethnic differences in cutaneous manifestations of these diseases. This article should help guide clinical management, as well as serve to highlight future areas for research.

  20. [Modulating the survival and maturation system of B lymphocytes: Current and future new therapeutic strategies in systemic lupus erythematosus].

    PubMed

    Valor, Lara; López-Longo, Francisco Javier

    2015-09-01

    Systemic lupus erythematosus is an autoimmune disease associated with an aberrant production of autoantibodies by self-reactive B lymphocytes. The study of the phenotypic characteristics of B lymphocytes and the identification of their surface receptors such as BAFF-R, TACI and BCMA, which are responsible of their survival and maturation, have contributed to the development of new therapeutic strategies in recent years.

  1. The spectrum of nasal involvement in systemic lupus erythematosus and its association with the disease activity.

    PubMed

    Kusyairi, K A; Gendeh, B S; Sakthiswary, R; Shaharir, S S; Haizlene, A H; Yusof, K H

    2016-04-01

    The purpose of this study was to determine the spectrum of nasal involvement in systemic lupus erythematosus (SLE) and its association with the disease activity of SLE based on the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). This was a cross-sectional and observational study involving 73 stable SLE patients. All subjects were evaluated for the SLEDAI scores and had nasal endoscopic examination. The most commonly reported symptom was nasal congestion (31.5%) followed by nasal itchiness (26.0%), runny nose (20.5%) and nasal dryness (19.2%). Almost half (42.9%) of the subjects had nasal mucosal abnormalities, which included mucositis, crusting, ulceration, bifid middle turbinate, septal spur, Jacobson's organ, deviated nasal septum, bilateral inferior turbinate hypertrophy, everted uncinate process, nasopharynx cleft and torus palatinus. The median SLEDAI score for subjects with nasal symptoms was significantly higher than subjects without nasal symptoms (p < 0.05). Similarly, subjects with moderate to high activity (SLEDAI scores of 6-19) had a significantly higher frequency of both nasal symptoms and nasal mucosal abnormalities (p < 0.05) compared to subjects with no to mild activity (SLEDAI scores of 0-5).

  2. T lymphocytes from patients with systemic lupus erythematosus are resistant to induction of autophagy

    PubMed Central

    Alessandri, Cristiano; Barbati, Cristiana; Vacirca, Davide; Piscopo, Paola; Confaloni, Annamaria; Sanchez, Massimo; Maselli, Angela; Colasanti, Tania; Conti, Fabrizio; Truglia, Simona; Perl, Andras; Valesini, Guido; Malorni, Walter; Ortona, Elena; Pierdominici, Marina

    2012-01-01

    Autophagy, the cytoprotection mechanism that takes place under metabolic impairment, has been implicated in the pathogenesis of autoimmunity. Here, we investigated the spontaneous and induced autophagic behavior of T lymphocytes from patients with systemic lupus erythematosus (SLE) compared with that of T lymphocytes from healthy donors by measuring the autophagy marker microtubule-associated protein 1 light chain 3 (LC3)-II. No significant differences in spontaneous autophagy were found between T lymphocytes from patients with SLE and from healthy donors, apart from CD4+ naive T cells from patients with SLE in which constitutively higher levels of autophagy (P<0.001) were detected. At variance, whereas treatment of T lymphocytes from healthy donors with serum IgG from patients with SLE resulted in a 2-fold increase in LC3-II levels (P<0.001), T lymphocytes from SLE patients were resistant to autophagic induction and also displayed an up-regulation of genes negatively regulating autophagy, e.g., α-synuclein. These findings could open new perspectives in the search for pathogenetic determinants of SLE progression and in the development of therapeutic strategies aimed to recover T-cell compartment homeostasis by restoring autophagic susceptibility.—Alessandri, C., Barbati, C., Vacirca, D., Piscopo, P., Confaloni, A., Sanchez, M., Maselli, A., Colasanti, T., Conti, F., Truglia, S., Perl, A., Valesini, G., Malorni, W., Ortona, E., Pierdominici, M. T lymphocytes from patients with systemic lupus erythematosus are resistant to induction of autophagy. PMID:22835828

  3. Predisposition to Cervical Atypia in Systemic Lupus Erythematosus: A Clinical and Cytopathological Study

    PubMed Central

    Al-Sherbeni, Hend Hilal; Fahmy, Ahmed Mohamed; Sherif, Nadine

    2015-01-01

    Introduction. Systemic lupus erythematosus (SLE) is a complex disease with variable presentations, course, and prognosis. The female genital tract may be a potential target organ in SLE since cervical inflammation may be associated with disease activity. An increase in cervical dysplasia, a precursor of cervical cancer, has been reported in females with SLE. Aim of the Work. This work aimed to study the prevalence of abnormal cervicovaginal smears in patients with systemic lupus erythematosus (SLE) and to correlate abnormal smear findings with exposure to infection with human papilloma virus (HPV) in SLE patients. Patients and Methods. Thirty-two patients with SLE, fulfilling the 1997 revised criteria for the classification of SLE, were included in this study. They were subjected to full history taking, clinical examination, laboratory investigations, and cervicovaginal smearing. Twenty healthy subjects not known to suffer from any rheumatological disease were used as controls, and they were subjected to cervicovaginal smearing. Results. Four out of 32 SLE patients showed abnormal Pap smears (12.5%) compared to none showing any cervical changes in the control group (0%). Among these 4 patients, 3 were having ASCU and one was having LSIL (HPV). Conclusion. Cervicovaginal smearing is an easy, economic, safe, repeatable, and noninvasive technique for screening and early detection of cervical neoplastic lesions in SLE. PMID:26240757

  4. Update on the use of topical calcineurin inhibitors in cutaneous lupus erythematosus

    PubMed Central

    Sticherling, Michael

    2011-01-01

    Cutaneous manifestations of lupus erythematosus (CLE) are manifold, presenting with unspecific skin manifestations or well-defined clinical dermatological entities. Their relation to each other as well as to systemic lupus erythematosus is variable, yet diagnostically and therapeutically challenging. Therapeutic decisions have to be based on the activity and distribution as well as the type of skin lesions and the extent of systemic disease. Limited skin manifestations may be amply tackled by topical therapy, so far, mainly relying on corticosteroids. In many cases, however, internal treatment has to be combined by using antimalarials, in addition to strict UV-protection. The advent of topical calcineurin inhibitors has contributed substantially to the armamentarium of external treatment options. By specifically interfering with intracytoplasmic signal transduction to activate the nuclear factor of activated T-cells (NF-AT), they are able to modulate various inflammatory mechanisms. The two available compounds, pimecrolimus and tacrolimus, do not induce the skin atrophy characteristic of corticosteroids. They have been studied in a number of case reports, but only in a few randomized, comparative studies. Both are well-tolerated, but differentially effective in the various subsets of CLE. Further studies are needed to directly compare the two compounds to each other, as well as to topical corticosteroids, before final recommendations can be made. PMID:21383913

  5. Neuropsychiatric Features of a Cohort of Patients with Systemic Lupus Erythematosus

    PubMed Central

    Moraes-Fontes, Maria Francisca; Lúcio, Isabel; Santos, Céu; Campos, Maria Manuel; Riso, Nuno; Vaz Riscado, Manuel

    2012-01-01

    In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations. The NPSLE and non-NPSLE groups were similar with regard to demographic characteristics, ACR criteria, serum autoantibodies, and frequency of hypertension and hypercholesterolemia. Of note, compared to the non-NPSLE group, NPSLE was associated with a higher frequency of smoking (78 versus 26%), organ damage (73 versus 34%), and cumulative mortality rate (14 versus 7%). The series of patients was further analysed according to the presence of antiphospholipid syndrome (APS). Significantly, the interval between the onset of NP disease and SLE diagnosis was shorter in the APS− (0.3 ± 1 years) than in the APS+ (5 ± 7 years) groups. Recurrence and/or persistence of NP events were only documented in the APS− group. Overall cumulative mortality was highest in NPSLE and in APS+ patients with inadequate anticoagulation control, identifying an aspect that requires improved vigilance and the development of novel therapeutic modalities. PMID:23227358

  6. IgE and non-IgE mediated allergic disorders in systemic lupus erythematosus

    PubMed Central

    Morton, S; Palmer, B; Muir, K; Powell, R

    1998-01-01

    OBJECTIVE—To ascertain the prevalence of IgE and non-IgE mediated allergic disorders in patients with systemic lupus erythematosus (SLE).
METHODS—49 SLE cases (all satisfying at least four "Revised ARA Criteria") and 98 healthy, age, and sex matched controls (randomly selected through two urban general practices and one rural general practice) were interviewed by telephone to screen for a history of allergy. Subjects with a history of allergic rhinitis, asthma or atopic eczema then underwent skin prick testing to confirm underlying IgE mediated disease.
RESULTS—Analysis of the data by conditional logistic regression revealed no significant difference in frequency of allergic disorders in SLE cases and controls (odds ratio (OR) 0.92, 95% confidence intervals (CI) 0.45, 1.86). In addition a subgroup analysis of subjects with IgE mediated/associated atopic disorders, showed that cases and controls were at a similar risk of having these conditions (OR 0.90, 95% CI 0.41, 1.96).
CONCLUSIONS—This study suggests that people with SLE are not at an increased risk of IgE mediated/associated allergic disorders, in contrast with previous reports.

 Keywords: systemic lupus erythematosus; allergy PMID:9924207

  7. Separation of Circulating MicroRNAs Using Apheresis in Patients With Systemic Lupus Erythematosus.

    PubMed

    Kusaoi, Makio; Yamaji, Ken; Ishibe, Yusuke; Murayama, Go; Nemoto, Takuya; Sekiya, Fumio; Kon, Takayuki; Ogasawara, Michihiro; Kempe, Kazuo; Tamura, Naoto; Takasaki, Yoshinari

    2016-08-01

    MicroRNAs (miRNAs), which are important inhibitors of mRNA translation, participate in differentiation, migration, cell proliferation, and cell death. The pathology of miRNAs results in alterations in protein expression. Recently, miRNAs circulating in peripheral blood have been shown to control the synthesis and translation of proteins at distal sites after intake into local cells. A number of studies are currently being conducted to investigate how to use miRNAs in disease treatment, but no studies have attempted to alleviate disease by directly eliminating miRNAs from blood. Therefore, we examined whether the removal or reduction of circulating miRNAs with apheresis improved pathologies caused by miRNAs. After approval of the study by our medical school's ethics committee, we collected blood and separated plasma samples from three patients with systemic lupus erythematosus who were undergoing plasmapheresis at our hospital. Peripheral blood was collected before and after it was passed through a primary membrane, centrifuged, and used to extract circulating miRNAs. A comprehensive expression analysis was then performed with a miRNA array chip. The levels of expression of a large number of circulating miRNAs were measured in the plasma samples separated by the primary membranes from all 3 patients with systemic lupus erythematosus. We present the first report that circulating miRNAs in peripheral blood can be separated and possibly directly removed using membrane separation apheresis. PMID:27523074

  8. Use of rituximab as a treatment for systemic lupus erythematosus: retrospective review

    PubMed Central

    Machado, Roberta Ismael Lacerda; Scheinberg, Morton Aaron; de Queiroz, Maria Yvone Carlos Formiga; de Brito, Danielle Christinne Soares Egypto; Guimarães, Maria Fernanda Brandao de Resende; Giovelli, Raquel Altoé; Freire, Eutilia Andrade Medeiros

    2014-01-01

    ABSTRACT Objective: To report the experience in three Brazilian institutions with the use of rituximab in patients with different clinical forms of lupus erythematosus systemic in activity. Methods: The study consisted of a sample of 17 patients with LES, who were already being treated, but that at some stage of the disease showed refractory symptoms. The patients were subdivided into groups according to the clinical manifestation, and the responses for the use of rituximab were rated as complete, partial or no response. Data were collected through a spreadsheet, and used specific parameters for each group. The treatment was carried on by using therapeutic dose of 1g, and repeating the infusion within an interval of 15 days. Results: The clinical responses to rituximab of the group only hematological and of the group only osteoarticular were complete in all cases. In the renal group there was a clinical complete response, two partial and one absent. In the renal and hematological group complete response, there was one death and a missing response. The pulmonary group presented a complete response and two partial. Conclusion: The present study demonstrated that rituximab can bring benefits to patients with lupus erythematosus systemic, with good tolerability and mild side effects; it presented, however, variable response according to the system affected. PMID:24728244

  9. The CLASI (Cutaneous LE Disease Area and Severity Index): an outcome instrument for cutaneous lupus erythematosus

    PubMed Central

    Albrecht, Joerg; Taylor, Lynne; Berlin, Jesse A.; Dulay, Samuel; Ang, Gina; Fakharzadeh, Steven; Kantor, Jonathan; Kim, Ellen; Militello, Giuseppe; McGinnis, Karen; Richardson, Stephen; Treat, James; Vittorio, Carmela; Van Voorhees, Abby; Werth, Victoria P.

