1/43. Pseudoporphyria associated with Relafen therapy.Various oral medications including nonsteroidal anti-inflammatory drugs (NSAIDs) have been associated with pseudoporphyria, although the pathogenetic basis has not been elucidated. A novel NSAID nabumetone (Relafen) has become popular because of its minimal gastrointestinal side effects. Its association with pseudoporphyria is not reported save for its listing in the Physician's Desk Reference (PDR) as a possible side effect. Biopsies of lesional skin from 4 patients manifesting blisters and erosions on the hands and face within 4 months of starting nabumetone were submitted for light microscopic and immunofluorescent (IF) studies. Histories and serology were obtained. Two patients had rheumatoid arthritis (RA), 1 had mixed connective tissue disease (MCTD), and 1 received diltiazem. All 4 had antinuclear antibodies. Characteristic clinical, light microscopic and IF features in the absence of elevated urine porphyrin levels confirmed a diagnosis of pseudoporphyria in all 4 patients. Biopsies in three patients showed features attributed to underlying connective tissue disease (CTD), including ectasia of the superficial vascular plexus, mild leukocytoclastic vasculitis, superficial and deep perivascular lymphocytic infiltrates with dermal mucinosis, granular deposition of IgM along the dermoepidermal junction indicative of a positive lupus band test, and of IgG and C5b-9 within keratinocytes. Nabumetone (Relafen) can provoke pseudoporphyria; an underlying CTD diathesis may be a predisposing factor.- - - - - - - - - - ranking = 1keywords = lupus (Clic here for more details about this article) |
2/43. Lupus-like syndrome caused by 5-aminosalicylic acid in patients with inflammatory bowel disease.BACKGROUND: Although 5-aminosalicylic acid (5-ASA) preparations used to treat inflammatory bowel disease are reported to have fewer side effects than sulphasalazine, increased clinical use of these compounds has resulted in increased reports of significant side effects. OBJECTIVE: To report four patients with antinuclear antibody-positive migratory arthralgias and acute inflammation unrelated to the underlying inflammatory bowel disease, fulfilling the criteria of a drug-induced lupus-like syndrome. SETTING: A university-affiliated teaching hospital. INTERVENTION: Cessation of treatment with 5-ASA compounds. RESULTS: The cases described constitute a drug-induced lupus-like syndrome. All patients improved rapidly after discontinuation of 5-ASA compounds. CONCLUSIONS: Reversible lupus-like syndrome appears to be a rare but significant side effect of 5-ASA compounds. patients treated with 5-ASA compounds who experience acute inflammatory symptoms or clinical deterioration not related to their gastrointestinal disease should be screened to rule out a lupus-like reaction.- - - - - - - - - - ranking = 4keywords = lupus (Clic here for more details about this article) |
3/43. magnetic resonance imaging in the evaluation of patients with aseptic meningoencephalitis and connective tissue disorders.OBJECTIVE: To describe the role of magnetic resonance imaging (MRI) in the evaluation of patients with chronic and recurrent aseptic meningitis. METHOD: A retrospective study of five patients with aseptic meningoencefalitis diagnosed by clinical and CSF findings. CT scans showed without no relevant findings. RESULTS: MRI showed small multifocal lesions hyperintense on T2 weighted images and FLAIR, with mild or no gadolinium enhancement, mainly in periventricular and subcortical regions. meningoencephalitis preceded the diagnosis of the underlying disease in four patients (Behcet's disease or systemic lupus erythematosus). After the introduction of adequate treatment for the rheumatic disease, they did not present further symptoms of aseptic meningoencephalitis. CONCLUSION: Aseptic meningoencephalitis can be an early presentation of an autoimmune disease. It is important to emphasize the role of MRI in the diagnosis and follow-up of these patients.- - - - - - - - - - ranking = 27.76952845552keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
4/43. thrombosis in patients with connective tissue diseases treated with specific cyclooxygenase 2 inhibitors. A report of four cases.Specific inhibitors of cyclooxygenase 2 (COX-2) have been approved for the treatment of osteoarthritis and rheumatoid arthritis. Unlike nonsteroidal anti-inflammatory drugs, specific COX-2 inhibitors do not inhibit platelet activation. However, these agents significantly reduce systemic production of prostacyclin. As a result, theoretical concerns have been raised that specific COX-2 inhibitors could shift the hemostatic balance toward a prothrombotic state. patients with connective tissue diseases (CTD), who may be predisposed to vasculopathy and thrombosis, often have arthritis or pain syndromes requiring