Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is well known that patients with progressive systemic sclerosis (PSS), as well as other autoimmune disease, have various kinds of autoantibodies. We presented two PSS patients with anti Wa antibody, which had recently been reported as a new anti cytoplasmic antibody in only one case with PSS. Our first case, 49 years old female had clinical features of arthralgia, Raynaud's phenomenon, sclerodactyly, transient myalgia and sicca complex. Her laboratory findings showed hypergammaglobulinemia (1.9 g/dl), positive RAHA (1: 640) anti Wa antibody (1: 256), and anti SS-A antibody (1: 256). Second case, 64 years old female had also clinical features of Raynaud's phenomenon, sclerodactyly, dysphagia, dyspnea on exertion and dry mouth. Furthermore lung fibrosis and dysmobility of upper GI tract were observed. FANA (1: 20) was detected as nucleolar pattern, and the titer of anti Wa antibody was 1: 64. Clinical findings of myositis were not seen in both cases. This study suggested that anti Wa antibody might be one of the diagnostic marker on PSS.
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PMID:[Clinical study on two cases of progressive systemic sclerosis with anti Wa antibody]. 263 85

A case of pediatric progressive systemic sclerosis is reported and a literature review concerning medical and dental aspects of this condition is provided. Systemic features include sclerodactyly, Raynaud's phenomenon, telangiectasia, calcinosis, myositis, arthritis, tenosynovitis, renal failure, esophageal hypomotility, pulmonary fibrosis and heart failure. Oral manifestations include reduced interincisal distance, xerostomia, telangiectasia, increased periodontal ligament width, osseous resorption of the mandible, periodontal disease, and increased decayed, missing, and filled teeth (DMFT). The prognosis is difficult to predict because spontaneous remission has been documented, but death may result from extensive visceral involvement (heart, kidney, and lung).
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PMID:Progressive systemic sclerosis in a child: case report. 824 4

Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus (SLE) characterized by unexplained dyspnea, a restrictive pattern on pulmonary function tests, and an elevated hemidiaphragm. A total of 59 cases are reported in literature including the current case. The mean age of these patients is 36.85 years (range, 15-61 years), and the female-to-male ratio is 6:1. This disorder is seen primarily during the later stages of SLE. The most common presenting features include dyspnea and pleuritic chest pain. Myositis has been reported in only 8 of 59 patients (13%). Diagnosis is made with chest x-ray showing an elevated hemidiaphragm and a restrictive pattern on pulmonary function testing without any evidence of interstitial lung disease along with decreased transdiaphragmatic pressure (Pdi). Corticosteroids are the most common method of treatment. Immunosuppressive therapy, beta-agonists, and theophylline are used in those resistant to steroids. The prognosis is generally good. This article reports the case of a 22-year-old man presenting with a 7-month history of dry mouth and dry eyes accompanied by increasing difficulty in breathing, progressing to dyspnea at rest. The patient's history included bilateral parotid gland swelling and nephrotic syndrome diagnosed 4 years earlier. Pertinent physical and laboratory findings included positive Schirmer's test results; bilateral parotid gland enlargement; bibasilar lung crackles; synovitis of the second and third proximal interphalangeal joints; a positive antinuclear antibody (Ro/SSA), Sm, and anticardiolipin antibodies; and elevated right hemidiaphragm on chest x-ray. Pulmonary function tests demonstrated restrictive lung disease with normal high-resolution computerized axial tomography. A dramatic response to oral prednisone (60 mg daily) was observed in all of the patient's complaints in a matter of several days. A diagnosis of SLE with secondary Sjogren's syndrome (SS) and SLS was made. Although SLS has been reported in association with SLE, there has been only one previous report of SLS in SLE/SS overlap syndrome. Early recognition with appropriate treatment can decrease the morbidity associated with this rare syndrome.
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PMID:Shrinking lung syndrome in systemic lupus erythematosus and Sjogren's syndrome. 1704 5

Graft-versus-host disease (GvHD) is a common and often serious complication of hematopoietic stem cell transplantation. There are two major forms of GvHD: an acute form which develops in the first 100 days after HSCT, and a chronic form which develops later. Chronic GvHD is a multiorgan syndrome with many features of autoimmune diseases, such as sclerodermatous skin changes, cholestasis, pulmonary fibrosis, xerostomia, oral ulcerations, myositis and fasciitis. Unlike acute GvHD which is characterized by acute alloreactivity,the etiology of chronic GvHD is controversial and is believed to be either an extension of acute GvHD and/or a result of dysfunctional immune reconstitution with generation of autoantibodies and autoreactive T-cell clones. GvHD is usually treated with corticosteroids and other immunosuppressants which do not always succeed in arresting its evolution. Extracorporeal photochemotherapy has been used in patients with both acute and chronic GvHD.
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PMID:Extracorporeal photopheresis in graft-versus-host disease. 1817 13