    2013-01-01

    We developed and validated a measurement instrument (CLASI - Cutaneous Lupus Erythematosus Disease Area and Severity Index) for lupus erythematosus that could be used in clinical trials. The instrument has separate scores for damage and activity. A group of 7 American Dermato-Rheumatologists and the “American College of Rheumatology Response Criteria Committee on SLE” assessed content validity. After a preliminary session, we conducted standardized interviews with the raters and made slight changes to the instrument. The final instrument was evaluated by 5 dermatologists and 6 residents who scored 9 patients to estimate inter-rater reliability and intra-rater reliability in two sessions. Consultation with experts has established content validity of the instrument. Reliability studies demonstrated an Intraclass Correlation Coefficient (ICC) for inter-rater reliability of 0.86 for the activity score (95% Confidence Interval [CI] 0.73 to 0.99) and of 0.92 for the damage score (95% CI 0.85 to 1.00). The Spearman’s Rho for intra-rater reliability for the activity score was 0.96 (95% CI 0.89 to 1.00) and for the damage score Spearman’s Rho was 0.99 (95% CI 0.97 to 1.00). Clinical responsiveness needs to be evaluated in a prospective clinical trial, which is ongoing. PMID:16297185

  10. CXCL13 as a new biomarker of systemic lupus erythematosus and lupus nephritis - from bench to bedside?

    PubMed

    Schiffer, L; Worthmann, K; Haller, H; Schiffer, M

    2015-01-01

    Different studies over the last decade have linked the B cell-attracting chemokine CXC ligand 13 (CXCL13) to the autoimmune disease systemic lupus erythematosus (SLE). A pathogenetic role of this chemokine for disease manifestation in SLE was described initially in mouse models for SLE. Mechanisms of CXCL13 actions were also identified in SLE patients. Moreover, various clinical studies have identified CXCL13 serum levels as a useful biomarker in patients with SLE of different ethnicities for disease activity. In addition, CXCL13 seems to be a promising marker for the diagnosis of lupus nephritis, one of the most severe complications of SLE. However, its exact place within the mechanisms that lead to SLE remains to be defined. Further research is needed to resolve more details of the pathomechanism and the signalling pathway of CXCL13 in SLE. Blocking CXCL13 or the signal pathways of CXCL13 is seen as a promising therapeutic approach for SLE and will be addressed in the near future. This review summarizes all papers that linked CXCL13 to SLE and highlights its importance in the pathogenesis and diagnosis of SLE.

  11. Altered glycosylation of complexed native IgG molecules is associated with disease activity of systemic lupus erythematosus.

    PubMed

    Sjöwall, C; Zapf, J; von Löhneysen, S; Magorivska, I; Biermann, M; Janko, C; Winkler, S; Bilyy, R; Schett, G; Herrmann, M; Muñoz, L E

    2015-05-01

    In addition to the redundancy of the receptors for the Fc portion of immunoglobulins, glycans result in potential ligands for a plethora of lectin receptors found in immune effector cells. Here we analysed the exposure of glycans containing fucosyl residues and the fucosylated tri-mannose N-type core by complexed native IgG in longitudinal serum samples of well-characterized patients with systemic lupus erythematosus. Consecutive serum samples of a cohort of 15 patients with systemic lupus erythematosus during periods of increased disease activity and remission were analysed. All patients fulfilled the 1982 American College of Rheumatology classification criteria. Sera of 15 sex- and age-matched normal healthy blood donors served as controls. The levels and type of glycosylation of complexed random IgG was measured with lectin enzyme-immunosorbent assays. After specifically gathering IgG complexes from sera, biotinylated lectins Aleuria aurantia lectin and Lens culinaris agglutinin were employed to detect IgG-associated fucosyl residues and the fucosylated tri-mannose N-glycan core, respectively. In sandwich-ELISAs, IgG-associated IgM, IgA, C1q, C3c and C-reactive protein (CRP) were detected as candidates for IgG immune complex constituents. We studied associations of the glycan of complexed IgG and disease activity according to the physician's global assessment of disease activity and the systemic lupus erythematosus disease activity index 2000 documented at the moment of blood taking. Our results showed significantly higher levels of Aleuria aurantia lectin and Lens culinaris agglutinin binding sites exposed on IgG complexes of patients with systemic lupus erythematosus than on those of normal healthy blood donors. Disease activity in systemic lupus erythematosus correlated with higher exposure of Aleuria aurantia lectin-reactive fucosyl residues by immobilized IgG complexes. Top levels of Aleuria aurantia lectin-reactivity were found in samples taken during the

  12. [Psychic changes in systemic lupus erythematosus: a multidisciplinary prospective study].

    PubMed

    Miguel Filho, E C; Pereira, R M; Busatto Filho, G; Shavitt, R G; Hirsch, R; de Sá, L C; de Arruda, P C

    1990-01-01

    Despite the high prevalence of psychic symptoms in lupus patients, there are few systematic studies in this area. Through a multidisciplinary approach, the authors developed a prospective study to characterize and correlate psychopathological aspects with clinical and laboratory data concerning neural manifestations of the disease. Out of 23 patients studied, 12 showed psychic alterations, which were interpreted as primary manifestations of the disease. All of them presented organic mental syndromes (DSM-III-R) in which cognitive symptoms were the most prominent, followed by affective, catatonic and hallucinatory features. The neurologic findings (seizure, migraine and muscular atrophy), as well as the ophthalmologic alterations (hemorrhage and soft exudates) were frequent and concomitant with the psychic features. The laboratory findings were: LE cells 50%; anti-Sm: 16%; anti-U1 RNP: 50%; anti-Ro/SS-A: 50%; anti-nDNA: 58%; decreased CH50 or fractions (C3, C4): 67%; anti-P: 18%; antigangliosides IgG: 67%; antigangliosides IgM: 78%. The cerebrospinal fluid analysis showed: increased cellularity: 18%; elevated protein: 36%; antigangliosides IgG: 67%; antigangliosides IgM: 33%; immunocomplexes: 36%. In spite of the absence of an adequate control group and of the small number of patients, the multidisciplinary approach leads to a better characterization of the nervous system involvement in this disease. PMID:1965671

  13. The apoptosis-1/Fas protein in human systemic lupus erythematosus.

    PubMed Central

    Mysler, E; Bini, P; Drappa, J; Ramos, P; Friedman, S M; Krammer, P H; Elkon, K B

    1994-01-01

    Three independent mutations involving the apoptosis-1 (APO-1)/Fas receptor or its putative ligand have led to lupuslike diseases associated with lymphadenopathy in different strains of mice. To determine whether humans with SLE also have a defect in this apotosis pathway, we analyzed the expression of APO-1 on freshly isolated blood mononuclear cells and on lymphocytes activated in vitro using flow cytometry and the monoclonal antibody anti-APO-1. Significantly higher level of APO-1 expression were detected on freshly isolated peripheral B cells and both CD4+ and CD8+ T lymphocyte populations obtained from lupus patients when compared with normal controls (P < 0.001). Almost 90% of the cells that stained positive for APO-1 also expressed the CD29 antigen, suggesting that APO-1 was upregulated after lymphocyte activation in vivo. No defect in APO-1 regulation was detected after activation of SLE T (with anti-CD3) or B (with Staphylococcus aureus Cowan 1) lymphocytes in the presence of IL-2 in vitro. Similarly, the anti-APO-1 antibody induced apoptosis in 74 +/- 5% of activated SLE T cells in vitro compared with 79 +/- 6% of the normal controls (P > 0.05). These results reveal that, while APO-1/Fas may play an important role in the regulation of lymphocyte survival in SLE, no consistent defect in the expression or function of the receptor could be detected in these studies. Images PMID:7510716

  14. Pediatric systemic lupus erythematosus: more than a positive antinuclear antibody.

    PubMed

    Weiss, Jennifer E

    2012-02-01

    Based on strong research evidence and consensus, the most common disease manifestations at diagnosis of pSLE are constitutional symptoms, arthritis, and malar rash. Based on some research evidence and consensus, patients with pSLE tend to have major organ system involvement (renal/central nervous system) and a greater disease burden compared with adults. Despite these findings, mortality is low. Based on some research evidence and consensus, the diagnosis of pSLE is unlikely if the ANA is negative, and most patients with SLE have a positive ANA at a titer ≥1:160. Based on strong research evidence, both MMF and cyclophosphamide can be used for induction therapy in class III and IV lupus nephritis. Based on strong research evidence, patients with SLE and anticardiolipin antibodies or LA have a two and six times greater risk of venous thrombosis, respectively, compared with patients with SLE without antiphospholipid antibodies. Based on strong research evidence, patients with pSLE have a higher risk for subclinical atherosclerosis when there is weight-adjusted prednisone use, azathioprine use, increasing age, male gender, high BMI, abnormal creatinine clearance, and elevated lipoprotein(a) levels.

  15. Symptomatic knee osteonecrosis in patients with systemic lupus erythematosus: a case-control study.

    PubMed

    Zhao, Lidan; Wu, Xiuhua; Wu, Honghua; Su, Jinmei; Zhang, Wen; Zhao, Yan; Zhang, Xuan; Zheng, Wenjie

    2016-08-01

    To explore the associated risk factors of symptomatic knee osteonecrosis (KON) in patients with systemic lupus erythematosus (SLE), we conducted a retrospective case-control study to compare the clinical and laboratory features between SLE patients with and without symptomatic KON matched by age and gender. Univariate and multivariate regression analyses were used to evaluate possible associated risk factors. Twenty (one male, nineteen females) out of 3941 lupus patients were identified as symptomatic KON, which was confirmed by magnetic resonance imaging. The mean age at KON onset was 34.4 (range 12-67) years, and the median course of lupus at KON onset was 72.5 (range 8-123) months. Univariate and multivariate analyses identified that the prevalence of cutaneous vasculitis (OR 5.23; 95 % CI 1.11-24.70), hyperfibrinogenemia (OR 4.75; 95 % CI 1.08-20.85), and elevated IgG levels (OR 6.05; 95 % CI 1.58-23.16) were statistically higher in KON group, and hydroxychloroquine (HCQ) usage was statistically lower in KON group (OR 0.27; 95 % CI 0.07-0.97). Glucocorticoid usage, in terms of maximal dose, duration of treatment, and the percentage of receiving methylprednisolone pulse therapy, did not show statistical difference between the two groups (p > 0.05). Symptomatic KON is a relatively rare complication of SLE. Cutaneous vasculitis, hyperfibrinogenemia, and elevated IgG levels are possible risk factors, whereas HCQ may provide a protective effect. Our results suggest that lupus activity as well as hypercoagulation status may play a role in the pathogenesis of KON in lupus. PMID:27230994

  16. Resveratrol counters systemic lupus erythematosus-associated atherogenicity by normalizing cholesterol efflux.

    PubMed

    Voloshyna, Iryna; Teboul, Isaac; Littlefield, Michael J; Siegart, Nicolle M; Turi, George K; Fazzari, Melissa J; Carsons, Steven E; DeLeon, Joshua; Reiss, Allison B

    2016-08-01

    Resveratrol is a bioactive molecule used in dietary supplements and herbal medicines and consumed worldwide. Numerous investigations by our group and others have indicated cardioprotective and anti-inflammatory properties of resveratrol. The present study explored potential atheroprotective actions of resveratrol on cholesterol efflux in cultured human macrophages exposed to plasma from systemic lupus erythematosus (SLE) patients. These results were confirmed in ApoE(-/-)Fas(-/-) double knockout mice, displaying a lupus profile with accelerated atherosclerosis. Resveratrol treatment attenuated atherosclerosis in these mice. THP-1 human macrophages were exposed to 10% pooled or individual plasma from patients who met diagnostic criteria for SLE. Expression of multiple proteins involved in reverse cholesterol transport (ABCA1, ABCG1, SR-B1, and cytochrome P450 27-hydroxylase) was assessed using QRT-PCR and Western blotting techniques. Ten-week-old ApoE(-/-)Fas(-/-) double knockout mice (n = 30) were randomly divided into two equal groups of 15, one of which received 0.01% resveratrol for 10 consecutive weeks. Atherosclerosis progression was evaluated in murine aortas. Bone marrow-derived macrophages (BMDM) were cultured and expression of cholesterol efflux proteins was analyzed in each group of mice. Our data indicate that inhibition of cholesterol efflux by lupus plasma in THP-1 human macrophages is rescued by resveratrol. Similarly, administration of resveratrol in a lupus-like murine model reduces plaque formation in vivo and augments cholesterol efflux in BMDM. This study presents evidence for a beneficial role of resveratrol in atherosclerosis in the specific setting of SLE. Therefore, resveratrol may merit investigation as an additional resource available to reduce lipid deposition and atherosclerosis in humans, especially in such vulnerable populations as lupus patients. PMID:27190277