treatment with antiinflammatory agents. Herein we describe 4 patients with CTD who developed ischemic complications after receiving celecoxib. All patients had a history of Raynaud's phenomenon, as well as elevated anticardiolipin antibodies, lupus anticoagulant, or a history compatible with antiphospholipid syndrome. It was possible to measure a urinary metabolite of thromboxane a2 in 2 of the patients as an indicator of in vivo platelet activation, and this was markedly elevated in both. In addition, the patients had evidence of ongoing inflammation as indicated by elevated erythrocyte sedimentation rate, hypocomplementemia, and/or elevated levels of anti-dna antibodies. The findings in these 4 patients suggest that COX-2 inhibitor-treated patients with diseases that predispose to thrombosis should be monitored carefully for this complication.- - - - - - - - - - ranking = 1keywords = lupus (Clic here for more details about this article) |
5/43. Choroidal vascular occlusion in a child with a connective tissue disease and complement c4 deficiency.OBJECTIVE: To report the histopathologic findings in the eyes of a patient with a connective tissue disease and complement deficiency. DESIGN: Human postmortem ocular histopathologic study. INTERVENTION: A 15-year-old female died from complications of a connective tissue disease of uncertain etiology, particularly acute respiratory distress syndrome. Abnormalities seen in the eyes at autopsy were consistent with complement activation, granulocyte aggregation, and leukocyte embolization. MAIN OUTCOME MEASURES: Both eyes were examined by light microscopy. RESULTS: Some choroidal vessels were occluded by platelet-fibrin thrombi and occasionally by aggregates of granulocytes and fibrin. Serous retinal detachment involving the macula and peripheral retina was present in both eyes. CONCLUSIONS: This is a report of the ocular histopathologic findings in a patient with connective tissue disease and complement c4 deficiency. The light microscopy findings were consistent with complement activation with granulocyte aggregation and leukocyte embolization and may represent another mechanism to explain the clinical findings in patients with connective tissue disease, particularly systemic lupus erythematosus.- - - - - - - - - - ranking = 27.76952845552keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
6/43. osteoporosis with underlying connective tissue disease: an unusual case.A 44-year-old male was initially seen by dermatologists, who noted an erythematous rash on sun-exposed areas, the back, shoulders, and upper arms. There was associated muscle weakness and significant weight loss. Investigation revealed mildly raised aspartate and alanine transaminases but normal creatine kinase. Inflammatory indices and antinuclear antibodies (ANAs) were normal. biopsy of the rash was reported as consistent with either dermatomyositis (DM) or acute lupus erythematosus. A diagnosis of DM was made, and prednisolone was given with improvement of the rash but deteriorating myopathy. The patient was referred to the rheumatology department, and further history revealed multiple vertebral fractures after falling from standing height; these had occurred six months prior to starting steroids. Besides smoking he had no other risk factors for osteoporosis. Examination showed normal muscle strength, no muscle tenderness, and no joint abnormality. Repeat muscle enzymes were normal, and ANAs were now 1 : 100, but dsDNA antibodies and extractable nuclear antigens were normal. Investigations for osteoporosis revealed a hypergonadotrophic hypogonadism picture. Further examination indicated scanty pubic and auxiliary hair, small testicles, and mild gynecomastia. He is married, though has no children of his own. The hormonal profile raised the possibility of Klinefelter's syndrome, which was subsequently confirmed with karyotyping of 47 XXY. hypogonadism has been established as a cause of osteoporosis in males, and in this case would explain the occurrence of fractures in the absence of other major risk factors. Systemic lupus erythematosus has been recognized in association with Klinefelter's syndrome; in view of the normal muscle enzymes, his rash is most likely due to acute discoid lupus with androgen deficiency causing muscle weakness.- - - - - - - - - - ranking = 46.326971668041keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