  17. Inflammasome activation of IL-18 results in endothelial progenitor cell dysfunction in systemic lupus erythematosus.

    PubMed

    Kahlenberg, J Michelle; Thacker, Seth G; Berthier, Celine C; Cohen, Clemens D; Kretzler, Matthias; Kaplan, Mariana J

    2011-12-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with heterogeneous manifestations including severe organ damage and vascular dysfunction leading to premature atherosclerosis. IFN-α has been proposed to have an important role in the development of lupus and lupus-related cardiovascular disease, partly by repression of IL-1 pathways leading to impairments in vascular repair induced by endothelial progenitor cells (EPCs) and circulating angiogenic cells (CACs). Counterintuitively, SLE patients also display transcriptional upregulation of the IL-1β/IL-18 processing machinery, the inflammasome. To understand this dichotomy and its impact on SLE-related cardiovascular disease, we examined cultures of human and murine control or lupus EPC/CACs to determine the role of the inflammasome in endothelial differentiation. We show that caspase-1 inhibition improves dysfunctional SLE EPC/CAC differentiation into mature endothelial cells and blocks IFN-α-mediated repression of this differentiation, implicating inflammasome activation as a crucial downstream pathway leading to aberrant vasculogenesis. Furthermore, serum IL-18 levels are elevated in SLE and correlate with EPC/CAC dysfunction. Exogenous IL-18 inhibits endothelial differentiation in control EPC/CACs and neutralization of IL-18 in SLE EPC/CAC cultures restores their capacity to differentiate into mature endothelial cells, supporting a deleterious effect of IL-18 on vascular repair in vivo. Upregulation of the inflammasome machinery was operational in vivo, as evidenced by gene array analysis of lupus nephritis biopsies. Thus, the effects of IFN-α are complex and contribute to an elevated risk of cardiovascular disease by suppression of IL-1β pathways and by upregulation of the inflammasome machinery and potentiation of IL-18 activation.

  18. Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature.

    PubMed

    Borrell, Helena; Narváez, Javier; Alegre, Juan José; Castellví, Ivan; Mitjavila, Francesca; Aparicio, María; Armengol, Eulàlia; Molina-Molina, María; Nolla, Joan M

    2016-08-01

    Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate

  19. Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature.

    PubMed

    Borrell, Helena; Narváez, Javier; Alegre, Juan José; Castellví, Ivan; Mitjavila, Francesca; Aparicio, María; Armengol, Eulàlia; Molina-Molina, María; Nolla, Joan M

    2016-08-01

    Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate

  20. Mercury in Hair Is Inversely Related to Disease Associated Damage in Systemic Lupus Erythematosus.

    PubMed

    Crowe, William; Doherty, Leanne; Watson, Gene; Armstrong, David; Ball, Elisabeth; Magee, Pamela; Allsopp, Philip; Bell, Aubrey; Strain, J J; McSorley, Emeir

    2015-12-23

    Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease, and environmental factors are proposed to exacerbate existing symptoms. One such environmental factor is mercury. The aim of this study was to investigate the relationship between exposure to mercury (Hg) and disease activity and disease associated damage in Total Hg concentrations in hair and urine were measured in 52 SLE patients. Dental amalgams were quantified. Disease activity was assessed using three indexes including the British Isles Lupus Assessment Group Index (BILAG). Disease associated damage was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology SLICC/ACR Damage Index. Pearson's correlation identified a significant negative correlation between hair Hg and BILAG (r = -0.323, p = 0.029) and SLICC/ACR (r = -0.377, p = 0.038). Multiple regression analysis identified hair Hg as a significant predictor of disease associated damage as determined by SLICC/ACR (β = -0.366, 95% confidence interval (CI): -1.769, -0.155 p = 0.019). Urinary Hg was not related to disease activity or damage. Fish consumption is the primary route of MeHg exposure in humans and the inverse association of hair Hg with disease activity observed here might be explained by the anti-inflammatory effects of n-3 long chain polyunsaturated fatty acids also found in fish.

  1. Mercury in Hair Is Inversely Related to Disease Associated Damage in Systemic Lupus Erythematosus

    PubMed Central

    Crowe, William; Doherty, Leanne; Watson, Gene; Armstrong, David; Ball, Elisabeth; Magee, Pamela; Allsopp, Philip; Bell, Aubrey; Strain, J. J.; McSorley, Emeir

    2015-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease, and environmental factors are proposed to exacerbate existing symptoms. One such environmental factor is mercury. The aim of this study was to investigate the relationship between exposure to mercury (Hg) and disease activity and disease associated damage in Total Hg concentrations in hair and urine were measured in 52 SLE patients. Dental amalgams were quantified. Disease activity was assessed using three indexes including the British Isles Lupus Assessment Group Index (BILAG). Disease associated damage was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology SLICC/ACR Damage Index. Pearson’s correlation identified a significant negative correlation between hair Hg and BILAG (r = −0.323, p = 0.029) and SLICC/ACR (r = −0.377, p = 0.038). Multiple regression analysis identified hair Hg as a significant predictor of disease associated damage as determined by SLICC/ACR (β = −0.366, 95% confidence interval (CI): −1.769, −0.155 p = 0.019). Urinary Hg was not related to disease activity or damage. Fish consumption is the primary route of MeHg exposure in humans and the inverse association of hair Hg with disease activity observed here might be explained by the anti-inflammatory effects of n-3 long chain polyunsaturated fatty acids also found in fish. PMID:26703710

  2. Impact of systemic lupus erythematosus on burden of illness and work productivity in the United States.

    PubMed

    Garris, C; Oglesby, A; Sulcs, E; Lee, M

    2013-09-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by clinical manifestations that can cause diminished activity and productivity. The objectives of this study were to: (a) longitudinally evaluate patient-reported SLE disease activity, and (b) measure work productivity, missed work hours, and associated lost income among employed patients with SLE. Three cohorts (employed subjects with SLE (n = 281), nonemployed subjects with SLE (n = 265), and a control group of employed individuals without SLE (n = 300)) completed a baseline survey. Employed subjects with SLE completed follow-up surveys every two weeks during a six-month period. Measured outcomes included perceived health, disease manifestations and severity, the Lupus Impact Tracker, the Modified Systemic Lupus Activity Questionnaire, and Work Productivity and Activity Impairment Questionnaire. Higher self-reported SLE disease severity was directly associated with experiencing more frequent and more severe symptoms as well as higher levels of lost work time and lost work productivity. Though patient self-assessment may differ from physician's clinical assessment, it is important to incorporate the patient perspective in clinical decision-making to optimally manage SLE patients. Given the evidence associating SLE with work disability and job loss, it may be beneficial for professionals addressing worksite modifications or compensatory strategies to be included as members of SLE medical teams.

  3. Alternatively activated dendritic cells derived from systemic lupus erythematosus patients have tolerogenic phenotype and function.

    PubMed

    Wu, Hai Jing; Lo, Yi; Luk, Daniel; Lau, Chak Sing; Lu, Liwei; Mok, Mo Yin

    2015-01-01

    Tolerogenic dendritic cells (DCs) are potential cell-based therapy in autoimmune diseases. In this study, we generated alternatively activated DCs (aaDCs) by treating monocyte-derived DCs from patients with systemic lupus erythematosus (SLE) and healthy subjects with combination of 1,25 dihydroxyvitamin D(3) (vitD3) and dexamethasone followed by lipopolysaccharide-induced maturation. Lupus aaDCs were found to acquire semi-mature phenotype that remained maturation-resistant to immunostimulants. They produced low level of IL-12 but high level of IL-10. They had attenuated allostimulatory effects on T cell activation and proliferation comparable to normal aaDCs and demonstrated differential immunomodulatory effects on naïve and memory T cells. These aaDCs were capable of inducing IL-10 producing regulatory T effectors from naïve T cells whereas they modulated cytokine profile with suppressed production of IFN-γ and IL-17 by co-cultured memory T cells with attenuated proliferation. These aaDCs were shown to be superior to those generated using vitD3 alone in lupus patients.

  4. Tolerogenic dendritic cells: role and therapeutic implications in systemic lupus erythematosus.

    PubMed

    Mok, Mo Yin

    2015-02-01

    Dendritic cells (DCs) are antigen presenting cells that activate T cells and determine the outcome of immune response. In addition to their important function in defense against pathogens, DCs are increasingly recognized as playing a crucial role in the regulation of immune tolerance. Plasticity of DCs with different maturity status and functions enable them to be exploited as potential cell-based therapy to restore immune tolerance in autoimmune diseases. Various ex vivo methods have been developed to generate stable tolerogenic DCs that are able to induce and maintain regulatory T cell homeostasis. The beneficial effect of tolerogenic DCs have been studied in murine autoimmune models with promising results. Systemic lupus erythematosus (SLE) is a prototypic multi-systemic autoimmune disease characterized by autoantibody production and deposition of immune complexes in organs. There are evidences that dysregulated DCs play a pivotal role in the initiation and perpetuation of lupus disease. Peripheral blood monocytes in SLE patients were found to have active phenotype with accelerated differentiation into DCs efficient in antigen presentation. Plasmacytoid DCs in SLE patients produce high levels of interferon-alpha, the signature cytokine of this disease, that cause a positive feedback loop in the amplification of activation of innate and adaptive immunity. Furthermore, manipulation of DCs via toll-like receptor knockout in a murine lupus model leads to alteration in disease severity and survival. Thus, tolerogenic DCs may appear as a potential cell-based therapeutic option in SLE. PMID:25639676

  5. Netting neutrophils are major inducers of type I IFN production in pediatric systemic lupus erythematosus.

    PubMed

    Garcia-Romo, Gina S; Caielli, Simone; Vega, Barbara; Connolly, John; Allantaz, Florence; Xu, Zhaohui; Punaro, Marilynn; Baisch, Jeanine; Guiducci, Cristiana; Coffman, Robert L; Barrat, Franck J; Banchereau, Jacques; Pascual, Virginia

    2011-03-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by a breakdown of tolerance to nuclear antigens and the development of immune complexes. Genomic approaches have shown that human SLE leukocytes homogeneously express type I interferon (IFN)-induced and neutrophil-related transcripts. Increased production and/or bioavailability of IFN-α and associated alterations in dendritic cell (DC) homeostasis have been linked to lupus pathogenesis. Although neutrophils have long been shown to be associated with lupus, their potential role in disease pathogenesis remains elusive. Here, we show that mature SLE neutrophils are primed in vivo by type I IFN and die upon exposure to SLE-derived anti-ribonucleoprotein antibodies, releasing neutrophil extracellular traps (NETs). SLE NETs contain DNA as well as large amounts of LL37 and HMGB1, neutrophil proteins that facilitate the uptake and recognition of mammalian DNA by plasmacytoid DCs (pDCs). Indeed, SLE NETs activate pDCs to produce high levels of IFN-α in a DNA- and TLR9 (Toll-like receptor 9)-dependent manner. Our results reveal an unsuspected role for neutrophils in SLE pathogenesis and identify a novel link between nucleic acid-recognizing antibodies and type I IFN production in this disease.

  6. Is there any correlation between high sensitive CRP and disease activity in systemic lupus erythematosus?

    PubMed

    Rezaieyazdi, Z; Sahebari, M; Hatef, M R; Abbasi, B; Rafatpanah, H; Afshari, J Tavakol; Esmaily, H

    2011-12-01

    The role of C-reactive protein (CRP) in systemic lupus erythematosus (SLE) as an inflammatory marker is still controversial. Recently, more sensitive methods, such as high sensitive CRP (hs-CRP) have been used to detect micro-inflammation. The role of hs-CRP in lupus flare has not been documented well. We conducted this study to examine the correlation between hs-CRP serum concentrations and disease activity in lupus. Ninety-two SLE patients and 49 healthy controls contributed to our study. Most confounding factors influencing the hs-CRP values were excluded. Disease activity was estimated using the SLE Disease Activity Index (SLEDAI-2K). hs-CRP values were determined using an enzyme-linked immunosorbent assay (ELISA) kit. Serum values of hs-CRP were significantly higher (p < 0.001, z = 3.29) in patients compared with healthy controls. The cutoff point for hs-CRP between patients and controls was 0.93 mg/L (Youden's Index = 0.39). There was no correlation between hs-CRP serum levels and disease activity. Furthermore, hs-CRP values did not correlate with any of the laboratory parameters, except for C3 (p = 0.003, r(s)  = -0.2) and C4 (p = 0.02, r(s) = -0.1). Although hs-CRP serum levels were significantly higher in lupus patients compared with healthy controls, it seems that this marker is not a good indicator for disease activity.