7/43. sarcoidosis in autoimmune disease.sarcoidosis is a multisystem granulomatous disease of unknown etiology. sarcoidosis coexisting with connective tissue diseases, once considered rare, complicates various such disorders, including rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, sjogren's syndrome, and the spondyloarthropathies. Symptoms common to sarcoidosis and autoimmune disease include keratoconjunctivitis sicca, weight loss, fever, lymphadenopathy, pulmonary complaints, and cutaneous lesions. Consequently, the diagnosis of sarcoidosis in association with connective tissue disease is often difficult and may require biopsy of the lung, liver, skin, lymph node, muscle, or bone marrow for pathological confirmation. Abnormalities of immune function as well as autoantibody production, including rheumatoid factor and antinuclear antibodies, are seen in sarcoidosis and in connective tissue diseases, suggesting a common immunopathogenic mechanism. The severity and course of sarcoidosis associated with autoimmune disease is variable. The incidence of sarcoidosis in association with rheumatic disease may be underestimated if new symptoms of sarcoidosis are attributed to the primary rheumatic disease and a secondary diagnosis is not pursued.- - - - - - - - - - ranking = 27.76952845552keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
8/43. A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease.A previously healthy 14-year-old Korean male presented with striking hemorrhagic acral livedo associated with anti-ribonucleoprotein antibodies. His skin biopsy revealed a striking lymphocytic vascular reaction with mild superficial perivascular lymphocytic infiltrates, perivascular extravasation of red blood cells, and leukocytoclastic debris. Lymphocytic vasculitis (LV) is defined in different ways by different authors. The above biopsy findings do not fulfill the criteria for LV of all authors. Chronic lymphocyte-mediated endothelial cell injury may play an important role in the pathogenesis of collagen vascular disease. Cell-mediated cytotoxicity against endothelial cells or other vessel wall components is thought to be the possible pathologic mechanism of LV. We present a patient with early undifferentiated connective tissue disease (EUCTD) with hemorrhagic acral livedo, who finally developed systemic lupus erythematosus. Even though the histopathologic findings do not meet the most rigorous definition of LV, LV may a presenting sign of EUCTD. The purpose of this report is to suggest reconsideration of the diagnostic criteria of lymphocytic vasculitis.- - - - - - - - - - ranking = 27.76952845552keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
9/43. anetoderma associated with antiphospholipid syndrome and systemic lupus erythematosus.anetoderma is an uncommon disorder characterized by the loss of elastic fibres in the dermis histologically and herniation of subcutaneous tissue clinically. Recent studies indicate that immunologic mechanisms may play a role in this process. Here we report a 33-year-old woman with numerous well-circumscribed, asymptomatic skin lesions in whom clinical and histopathologic features were consistent with anetoderma. Additionally, history and investigations revealed antiphospholipid syndrome and systemic lupus erythematosus. It has been speculated that immune deposits in the dermis or within the capillary walls may lead to ischaemia and subsequent degeneration of the elastic fibres.- - - - - - - - - - ranking = 138.8476422776keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
10/43. Protein-losing enteropathy and pancreatic involvement in a case of connective tissue disease.A patient with connective tissue disease presenting with both protein-losing enteropathy and pancreatic involvement is reported. A 52-year-old female was admitted because of mild epigastralgia, anasarca and ascites. serum albumin, transferrin and zinc, showed low levels. An Upper G.I. series and endoscopy showed thickened folds of the duodenum and the jejunum. biopsy specimens revealed lymphangiectasia in edematous villi. 99mTc-labeled human serum albumin scintigram showed abnormal radioactivity in the small intestine 90 minutes after intravenous injection, indicating protein-losing enteropathy. hypoalbuminemia was ameliorated by glucocorticoid therapy, but recurred twice when glucocorticoid treatment was tapered. hypoalbuminemia has not occurred since intestinal lymphangiectasia was improved with glucocorticoid treatment. Levels of elastase 1 and lipase were high in serum and ascites on admission. Endoscopic retrograde pancreatogram showed no abnormalities. Serum pancreatic enzymes were also ameliorated by glucocorticoid therapy, but slightly high levels continued for about one year and a half. This case might have been diagnosed as systemic lupus erythematosus although mixed connective tissue disease was also suspected. There are few reports of protein-losing enteropathy and pancreatic involvement associated with connective tissue diseases. Protein-losing enteropathy and pancreatic involvement were ameliorated with glucocorticoid treatment, suggesting participation of immunological mechanisms.- - - - - - - - - - ranking = 27.76952845552keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
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