  7. Socioeconomic status and organ damage in Mexican systemic lupus erythematosus women.

    PubMed

    Mendoza-Pinto, C; Méndez-Martínez, S; Soto-Santillán, P; Galindo Herrera, J; Pérez-Contreras, I; Macías-Díaz, S; Taboada-Cole, A; García-Carrasco, M

    2015-10-01

    The objective of this cross-sectional study was to determine relationships between socioeconomic status and organ damage in Mexican systemic lupus erythematosus (SLE) patients. Demographic and clinical variables were assessed. Socioeconomic status was evaluated using the Graffar method and monthly household income. Lupus activity and organ damage were measured using the SLE disease activity scale, validated for the Mexican population (Mex-SLEDAI), and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) scale. The 143 Mexican female SLE patients included (mean age 40.1 ± 8.9 years, mean disease duration 8.9 ± 6.3 years) had a mean monthly household income of $ 407.2 ± 326.5. According to the Graffar index, 18.9%, 52.5%, and 28.7% had high/medium-high, medium, and medium-low/low socioeconomic status, respectively. Organ damage was observed in 61 patients (42.7%). Patients with organ damage had lower monthly household incomes ($241.4 ± 152.4 vs. $354.8 ± 288.3) and were more frequently unemployed (57.3% vs. 35.3%; p = 0.01) than those without. Low monthly income was not associated with lupus activity or self-reported health status. In the adjusted multivariate analysis, low monthly income ( < $300) was associated with organ damage. In conclusion, low income may be associated with organ damage in Mexican SLE patients.

  8. Molecular Basis of 9G4 B Cell Autoreactivity in Human Systemic Lupus Erythematosus

    PubMed Central

    Richardson, Christopher; Chida, Asiya Seema; Adlowitz, Diana; Silver, Lin; Fox, Erin; Jenks, Scott A.; Palmer, Elise; Wang, Youliang; Heimburg-Molinaro, Jamie; Li, Quan-Zhen; Mohan, Chandra; Cummings, Richard; Tipton, Christopher

    2013-01-01

    9G4+ IgG Abs expand in systemic lupus erythematosus (SLE) in a disease-specific fashion and react with different lupus Ags including B cell Ags and apoptotic cells. Their shared use of VH4-34 represents a unique system to understand the molecular basis of lupus autoreactivity. In this study, a large panel of recombinant 9G4+ mAbs from single naive and memory cells was generated and tested against B cells, apoptotic cells, and other Ags. Mutagenesis eliminated the framework-1 hydrophobic patch (HP) responsible for the 9G4 idiotype. The expression of the HP in unselected VH4-34 cells was assessed by deep sequencing. We found that 9G4 Abs recognize several Ags following two distinct structural patterns. B cell binding is dependent on the HP, whereas anti-nuclear Abs, apoptotic cells, and dsDNA binding are HP independent and correlate with positively charged H chain third CDR. The majority of mutated VH4-34 memory cells retain the HP, thereby suggesting selection by Ags that require this germline structure. Our findings show that the germline-encoded HP is compulsory for the anti–B cell reactivity largely associated with 9G4 Abs in SLE but is not required for reactivity against apoptotic cells, dsDNA, chromatin, anti-nuclear Abs, or cardiolipin. Given that the lupus memory compartment contains a majority of HP+ VH4-34 cells but decreased B cell reactivity, additional HP-dependent Ags must participate in the selection of this compartment. This study represents the first analysis, to our knowledge, of VH-restricted autoreactive B cells specifically expanded in SLE and provides the foundation to understand the antigenic forces at play in this disease. PMID:24108696

  9. Lupus anticoagulant and history of thrombosis are not associated with persistent endothelial cell activation in systemic lupus erythematosus

    PubMed Central

    Frijns, C J M; Derksen, R H W M; De Groot, PH G; Algra, A; Fijnheer, R

    2001-01-01

    Antiphospholipid antibodies (aPL), especially lupus anticoagulant (LAC), characterize systemic lupus erythematosus (SLE) patients at increased risk for arterial and venous thromboembolic complications. It has been reported that purified human anti-phospholipid antibodies cause endothelial cell activation in in vitro experiments. In order to investigate whether increased endothelial cell activation is associated with thromboembolic events in SLE patients with LAC, we measured plasma levels of thrombomodulin (TM), von Willebrand factor (vWf), sP-selectin, vascular cell adhesion molecule-1 (sVCAM-1) and ED1-fibronectin in a study of 76 patients with SLE. Patients were subdivided on the basis of: no history of thrombosis and LAC-negative (n = 22) or LAC-positive (n = 17); positive history of thrombosis and LAC-negative (n = 16) or LAC-positive (n = 21). The median SLE disease activity index (SLEDAI) was 4. Although concentrations of sTM, vWf, sP-selectin and sVCAM-1 were significantly elevated in SLE compared with values in healthy controls, they did not differ between the four groups, between patients with or without history of thrombosis, and between patients with or without LAC. Presence of anticardiolipin antibodies could not explain these negative findings. Adjustment of the concentrations for significantly associated variables, such as age, hypertension, smoking, immunosuppressive treatment and concentrations of creatinine, cholesterol and homocysteine, did not change the main results of the study. Only sTM was significantly lower in patients with both LAC and thrombosis than in patients without both these features after adjustment for serum creatinine concentrations. In conclusion, we did not find an association between endothelial cell activation and presence of LAC or history of thrombosis in SLE. PMID:11472438

  10. Generalized Lymphadenopathy as Presenting Feature of Systemic Lupus Erythematosus: Case Report and Review of the Literature

    PubMed Central

    Afzal, Wais; Arab, Talal; Ullah, Tofura; Teller, Katerina; Doshi, Kaushik J.

    2016-01-01

    Lymphadenopathy could represent a vast spectrum of etiologies including infectious and non-infectious diseases. Besides proper history taking, physical examination, and laboratory investigations, a tissue diagnosis is often necessary to unmask the cause of generalized lymphadenopathy. Here we present a 23-year-old woman who was admitted for diffuse generalized lymphadenopathy, fatigue, malaise, weight loss, nausea, and bilateral lower extremity edema. She reported a history of seizures as well as stroke 2 years prior with no other medical conditions present. Although malignant and infectious etiologies were initially the primary targets for workup, her history of seizures and stroke remained a dilemma. Extensive workup for malignant and infectious diseases was unrevealing; however, rheumatologic workup was eventually positive for systemic lupus erythematosus (SLE). This case illustrates that extensive generalized diffuse lymphadenopathy may be a presenting feature of SLE and should be considered in the differential diagnosis of patients presenting with diffuse lymphadenopathy and constitutional symptoms. PMID:27738484

  11. Strongyloidiasis Hyperinfection in a Patient with a History of Systemic Lupus Erythematosus

    PubMed Central

    Yung, Evan E.; Lee, Cassie M. K. L.; Boys, Joshua; Grabo, Daniel J.; Buxbaum, James L.; Chandrasoma, Parakrama T.

    2014-01-01

    Strongyloidiasis is a parasitic disease caused by Strongyloides stercoralis, a nematode predominately endemic to tropical and subtropical regions, such as Southeast Asia. Autoinfection enables the organism to infect the host for extended periods. Symptoms, when present, are non-specific and may initially lead to misdiagnosis, particularly if the patient has additional co-morbid conditions. Immunosuppressive states place patients at risk for the Strongyloides hyperinfection syndrome (SHS), whereby the organism rapidly proliferates and disseminates within the host. Left untreated, SHS is commonly fatal. Unfortunately, the non-specific presentation of strongyloidiasis and the hyperinfection syndrome may lead to delays in diagnosis and treatment. We describe an unusual case of SHS in a 30-year-old man with a long-standing history of systemic lupus erythematosus who underwent a partial colectomy. The diagnosis was rendered on identification of numerous organisms during histologic examination of the colectomy specimen. PMID:25092815

  12. Fasciolopsis buski infection in a Vietnamese pregnant woman with systemic lupus erythematosus.

    PubMed

    Fiamma, Maura; Longoni, Silvia Stefania; Ngo, Thi Minh Chau; Le Phan, Minh Triet; Santona, Antonella; Ton Nu, Phuong An; Paglietti, Bianca

    2015-06-01

    A clinical case of infection caused by Fasciolopsis buski in a 24 weeks pregnant woman from Vietnam affected by systemic lupus erythematosus (SLE) is reported here. On 22 February 2012 the patient was admitted to Hue Hospital in Hue, Vietnam, with a diagnosis of general illness and suspected acute anaemia. Laboratory analysis indicated possible SLE syndrome and coprological tests demonstrated the presence of F. buski eggs. During hospitalization the patient naturally eliminated the adult form in faeces suggesting the infection had already progressed at least for three months. One month after hospitalization due to the high severity of both SLE and fasciolopsiasis, a medical abortion was carried out and the following day the patient died. Even though infection due to Fasciolopsis buski is rare, this case highlights the importance of an accurate and prompt diagnosis of this infectious agent, which may have saved the foetus and mother's lives. PMID:26142680

  13. Moyamoya syndrome occurred in a girl with an inactive systemic lupus erythematosus.

    PubMed

    Lee, Yun-Jin; Yeon, Gyu Min; Nam, Sang Ook; Kim, Su Yung

    2013-12-01

    We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed stenosis of the left internal carotid artery and right anterior cerebral artery with accompanying collateral circulation (moyamoya vessels). After the patient underwent bypass surgery on the left side, she recovered from the neurological problems and did not experience any additional ischemic attack during the 14-month follow-up period. This case represents an unusual association between moyamoya syndrome and inactive SLE (inactive for a relatively long interval of 2 years) in a young girl.

  14. Occupational and environmental exposures as risk factors for systemic lupus erythematosus.

    PubMed

    Cooper, Glinda S; Parks, Christine G

    2004-10-01

    Although genetic susceptibility plays a strong role in the etiology of systemic lupus erythematosus (SLE), recent research has provided new evidence of the potential influence of environmental factors in the risk for this disease. This paper describes epidemiologic and experimental research pertaining to occupational and environmental sources of exposure to respirable crystalline silica, solvents and pesticides, and two "lifestyle" factors (smoking and hair dye use). As has been seen with other systemic autoimmune diseases (eg, systemic sclerosis and rheumatoid arthritis), a series of epidemiologic studies, using different designs in different settings, have demonstrated relatively strong and consistent associations between occupational silica exposure and SLE. The type and quality of exposure assessment is an important consideration in evaluating these studies. Recent experimental studies examined the effect of trichloroethylene exposure in MRL+/+ mice, but to date there have been few epidemiologic studies of solvents and SLE. There are numerous avenues with respect to environmental factors in SLE that need additional research.

  15. Effect of high-dose methylprednisolone therapy on phagocyte function in systemic lupus erythematosus.

    PubMed Central

    Boghossian, S H; Isenberg, D A; Wright, G; Snaith, M L; Segal, A W

    1984-01-01

    Circulating phagocytes play a major role in the defence of the host against microbial infection. In an attempt to identify the reason for the unusual susceptibility to infection of patients with systemic lupus erythematosus (SLE) various parameters of phagocytic cell function were assessed kinetically in whole blood, and the accumulation of cells in areas of inflammation was studied in vivo with the skin window technique. The effect on these parameters of conventional therapy with glucocorticoids and pulse therapy with large doses of methylprednisolone were examined. Patients on conventional doses of steroids had no abnormality of phagocyte function that might have predisposed to infection, apart from a reduced accumulation of monocytes in areas of inflammation and decreased lactoferrin secretion. Pulse therapy with methylprednisolone considerably delayed the secretion of lactoferrin and the adherence of neutrophils in most of the patients, as well as impairing bacterial killing and digestion. PMID:6383232

  16. Osteonecrosis in patients with systemic lupus erythematosus develops very early after starting high dose corticosteroid treatment

    PubMed Central

    Oinuma, K; Harada, Y; Nawata, Y; Takabayashi, K; Abe, I; Kamikawa, K; Moriya, H

    2001-01-01

    OBJECTIVES—To investigate the actual time of onset of osteonecrosis (ON) after high dose corticosteroid treatment in systemic lupus erythematosus (SLE).
METHODS—72 patients with active SLE, who received high dose corticosteroid for the first time, for the development of ON at hips and knees were monitored by magnetic resonance imaging for at least 12 months.
RESULTS—ON lesions were detected in 32/72 patients (44%) between one and five months (3.1 months on average) after starting high dose corticosteroid treatment. No osteonecrotic lesion was newly detected from the sixth month of treatment until the end of the follow up period.
CONCLUSION—The findings suggested that the actual time of onset of ON in SLE is within the first month of high dose corticosteroid treatment.

 PMID:11709458

  17. Linear cutaneous lupus erythematosus following the lines of Blaschko - Case report*

    PubMed Central

    Marinho, Ayana Karla de Oliveira Ferreira; Ramos, Ticiana Batista; Barbosa, Deborah Maria de Castro; Kozmhinsky, Valter; Takano, Daniela Mayumi; Figueira, Marcella Maria de Souza Araújo

    2016-01-01

    Chronic cutaneous lupus erythematosus in a linear configuration is rare, particularly in children, demonstrating similar incidence in both genders, no photo-sensitivity and lower probability of progression to systemic disease. We describe the case of a 9-year-old girl who presented erythematous papules with central atrophy on the upper and lower right limbs, asymptomatic and following the lines of Blaschko, since age four. Histological examination showed atrophy of the epidermis with aggression from epidermal-dermal interface and periadnexal and perivascular lymphocytic inflammatory infiltrate. Laboratory tests showed ANA in a titer of 1:320, in a dense and fine speckled pattern. Due to the rarity of presentation and location of the disease, this case is reported here. PMID:27579750

  18. Oxidative Stress and Treg and Th17 Dysfunction in Systemic Lupus Erythematosus

    PubMed Central

    Yang, Xue

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organ systems. The pathogenic mechanisms that cause SLE remain unclear; however, it is well recognized that the immune balance is disturbed and that this imbalance contributes to the autoimmune symptoms of SLE. Oxidative stress represents an imbalance between the production and manifestation of reactive oxygen species and the ability of the biological system to readily detoxify the reactive intermediates or to repair the resulting damage. In humans, oxidative stress is involved in many diseases, including atherosclerosis, myocardial infarction, and autoimmune diseases. Numerous studies have confirmed that oxidative stress plays an important role in the pathogenesis of SLE. This review mainly focuses on the recent research advances with respect to oxidative stress and regulatory T (Treg)/helper T 17 (Th17) cell dysfunction in the pathogenesis of SLE. PMID:27597882

  19. Mycobacterium kansasii infection presenting as cellulitis in a patient with systemic lupus erythematosus.

    PubMed

    Hsu, Pei-Yu; Yang, Yao-Hsu; Hsiao, Cheng-Hsiang; Lee, Ping-Ing; Chiang, Bor-Luen

    2002-08-01

    The prevalence of mycobacterial infection has increased in recent years, especially in patients immunocompromised due to autoimmune disease, malignancy and AIDS. Mycobacterium kansasii infection most commonly presents as tuberculosis-like pulmonary disease. We report the case of a 38-year-old woman with systemic lupus erythematosus (SLE) who developed cellulitis over the left lower leg and had poor response to antibiotics. Two months before this admission, she had sustained a small wound over the right pretibial area and had noticed erythematous swelling after swimming at the beach. Pathologic examination of biopsied tissue showed acid-fast bacilli, and culture yielded M. kansasii. The cellulitis improved gradually during treatment with antimycobacterial agents for 1 year. This case emphasizes the possibility that cutaneous M. kansasii infection may occur in an immunocompromised patient and that exposure to contaminated water is a possible source. With early diagnosis, the response to an antimycobacterial multidrug regimen is usually satisfactory.

  20. Cytokines and MicroRNAs as Candidate Biomarkers for Systemic Lupus Erythematosus

    PubMed Central

    Stypińska, Barbara; Paradowska-Gorycka, Agnieszka

    2015-01-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, with varied course and symptoms. Its etiology is very complex and not clearly understood. There is growing evidence of the important role of cytokines in SLE pathogenesis, as well as their utility as biomarkers and targets in new therapies. Other potential new SLE biomarkers are microRNAs. Recently, over one hundred different microRNAs have been demonstrated to have a significant impact on the immune system. Various alterations in these microRNAs, associated with disease pathogenesis, have been described. They influence the signaling pathways and functions of immune response cells. Here, we aim to review the emerging new data on SLE etiology and pathogenesis. PMID:26473848

  1. Chronic inflammatory demyelinating polyradiculoneuropathy in a boy with systemic lupus erythematosus.

    PubMed

    Zoilo, Morel Ayala; Eduardo, Benadón; Enrique, Faugier; del Rocio, Maldonado V M

    2010-05-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, autoimmune peripheral neuropathy. Systemic lupus erythematosus (SLE) is a multisystemic, autoimmune disease that can affect the central nervous system in about 40% of patients, with prevalence and incidence unknown in the pediatric population due to lack of multicenter studies. We report the case of a 13-year-old Mexican boy, diagnosed with CIDP at the onset of SLE, beginning with progressive muscle weakness of lower and upper limbs, without affection of the central nervous system. The patient had positive ANA, antiDNAdc, antiBeta2glycoprotein, anti-cardiolipin, ANCA-C and X. He received intravenous immunoglobulin, cyclophosphamide, steroids, and azathioprine and showed clinical improvement. It is important to take into account the presence of peripheral neurological disorders in patients with pediatric SLE, considering CIDP as an uncommon presentation, making the diagnosis important for better treatment and evolution.

  2. Oxidative Stress and Treg and Th17 Dysfunction in Systemic Lupus Erythematosus

    PubMed Central

    Yang, Xue

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organ systems. The pathogenic mechanisms that cause SLE remain unclear; however, it is well recognized that the immune balance is disturbed and that this imbalance contributes to the autoimmune symptoms of SLE. Oxidative stress represents an imbalance between the production and manifestation of reactive oxygen species and the ability of the biological system to readily detoxify the reactive intermediates or to repair the resulting damage. In humans, oxidative stress is involved in many diseases, including atherosclerosis, myocardial infarction, and autoimmune diseases. Numerous studies have confirmed that oxidative stress plays an important role in the pathogenesis of SLE. This review mainly focuses on the recent research advances with respect to oxidative stress and regulatory T (Treg)/helper T 17 (Th17) cell dysfunction in the pathogenesis of SLE.

  3. Life Threatening Severe QTc Prolongation in Patient with Systemic Lupus Erythematosus due to Hydroxychloroquine

    PubMed Central

    O'Laughlin, John P.; Wong, Brian C.

    2016-01-01

    We present a case of a syncopal episode resulting from significant QT interval prolongation in a patient on hydroxychloroquine for the treatment of systemic lupus erythematosus and end stage renal disease. The patient had been treated with hydroxychloroquine for two years prior to presentation. After thorough workup for secondary causes of QT interval prolongation hydroxychloroquine was discontinued and the patient's QT interval shortened. The patient was treated with mexiletine to prevent sudden ventricular arrhythmias, which was unique compared to other documented cases in which lidocaine was used. The patient was noted to have mild prolongation of the QT interval on electrocardiogram prior to initiation of hydroxychloroquine therapy which was exacerbated by its use and may have been caused due to toxicity from underlying renal failure. PMID:27478650

  4. Bilateral isolated concurrent superior ophthalmic vein thrombosis in systemic lupus erythematosus.

    PubMed

    Sambhav, Kumar; Shakir, Omar; Chalam, Kakarla V

    2015-01-01

    We describe a case of bilateral consecutive superior ophthalmic vein thrombosis as a presenting feature in a patient previously not known to have systemic lupus erythematosus (SLE). A 68-year-old African-American female presented with decreased vision in right eye, mild right orbital tenderness, and frontotemporal headache of 3 days duration. MRI of the orbits confirmed thrombosis of the right superior ophthalmic vein without extension into the cavernous sinus. Sequential MRI at 1 month showed interval improvement of the right superior ophthalmic vein thrombosis and a new thrombosis in the left superior ophthalmic vein. Renal biopsy revealed granular membranous and mesangial deposits of IgG, IgA, IgM, C3, and C1q and confirmed the diagnosis of SLE. PMID:26392788

  5. Targeted B cell therapies in the treatment of adult and pediatric systemic lupus erythematosus.

    PubMed

    Hui-Yuen, J S; Nguyen, S C; Askanase, A D

    2016-09-01

    Belimumab (Benlysta) is a fully-humanized monoclonal antibody that inhibits B-lymphocyte stimulator (also known as B cell activating factor) and was approved by the U.S. Federal Drug Administration and European Medicines Evaluation Agency for treatment in adults with autoantibody-positive systemic lupus erythematosus (SLE). Rituximab (Rituxan) is a chimeric anti-CD20 monoclonal antibody targeting B lymphocytes. This review discusses the key findings of the phase III trials in adults with SLE and of real-world use of belimumab and rituximab in the care of both adult and pediatric SLE patients. It highlights the safety profile of belimumab and rituximab and gives insight into the consideration of these therapies for specific SLE disease states. It concludes with a discussion of the current clinical trials investigating B cell therapies in specific SLE disease states and a look to the future, with ongoing clinical trials. PMID:27497253

  6. Life Threatening Severe QTc Prolongation in Patient with Systemic Lupus Erythematosus due to Hydroxychloroquine.

    PubMed

    O'Laughlin, John P; Mehta, Parag H; Wong, Brian C

    2016-01-01

    We present a case of a syncopal episode resulting from significant QT interval prolongation in a patient on hydroxychloroquine for the treatment of systemic lupus erythematosus and end stage renal disease. The patient had been treated with hydroxychloroquine for two years prior to presentation. After thorough workup for secondary causes of QT interval prolongation hydroxychloroquine was discontinued and the patient's QT interval shortened. The patient was treated with mexiletine to prevent sudden ventricular arrhythmias, which was unique compared to other documented cases in which lidocaine was used. The patient was noted to have mild prolongation of the QT interval on electrocardiogram prior to initiation of hydroxychloroquine therapy which was exacerbated by its use and may have been caused due to toxicity from underlying renal failure. PMID:27478650

  7. An unusual association of three autoimmune disorders: celiac disease, systemic lupus erythematosus and Hashimoto's thyroiditis.

    PubMed

    Boccuti, Viera; Perrone, Antonio; D'Introno, Alessia; Campobasso, Anna; Sangineto, Moris; Sabbà, Carlo

    2016-12-01

    Autoimmune disorders are known to be more frequent in women and often associated each others, but it is rare to see multiple autoimmune diseases in a single patient. Recently, the concept of multiple autoimmune syndrome has been introduced to describe patients with at least three autoimmune diseases. We describe a case of a young man with a clinical history of psychiatric symptoms and celiac disease (CD) who was diagnosed to have other two autoimmune disorders: systemic lupus erythematosus (SLE) and Hashimoto's thyroiditis. This case is unusual upon different patterns: the rare combination of the three autoimmune diseases, their appearance in a man and the atypical onset of the diseases with psychiatric symptoms likely to be related either to CD or to SLE. PMID:27383232

  8. Multiplex giant magnetoresistive biosensor microarrays identify interferon-associated autoantibodies in systemic lupus erythematosus

    NASA Astrophysics Data System (ADS)

    Lee, Jung-Rok; Haddon, D. James; Wand, Hannah E.; Price, Jordan V.; Diep, Vivian K.; Hall, Drew A.; Petri, Michelle; Baechler, Emily C.; Balboni, Imelda M.; Utz, Paul J.; Wang, Shan X.

    2016-06-01

    High titer, class-switched autoantibodies are a hallmark of systemic lupus erythematosus (SLE). Dysregulation of the interferon (IFN) pathway is observed in individuals with active SLE, although the association of specific autoantibodies with chemokine score, a combined measurement of three IFN-regulated chemokines, is not known. To identify autoantibodies associated with chemokine score, we developed giant magnetoresistive (GMR) biosensor microarrays, which allow the parallel measurement of multiple serum antibodies to autoantigens and peptides. We used the microarrays to analyze serum samples from SLE patients and found individuals with high chemokine scores had significantly greater reactivity to 13 autoantigens than individuals with low chemokine scores. Our findings demonstrate that multiple autoantibodies, including antibodies to U1-70K and modified histone H2B tails, are associated with IFN dysregulation in SLE. Further, they show the microarrays are capable of identifying autoantibodies associated with relevant clinical manifestations of SLE, with potential for use as biomarkers in clinical practice.

  9. Glutamate receptor biology and its clinical significance in neuropsychiatric systemic lupus erythematosus.

    PubMed

    Aranow, Cynthia; Diamond, Betty; Mackay, Meggan

    2010-02-01

    The recent appreciation that a subset of anti-DNA antibodies cross-reacts with the N-methyl-d-aspartate receptor encourages a renewed examination of antibrain reactivity in systemic lupus erythematosus (SLE) autoantibodies. Moreover, investigations of their autospecificity present a paradigm for studies of antibrain reactivity and show that (1) serum antibodies access brain tissue only after a compromise of blood-brain barrier integrity, (2) the same antibodies have differential effects on brain function depending on the region of brain exposed to the antibodies, and (3) insults to the blood-brain barrier are regional rather than diffuse. These studies suggest that an anatomic classification scheme for neuropsychiatric SLE may facilitate research on etiopathogenesis and the design of clinical trials.

  10. Oxidative Stress and Treg and Th17 Dysfunction in Systemic Lupus Erythematosus.

    PubMed

    Yang, Ji; Yang, Xue; Zou, Hejian; Li, Ming

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organ systems. The pathogenic mechanisms that cause SLE remain unclear; however, it is well recognized that the immune balance is disturbed and that this imbalance contributes to the autoimmune symptoms of SLE. Oxidative stress represents an imbalance between the production and manifestation of reactive oxygen species and the ability of the biological system to readily detoxify the reactive intermediates or to repair the resulting damage. In humans, oxidative stress is involved in many diseases, including atherosclerosis, myocardial infarction, and autoimmune diseases. Numerous studies have confirmed that oxidative stress plays an important role in the pathogenesis of SLE. This review mainly focuses on the recent research advances with respect to oxidative stress and regulatory T (Treg)/helper T 17 (Th17) cell dysfunction in the pathogenesis of SLE. PMID:27597882

  11. Bilateral isolated concurrent superior ophthalmic vein thrombosis in systemic lupus erythematosus

    PubMed Central

    Sambhav, Kumar; Shakir, Omar; Chalam, Kakarla V

    2015-01-01

    We describe a case of bilateral consecutive superior ophthalmic vein thrombosis as a presenting feature in a patient previously not known to have systemic lupus erythematosus (SLE). A 68-year-old African–American female presented with decreased vision in right eye, mild right orbital tenderness, and frontotemporal headache of 3 days duration. MRI of the orbits confirmed thrombosis of the right superior ophthalmic vein without extension into the cavernous sinus. Sequential MRI at 1 month showed interval improvement of the right superior ophthalmic vein thrombosis and a new thrombosis in the left superior ophthalmic vein. Renal biopsy revealed granular membranous and mesangial deposits of IgG, IgA, IgM, C3, and C1q and confirmed the diagnosis of SLE. PMID:26392788

  12. Defect of a complement receptor 3 epitope in a patient with systemic lupus erythematosus.

    PubMed Central

    Witte, T; Dumoulin, F L; Gessner, J E; Schubert, J; Götze, O; Neumann, C; Todd, R F; Deicher, H; Schmidt, R E

    1993-01-01

    Complement receptor 3 (CR3) is expressed on cells of the reticuloendothelial system and involved in the clearance of immune complexes. In this article a patient with a deficiency of the C3bi binding site of this receptor is described. Clinically this patient exhibited predominantly cutaneous manifestations of a systemic lupus erythematosus with an immune vasculitis and panniculitis. The deficiency of the CR3 epitope was demonstrated using flow cytometry. The functional relevance of this defect was demonstrated in a rosetting assay with C3bi-loaded erythrocytes. C3bi binding was found to be significantly decreased. Furthermore, there was an impairment of phagocytosis of opsonized Escherichia coli. The CR3 defect is not due to an autoantibody but is assumed to have a genetic basis. These data suggest that the defect of the CR3 may be involved in the pathogenesis of the immune vasculitis in this patient. Images PMID:7690773

  13. Updated review of complementary and alternative medicine treatments for systemic lupus erythematosus.

    PubMed

    Greco, Carol M; Nakajima, Claire; Manzi, Susan

    2013-11-01

    It is estimated that over 50 % of patients with systemic lupus erythematosus (SLE) have utilized complementary and alternative medicine (CAM) treatments to reduce symptoms and manage their health. However, there are relatively few randomized controlled trials of CAM for SLE. This review describes recent studies of vitamins and supplements, acupuncture, and mind-body interventions in SLE patients. The recent trials of CAM treatments for SLE indicate that supplements such as vitamin D, omega 3 fatty acids, N-acetyl cysteine and turmeric show some promise for reducing SLE disease activity. In addition, mind-body methods such as cognitive-behavioral therapy and other counseling interventions may improve mood and quality of life in SLE.

  14. The Role of γδ T Cells in Systemic Lupus Erythematosus.

    PubMed

    Wu, Meng; Yang, Jinhua; Li, Xiaofeng; Chen, Junwei

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that is characterized by the overproduction of autoantibodies against an array of nuclear and cytoplasmic antigens and affects multiple organs, such as the skin, joints, kidneys, and neuronal tissues. T cells have been recognized as important players in the development of SLE due to their functions in cytokine secretion, antigen presentation, and supporting B cells for antibody production. γδ T cells are a minor population of T cells that play important roles in infection and tumor-associated disease. In recent years, the role of γδ T cells in autoimmune diseases has been investigated. In this review, we discussed the role of γδ T cells in the pathogenesis of SLE. PMID:26981547

  15. Antiphospholipid antibodies, systemic lupus erythematosus, and non-traumatic metatarsal fractures

    PubMed Central

    Sangle, S; D'Cruz, D; Khamashta, M; Hughes, G

    2004-01-01

    Background: Stress fractures are common in military recruits and athletes and are thought to be secondary to stress and overuse. Less often they are associated with metabolic disorders such as osteoporosis, hypophosphataemia, and diabetes mellitus. Objective: Analysis of 19 patients with systemic lupus erythematosus and antiphospholipid antibodies presenting consecutively with foot pain. All had metatarsal fractures (six bilateral) without any obvious history of trauma. Results: 13 of the 19 patients had antiphospholipid syndrome. Among the whole group, 13 had normal bone mineral density, one had osteopenia, and five others had osteoporosis as defined by WHO criteria; 10 had received steroids, mostly in low dosage; 13 were receiving warfarin. There was no evidence that a metabolic bone abnormality was a unifying factor in the pathogenesis. Conclusions: Atraumatic metatarsal stress fractures may occur in SLE, particularly in association with the antiphospholipid syndrome. The pathogenesis of these fractures remains uncertain but microinfarcts in the metatarsal bones are a possible cause. PMID:15361379

  16. Multibacillary leprosy mimicking systemic lupus erythematosus: case report and literature review.

    PubMed

    Horta-Baas, G; Hernández-Cabrera, M F; Barile-Fabris, L A; Romero-Figueroa, M del S; Arenas-Guzmán, R

    2015-09-01

    Leprosy is an infectious chronic disease with a wide range of clinical and serological manifestations. We report a case of a woman presenting with a malar rash, painless oral ulcers, photosensitivity, arthritis, positive antinuclear antibodies test and leuko-lymphopenia. Our case illustrates an unusual presentation of leprosy initially diagnosed as systemic lupus erythematosus (SLE). After the confirmation of multibacillary leprosy and multidrug therapy recommended by the World Health Organization, a good clinical response was observed. Recognition of rheumatic manifestations in leprosy is important as they may be confused with SLE. A literature review is presented to encourage clinicians to consider leprosy as a differential diagnosis. Specifically in patients with unusual rheumatic manifestations and persistent skin lesions, and when neurological symptoms are present. Leprosy has not been eradicated, so misdiagnosis can be frequent. It is necessary to increase medical practitioner awareness in order start proper treatment.

  17. An interesting case of systemic lupus erythematosus presenting with hypercalcemia: A diagnostic dilemma

    PubMed Central

    Abdul Gafor, Abdul Halim; Cader, Rizna Abdul; Das, Srijit; Masir, Noraidah; Wahid, Fadilah Abdul

    2013-01-01

    Background Hypercalcemia is common in primary hyperparathyroidism malignancies and even in tuberculosis. Interestingly, systemic lupus erythematosus (SLE) rarely presents with hypercalcemia. Case Report: We describe an interesting case of SLE in a patient who was otherwise thought to have either tuberculosis or a malignancy. The patient initially presented with feeling unwell, with generalized lymphadenopathy, bilateral pleural effusion, and bilateral corneal calcium deposits secondary to severe hypercalcemia. The diagnosis of SLE was made based on positivity of antinuclear antibodies (ANA) and anti-dsDNA, the presence of serositis, lymphadenopathy, autoimmune hemolytic anemia, and constitutional symptoms. She was treated with steroids, with tremendous improvement in her general well-being, resolution of lymphadenopathy and pleural effusion, and normalization of her hemoglobin and serum calcium. The atypical presentation of SLE with hypercalcemia with pleural effusion is discussed. Conclusions: SLE should be one of the differential diagnoses in patients presenting with severe hypercalcemia. PMID:23569551

  18. Necrotising fasciitis in systemic lupus erythematosus: a case report and literature review

    PubMed Central

    Tung Chen, Y; Isenberg, D

    2014-01-01

    Necrotising fasciitis (NF) is a rare infection of the subcutaneous tissue, known to be rapidly progressive and potentially fatal. Patients with systemic lupus erythematosus (SLE) may be predisposed to this condition, and early clinical recognition can be difficult. We report a case of necrotising fasciitis in a 26-year-old woman with SLE. She presented with painful swelling of her left leg, then developed clinical features of septic shock. Emergency debridement was performed. Intraoperative findings revealed NF and cultures grew Pseudomonas aeruginosa. The patient survived after a lengthy hospital admission, following several further debridements complicated by recurrent chest sepsis and multiorgan failure. We also review and discuss the published cases of NF in SLE patients. PMID:25396059

  19. Linear cutaneous lupus erythematosus following the lines of Blaschko - Case report.

    PubMed

    Marinho, Ayana Karla de Oliveira Ferreira; Ramos, Ticiana Batista; Barbosa, Deborah Maria de Castro; Kozmhinsky, Valter; Takano, Daniela Mayumi; Figueira, Marcella Maria de Souza Araújo

    2016-01-01

    Chronic cutaneous lupus erythematosus in a linear configuration is rare, particularly in children, demonstrating similar incidence in both genders, no photo-sensitivity and lower probability of progression to systemic disease. We describe the case of a 9-year-old girl who presented erythematous papules with central atrophy on the upper and lower right limbs, asymptomatic and following the lines of Blaschko, since age four. Histological examination showed atrophy of the epidermis with aggression from epidermal-dermal interface and periadnexal and perivascular lymphocytic inflammatory infiltrate. Laboratory tests showed ANA in a titer of 1:320, in a dense and fine speckled pattern. Due to the rarity of presentation and location of the disease, this case is reported here. PMID:27579750

  20. Multiplex giant magnetoresistive biosensor microarrays identify interferon-associated autoantibodies in systemic lupus erythematosus

    PubMed Central

    Lee, Jung-Rok; Haddon, D. James; Wand, Hannah E.; Price, Jordan V.; Diep, Vivian K.; Hall, Drew A.; Petri, Michelle; Baechler, Emily C.; Balboni, Imelda M.; Utz, Paul J.; Wang, Shan X.

    2016-01-01

    High titer, class-switched autoantibodies are a hallmark of systemic lupus erythematosus (SLE). Dysregulation of the interferon (IFN) pathway is observed in individuals with active SLE, although the association of specific autoantibodies with chemokine score, a combined measurement of three IFN-regulated chemokines, is not known. To identify autoantibodies associated with chemokine score, we developed giant magnetoresistive (GMR) biosensor microarrays, which allow the parallel measurement of multiple serum antibodies to autoantigens and peptides. We used the microarrays to analyze serum samples from SLE patients and found individuals with high chemokine scores had significantly greater reactivity to 13 autoantigens than individuals with low chemokine scores. Our findings demonstrate that multiple autoantibodies, including antibodies to U1-70K and modified histone H2B tails, are associated with IFN dysregulation in SLE. Further, they show the microarrays are capable of identifying autoantibodies associated with relevant clinical manifestations of SLE, with potential for use as biomarkers in clinical practice. PMID:27279139

  1. Guillain Barré Syndrome, Systemic Lupus Erythematosus and Acute Intermittent Porphyria - A Deadly Trio.

    PubMed

    Patil, Ankita D; Karnik, Niteen D; Nadkar, Milind Y; Gupta, Vishal A; Muralidhara, Krithika; Passidhi, Suresh

    2015-11-01

    Peripheral nervous system involvement occurs in 3-18% patients of systemic lupus erythematosus (SLE) cases. American College of Rheumatology (ACR) includes 19 neuropsychiatric syndromes for diagnosis of SLE divided into neurological syndromes of central, peripheral and autonomic nervous systems along with the psychiatric syndromes. Sensorimotor quadriparesis in a suspected case of SLE could be due to a Guillain Barré (GBS)-like illness, mononeuritis multiplex presenting as plexopathies, an anterior spinal artery syndrome or it can present like an acute transverse myelitis or hypokalemic periodic paralysis related to Sjogren's syndrome with renal tubular acidosis. We here report a case of a fulminant quadriparesis due to a SLE flare which subsequently was also found to be a case of Acute Intermittent Porphyria. PMID:27608785

  2. Visualization of dermal alteration in skin lesions with discoid lupus erythematosus by multiphoton microscopy

    NASA Astrophysics Data System (ADS)

    Lin, L. H.; Yu, H. B.; Zhu, X. Q.; Zhuo, S. M.; Wang, Y. Y.; Yang, Y. H.; Chen, J. X.

    2013-04-01

    Discoid lupus erythematosus (DLE) is a chronic dermatological disease which lacks valid methods for early diagnosis and therapeutic monitoring. Considering the collagen and elastin disorder due to mucin deposition of DLE, multiphoton microscopy (MPM) imaging techniques were employed to obtain high-resolution collagen and elastin images from the dermis. The content and distribution of collagen and elastin were quantified to characterize the dermal pathological status of skin lesions with DLE in comparison with normal skin. Our results showed a significant difference between skin lesions with DLE and normal skin in terms of the morphological structure of collagen and elastin in the dermis, demonstrating the possibility of MPM for noninvasively tracking the pathological process of DLE even in its early stages and evaluating the therapeutic efficacy at the molecular level.

  3. Discovery of Biomarkers for Systemic Lupus Erythematosus Using a Library of Synthetic Autoantigen Surrogates

    PubMed Central

    Quan, Jiexia; Lakhanpal, Akshai; Reddy, M.Muralidhar; Zaman, Sayed; Li, Quan-Zhen; German, Dwight C.; Olsen, Nancy J.; Kodadek, Thomas; Karp, David R.

    2014-01-01

    Antibodies to a wide range of self-antigens, including those directed against nucleic acids or nucleic acid-binding proteins are the essential biomarkers for diseases such as systemic lupus erythematosus (SLE). Highly complex libraries of nonamers consisting of N-substituted glycines (peptoids) were screened for compounds that bound IgG from patients with SLE and earlier, incomplete autoimmune syndromes. Peptoids were identified that could identify subjects with SLE and related syndromes with a high sensitivity (70%) and specificity (97.5%). Immobilized peptoids were used to isolate IgG from both healthy subjects and SLE patients that reacted with known RNA-binding proteins. In the case of SLE patients, the peptoid-purified IgG reacted with several autoantigens, suggesting that the peptoids are capable of interacting with multiple, structurally similar molecules. These results show that the measurement of IgG binding to peptoids can identify subjects with high levels of pathogenic autoantibodies. PMID:24269750

  4. Prolactin levels and autoantibodies in female patients with systemic lupus erythematosus.

    PubMed

    Kozáková, D; Rovenský, J; Cebecauer, L; Bosák, V; Jahnová, E; Vigas, M

    2000-01-01

    We investigated the relationships between prolactin (PRL) levels and antibody occurrence in systemic lupus erythematosus (SLE). No significant association between PRL levels and the majority of the autoantibodies studied (anti-U1 RNP, anti-rRNP, anti-Sm, anti-dsDNA, anti-DNP, auto-LCA, anti-EACA) could be confirmed (P > 0.05), anti-Ro/SSA antibodies being an exception. Our results showed significantly increased frequencies of these antibodies in the group of female SLE patients with normal PRL levels (< 20 micrograms/L): anti Ro/SSA in 53% (P < 0.025, chi 2 = 5.80, RR = 4.0) and anti-Ro/SSA + anti-Ro/La in 60% (P < 0.05, chi 2 = 4.05) compared with female SLE patients with hyperprolactinemia. PMID:11155810

  5. The Role of γδ T Cells in Systemic Lupus Erythematosus

    PubMed Central

    Wu, Meng; Yang, Jinhua; Li, Xiaofeng; Chen, Junwei

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that is characterized by the overproduction of autoantibodies against an array of nuclear and cytoplasmic antigens and affects multiple organs, such as the skin, joints, kidneys, and neuronal tissues. T cells have been recognized as important players in the development of SLE due to their functions in cytokine secretion, antigen presentation, and supporting B cells for antibody production. γδ T cells are a minor population of T cells that play important roles in infection and tumor-associated disease. In recent years, the role of γδ T cells in autoimmune diseases has been investigated. In this review, we discussed the role of γδ T cells in the pathogenesis of SLE. PMID:26981547

  6. Ascending paresis as presentation of an unusual association between necrotizing autoimmune myopathy and systemic lupus erythematosus.

    PubMed

    García-Reynoso, Marco Julio; Veramendi-Espinoza, Liz Eliana; Ruiz-Garcia, Henry Jeison

    2014-01-01

    A 45 year-old man went to the emergency room due to disease duration of 15 days of insidious onset and progressive course. It began with symmetrical weakness and pain in feet and ankles that extends upward to the knees. Later, this progressed to paraparesis with Creatine phosphokinase levels of 44,270 U/L and respiratory failure that required mechanical ventilation. Electromyography and muscle biopsy of quadriceps were made. The patient responded to corticotherapy in pulses and supporting management. The presentation of ascending paresis suggested the diagnosis of Guillain-Barré syndrome. However, the degree of muscle involvement with rhabdomyolysis explains the neurological damage by itself. The biopsy revealed pathological criteria for necrotizing autoimmune myopathy (NAM), as well as other clinical and laboratory evidence. Patient disease continued and reached criteria for systemic lupus erythematosus (SLE). To our best knowledge, this is the first report of the NAM and SLE association.

  7. The potential role of autologous stem cell transplantation in patients with systemic lupus erythematosus.

    PubMed

    Hahn, B H

    1997-05-01

    Transfer of disease by bone marrow cells has been described in experimental models of systemic lupus erythematosus (SLE). In one experiment, marrow ablation followed by transfer of T depleted allogeneic marrow resulted in prolonged survival of animals with SLE. Some experimental studies suggest a rationale for autologous stem cell transplantation indicating this intervention might "reset the thermostat" so that normal immunoregulation can control disease, while others indicate it might not be beneficial. The pros and cons of offering patients with SLE autologous hematopoietic stem cell transplantation are considered. A profile of the patient with SLE who might be considered as a candidate for autologous stem cell transplantation can be constructed by evaluating causes of death and factors that increase mortality. This profile includes life threatening disease, inadequate response to aggressive immunosuppressive therapy, and adequate function of all major organs so that risks associated with stem cell transplantation can be minimized.

  8. Spontaneous patellar tendon rupture in a case followed up for diagnosis of systemic lupus erythematosus

    PubMed Central

    Albayrak, İlknur; Küçük, Adem; Arslan, Şevket; Özbek, Orhan

    2014-01-01

    Spontaneous patellar tendon rupture is a rare condition that usually occurs secondary to conditions, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), and use of steroids and fluoroquinolones. This paper presents a full-thickness patellar tendon rupture detected with magnetic resonance imaging, which was performed due to pain and swelling that started spontaneously on the front side of the left knee without a history of any trauma, of a 35-year-old male patient who had been followed up for a diagnosis of SLE for approximately 4 months and who had started taking methylprednisolone 4 mg/day 4 months prior, used it for 1 month, and then stopped using it. In patients who are followed up for a diagnosis of SLE, it should be kept in mind that there is a risk of developing a spontaneous tendon rupture secondary to chronic inflammation and use of corticosteroids.

  9. The CD11b-integrin (ITGAM) and systemic lupus erythematosus.

    PubMed

    Fagerholm, S C; MacPherson, M; James, M J; Sevier-Guy, C; Lau, C S

    2013-06-01

    Variations at the ITGAM gene, which encodes for the CD11b chain of the Mac-1 (alphaMbeta2; CD11b/CD18; complement receptor-3) integrin, is one of the strongest genetic risk factors for systemic lupus erythematosus (SLE). More specifically, a genetic variant (rs1143679) which results in an arginine to histidine substitution at position 77 in the extracellular portion of the integrin is associated with disease. It has recently been shown that this amino acid substitution results in a dysfunctional integrin, which is deficient in mediating cell adhesion to integrin ligands, phagocytosis and in addition cannot restrict inflammatory cytokine production in macrophages. In this review, we discuss immunological functions of the Mac-1 integrin and how defects in the genetic variant of Mac-1 may relate to SLE development.

  10. Feasibility of using DNA-immobilized nanocellulose-based immunoadsorbent for systemic lupus erythematosus plasmapheresis.

    PubMed

    Xu, Changgang; Carlsson, Daniel O; Mihranyan, Albert

    2016-07-01

    The goal of this project was to study the feasibility of using a DNA-immobilized nanocellulose-based immunoadsorbent for possible application in medical apheresis such as systemic lupus erythematosus (SLE) treatment. Calf thymus DNA was bound to high surface area nanocellulose membrane at varying concentrations using UV-irradiation. The DNA-immobilized samples were characterized with scanning electron microscopy, atomic force microscopy, and phosphorus elemental analysis. The anti-ds-DNA IgG binding was tested in vitro using ELISA. The produced sample showed high affinity in vitro to bind anti-ds-DNA-antibodies from mice, as much as 80% of added IgG was bound by the membrane. Furthermore, the binding efficiency was quantitatively dependent on the amount of immobilized DNA onto nanocellulose membrane. The described nanocellulose membranes are interesting immunoadsorbents for continued clinical studies. PMID:27011345

  11. Systemic Lupus Erythematosus, Radiotherapy, and the Risk of Acute and Chronic Toxicity: The Mayo Clinic Experience

    SciTech Connect

    Pinn, Melva E.; Gold, Douglas G. M.; Petersen, Ivy A.; Osborn, Thomas G.; Brown, Paul D.; Miller, Robert C.

    2008-06-01

    Purpose: To determine the acute and chronic toxic effects of radiotherapy in patients with systemic lupus erythematosus (SLE). Methods and Materials: Medical records of 21 consecutive patients with SLE, who had received 34 courses of external beam radiotherapy and one low-dose-rate prostate implant, were retrospectively reviewed. Patients with discoid lupus erythematosus were excluded. Results: Median survival was 2.3 years and median follow-up 5.6 years. Eight (42%) of 19 patients evaluable for acute toxicity during radiotherapy experienced acute toxicity of Grade 1 or greater, and 4 (21%) had acute toxicity of Grade 3 or greater. The 5- and 10-year incidence of chronic toxicity of Grade 1 or greater was 45% (95% confidence interval [CI], 22-72%) and 56% (95% CI, 28-81%), respectively. The 5- and 10-year incidence of chronic toxicity of Grade 3 or greater was 28% (95% CI, 18-60%) and 40% (95% CI, 16-72%), respectively. Univariate analysis showed that chronic toxicity of Grade 1 or greater correlated with SLE renal involvement (p < 0.006) and possibly with the presence of five or more American Rheumatism Association criteria (p < 0.053). Chronic toxicity of Grade 3 or greater correlated with an absence of photosensitivity (p < 0.02), absence of arthritis (p < 0.03), and presence of a malar rash (p < 0.04). Conclusions: The risk of acute and chronic toxicity in patients with SLE who received radiotherapy was moderate but was not prohibitive of the use of radiotherapy. Patients with more advanced SLE may be at increased risk for chronic toxicity.

  12. Milk fat globule E-8 and interleukin 17 in systemic lupus erythematosus: partners in crime?

    PubMed Central

    Elgengehy, Fatema; Niazy, Marwa; Ghoneim, Shada

    2016-01-01

    Objectives Systemic lupus erythematosus (SLE) is a multi-factorial, autoimmune disease with a wide array of manifestations. The pro-inflammatory cytokine interleukin (IL)-17 has been implicated in the inflammatory response and tissue damage in SLE; however, its correlation with disease activity is still questionable. Meanwhile, efficient clearance of apoptotic cells is required for immune tolerance. An abnormally low or high level of milk fat globule (MFG-E8) can result in impaired apoptotic cell clearance and the subsequent autoimmune response. In this study, we endeavoured to compare the levels of MFG-E8 and IL-17 in SLE patients and healthy controls and to reveal the alleged association of these levels with SLE disease activity. Material and methods Serum samples from 57 SLE patients and 30 healthy control subjects were examined for quantitation of MFG-E8 and IL-17 levels using ELISA. Systemic lupus erythematosus disease activity was calculated using the SLE Disease Activity Index (SLEDAI). Clinical manifestations and laboratory findings of the patients were also recorded. Results We report that serum MFG-E8 levels were significantly elevated in the sera of SLE patients compared to healthy controls (p-value = 0.019). Likewise, IL-17 levels were higher in SLE patients (p-value < 0.001). A positive correlation was revealed between MFG-E8 level and proteinuria. Surprisingly, there was a poor correlation between disease activity and the levels of either IL-17 or MFG-E8. Conclusions Although serum MFG-E8 and IL-17 levels were higher in SLE patients than in normal controls, our results indicate that they cannot accurately reflect the disease activity. Meanwhile, further studies are needed to assess MFG-E8 and IL-17 as potential therapeutic targets in SLE patients. PMID:27407263

  13. Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre

    PubMed Central

    Dickey, BZ; Holland, KE; Drolet, BA; Galbraith, SS; Lyon, VB; Siegel, DH; Chiu, YE

    2016-01-01

    Background Paediatric cutaneous lupus erythematosus (LE) is uncommon and inadequately described in the literature. Similar to adults, children with cutaneous LE develop LE-specific and/or LE-nonspecific skin findings. Similarities and differences in demographics and clinical course between paediatric and adult cutaneous LE have not been sufficiently described. Objectives The purpose of this study is to detail the demographic and clinical features of paediatric cutaneous LE and then compare these findings to those reported in the adult literature. Methods A retrospective chart review was performed of 53 children seen in a paediatric dermatology clinic with cutaneous manifestations of LE. Results Patients presented with all five major subtypes of cutaneous LE, with some notable differences from adult cutaneous LE and previously published reports of paediatric cutaneous LE. Progression from discoid LE to systemic lupus erythematosus (SLE) did not occur in our cohort. Patients with subacute cutaneous LE were more likely than adults to have lesions below the waist as well as concomitant SLE. Sex distribution for cutaneous LE in our study was equal prior to puberty and female-predominant in post-pubertal patients. Conclusions Children with cutaneous LE have variable clinical presentations and progression to SLE that may be different from adult disease. Specifically, children with acute and subacute cutaneous LE may be more likely than adults to have systemic disease; therefore, patients with these subtypes should be monitored closely for evidence of SLE. Study limitations included small patient numbers that may limit ability to generalize this data and relatively short follow-up intervals. PMID:23601021

  14. Use of Atorvastatin in Systemic Lupus Erythematosus in Children and Adolescents

    PubMed Central

    Schanberg, L. E.; Sandborg, C.; Barnhart, H. X.; Ardoin, S. P.; Yow, E.; Evans, G. W.; Mieszkalski, K. L.; Ilowite, N. T.; Eberhard, A.; Imundo, L. F.; Kimura, Y.; von Scheven, E.; Silverman, E.; Bowyer, S. L.; Punaro, M.; Singer, N. G.; Sherry, D. D.; McCurdy, D.; Klein-Gitelman, M.; Wallace, C.; Silver, R.; Wagner-Weiner, L.; Higgins, G. C.; Brunner, H. I.; Jung, L.; Soep, J. B.; Reed, A. M.; Provenzale, J.; Thompson, S. D.

    2014-01-01

    Objective Statins reduce atherosclerosis and cardiovascular morbidity in the general population, but their efficacy and safety in children and adolescents with systemic lupus erythematosus (SLE) are unknown. This study was undertaken to determine the 3-year efficacy and safety of atorvastatin in preventing subclinical atherosclerosis progression in pediatric-onset SLE. Methods A total of 221 participants with pediatric SLE (ages 10–21 years) from 21 North American sites were enrolled in the Atherosclerosis Prevention in Pediatric Lupus Erythematosus study, a randomized double-blind, placebo-controlled clinical trial, between August 2003 and November 2006 with 36-month followup. Participants were randomized to receive atorvastatin (n = 113) or placebo (n = 108) at 10 or 20 mg/day depending on weight, in addition to usual care. The primary end point was progression of mean-mean common carotid intima-media thickening (CIMT) measured by ultrasound. Secondary end points included other segment/wall-specific CIMT measures, lipid profile, high-sensitivity C-reactive protein (hsCRP) level, and SLE disease activity and damage outcomes. Results Progression of mean-mean common CIMT did not differ significantly between treatment groups (0.0010 mm/year for atorvastatin versus 0.0024 mm/year for placebo; P = 0.24). The atorvastatin group achieved lower hsCRP (P = 0.04), total cholesterol (P < 0.001), and low-density lipoprotein (P < 0.001) levels compared with placebo. In the placebo group, CIMT progressed significantly across all CIMT outcomes (0.0023–0.0144 mm/year; P < 0.05). Serious adverse events and critical safety measures did not differ between groups. Conclusion Our results indicate that routine statin use over 3 years has no significant effect on subclinical atherosclerosis progression in young SLE patients; however, further analyses may suggest subgroups that would benefit from targeted statin therapy. Atorvastatin was well tolerated without safety concerns. PMID

  15. Subclinical cerebrovascular cognitive function, and mood changes in patients with systemic lupus erythematosus

    PubMed Central

    Shehata, Ghaydaa A; Abdel-Kareem, Mohamed I; Yassin, Abd ellah N; El Adl, Abdel Hamid R

    2010-01-01

    Objective To estimate the prevalence of neuropsychiatric disorders, cerebral atherosclerosis in patients with systemic lupus erythematosus (SLE) and explore the relation between transcranial duplex findings of different intracranial vessels with neuropsychiatric affect, and Systemic lupus erythematosus disease activity index (SLEDAI). Methods Twenty-six consecutive SLE patients were evaluated for neurological and psychiatric disorders. Another 26 subjects matched with respect to age, sex, education, and socioeconomic status formed the control group. SLE disease activity was assessed by the SLEDAI. For each participant, a complete medical history was obtained and clinical, laboratory, and neurophysiological examinations, magnetic resonance imaging of the brain, transcranial duplex for intracranial vessels, and psychometric evaluations were performed. For the psychometric evaluation, we used the Modified Mini-mental State Examination and Cognitive Assessment Scale Inventory to assess cognitive function, and Hamilton Depression Rating Scale and Hamilton Anxiety Scale to assess symptoms of depression and anxiety. Results Anxiety in 65.4% is the most prevalent manifestation followed by depression in 57.7%, headache in 38.5%, peripheral neuropathy in 26.9%, seizures in 23.1%, psychosis in 19.2%, radiculopathy and dementia in 15.4% for each, myositis in 11.5%, and stroke in 7.7%. There was a significant increased mean velocity and decreased pulsatility index of most studied intracranial vessels in both patient groups than in the control group. There was significant negative correlation between SLEDAI and transcranial Doppler findings in the pulsatility index of medial circumflex artery and procoagulant activity. Conclusion Neurological disorders, cognitive impairment, depression, anxiety, psychosis and cerebrovascular changes detected by transcranial Doppler ultrasound are common in SLE.

  16. Health-related quality of life assessed by LupusQoL questionnaire and SF-36 in Turkish patients with systemic lupus erythematosus.

    PubMed

    Yilmaz-Oner, Sibel; Oner, Can; Dogukan, Fatih Mert; Moses, Toklong Filam; Demir, Kubra; Tekayev, Nazar; Atagunduz, Pamir; Tuglular, Serhan; Direskeneli, Haner

    2016-03-01

    The LupusQoL is a disease-specific health-related quality of life (HRQoL) measure for patients with lupus. We conducted this study to compare the efficiency of LupusQoL-TR (validated Turkish version of the LupusQoL questionnaire) with the 36-item Short-Form Health Survey (SF-36), a generic quality of life (QoL) scale, in Turkish patients with lupus. Both questionnaires were conducted at a single visit to the clinic. Disease activity was measured with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Associations between the LupusQoL-TR and SF-36 domains were examined while also examining age, disease duration, and disease activity for each questionnaire. Descriptive statistics, Spearman's correlation coefficients, and Students t test were performed to analyze the data. A total of 113 consecutive patients with lupus (F/M 108:5, mean age 40.6 ± 11.9 years, mean disease duration 8.5 ± 7.0 years) were included, and 69 % of these were active. The median SLEDAI score was 2 (0-24), the mean global LupusQoL-TR score was 60.9 ± 23.3, and the mean SF-36 score was 41.2 ± 9.0. There was a significant correlation between LupusQoL-TR and SF-36 mean scores (r = 0.83; p < 0.001). QoL assessed by LupusQoL-TR and SF-36 did not correlate with disease activity (r = -0.11; p = 0.244 and r = -0.03; p = 0.721, respectively). LupusQoL-TR and SF-36 questionnaires were beneficial instruments in evaluating HRQoL in Turkish lupus patients. However, LupusQoL-TR and SF-36 were not associated with SLEDAI scores, which suggested that QoL might be affected by other factors besides disease activity, especially in clinically inactive or mildly active patients.

  17. Cardiovascular events prior to or early after diagnosis of systemic lupus erythematosus in the systemic lupus international collaborating clinics cohort

    PubMed Central

    Urowitz, M B; Gladman, D D; Anderson, N M; Su, J; Romero-Diaz, J; Bae, S C; Fortin, P R; Sanchez-Guerrero, J; Clarke, A; Bernatsky, S; Gordon, C; Hanly, J G; Wallace, D J; Isenberg, D; Rahman, A; Merrill, J; Ginzler, E; Alarcón, G S; Fessler, B F; Petri, M; Bruce, I N; Khamashta, M; Aranow, C; Dooley, M; Manzi, S; Ramsey-Goldman, R; Sturfelt, G; Nived, O; Steinsson, K; Zoma, A; Ruiz-Irastorza, G; Lim, S; Kalunian, K C; Ỉnanç, M; van Vollenhoven, R; Ramos-Casals, M; Kamen, D L; Jacobsen, S; Peschken, C; Askanase, A; Stoll, T

    2016-01-01

    Objective To describe the frequency of myocardial infarction (MI) prior to the diagnosis of systemic lupus erythematosus (SLE) and within the first 2 years of follow-up. Methods The systemic lupus international collaborating clinics (SLICC) atherosclerosis inception cohort enters patients within 15 months of SLE diagnosis. MIs were reported and attributed on a specialised vascular event form. MIs were confirmed by one or more of the following: abnormal ECG, typical or atypical symptoms with ECG abnormalities and elevated enzymes (≥2 times upper limit of normal), or abnormal stress test, echocardiogram, nuclear scan or angiogram. Descriptive statistics were used. Results 31 of 1848 patients who entered the cohort had an MI. Of those, 23 patients had an MI prior to SLE diagnosis or within the first 2 years of disease. Of the 23 patients studied, 60.9% were female, 78.3% were Caucasian, 8.7% black, 8.7% Hispanic and 4.3% other. The mean age at SLE diagnosis was 52.5±15.0 years. Of the 23 MIs that occurred, 16 MIs occurred at a mean of 6.1±7.0 years prior to diagnosis and 7 occurred within the first 2 years of follow-up. Risk factors associated with early MI in univariate analysis are male sex, Caucasian, older age at diagnosis, hypertension, hypercholesterolaemia, family history of MI and smoking. In multivariate analysis only age (OR=1.06 95% CI 1.03 to 1.09), hypertension (OR=5.01, 95% CI 1.38 to 18.23), hypercholesterolaemia (OR=4.43, 95% CI 1.51 to 12.99) and smoking (OR=7.50, 95% CI 2.38 to 23.57) remained significant risk factors. Conclusions In some patients with lupus, MI may develop even before the diagnosis of SLE or shortly thereafter, suggesting that there may be a link between autoimmune inflammation and atherosclerosis. PMID:27099765

  18. [Systemic lupus erythematosus in a boy with chronic granulomatous disease: case report and review of the literature].

    PubMed

    Ben Abdallah Chabchoub, R; Turki, H; Mahfoudh, A

    2014-12-01

    The association of chronic granulomatosis disease (CGD) with autoimmune diseases such as lupus has been described but remains rare. K… is a boy born of a consanguineous marriage. In the family history, two brothers had died at a young age. He had been followed up since the age of 6 months for CGD. At 11 years of age, he developed malar rash, cheilitis, oral ulceration, and photosensitivity. Systemic lupus erythematosus (SLE) was confirmed by the presence of high levels of antinuclear antibodies. This observation demonstrates that with the clinical association of recurrent infections and skin lesions the diagnosis of CGD with SLE must be considered. PMID:25445129

  19. Development of various arrhythmias and conduction disturbances following corticosteroid therapy for systemic lupus erythematosus with antiphospholipid syndrome.

    PubMed

    Kasamatsu, Yu; Yoshioka, Katsunobu; Miyashita, Tomoko; Shibata, Mikiko; Nakamura, Tomoyuki; Yamagami, Keiko

    2010-08-01

    A 45-year-old Chinese woman with active systemic lupus erythematosus, lupus anticoagulant positive, was admitted to our hospital. Electrocardiography on admission was normal. Though anti-Sjögren's syndrome A (anti SS-A/Ro) antibodies were negative and ultrasound cardiographic findings were normal, she developed various arrhythmias/conduction disturbances shortly after starting corticosteroid. Nearly all were resolved with continuous corticosteroid and aspirin therapy before discharge. Vasculitis, the presence of antiphospholipid antibodies, and platelet aggregation due to corticosteroid were possible mechanisms underlying the arrhythmias/conduction disturbances.

  20. Spontaneous coronary artery dissection in the context of positive anticardiolipin antibodies and clinically undiagnosed systemic lupus erythematosus.

    PubMed

    Nisar, M K; Mya, T

    2011-11-01

    Spontaneous coronary artery dissection (SCAD) is an extremely uncommon condition that can lead to fatal acute myocardial infarction. There have been very few case reports of SCAD in patients with systemic lupus erythematosus (SLE) and even fewer in association with antiphospholipid antibodies - mainly postpartum. This is the first reported case of SCAD in a patient who was confirmed to have SLE and tested positive for anticardiolipin antibody and lupus anticoagulant. This case demonstrates the importance of carefully considering the differential diagnoses of SCAD at presentation. It also highlights the need for further research to explore the link between SLE, antiphospholipid antibodies and SCAD.