Evans' syndrome associated with dermatomyositis
Article Abstract:
Dermatomyositis is a rare connective tissue disease which may occur alone or in combination with other connective tissue disease, including pulmonary (lung) fibrosis. Evans' syndrome, an autoimmune disease (in which the body makes antibodies against its own tissues) characterized by thrombocytopenia (low levels of platelets, blood cells important for clotting) and hemolytic anemia, (red blood cell destruction), is frequently associated with connective tissue diseases such as systemic lupus erythematosus and scleroderma. A case is described of a 77-year-old woman who developed pulmonary fibrosis, Evans' syndrome, then dermatomyositis. She first sought medical care due to breathlessness, chest pain, and leg bruising. Hemolytic anemia and thrombocytopenia were disclosed by laboratory tests. Pulmonary function was abnormal, and X-rays from four years earlier showed pulmonary fibrosis. The anemia and platelet abnormalities responded well to steroid therapy. Eight months later, the patient developed red scaling skin lesions on the face, arms, and chest, as well as severe muscle weakness, which was diagnosed as dermatomyositis. Blood tests showed pronounced levels of a factor associated with systemic lupus erythematosus, indicating that some features of that disease may have been present. Steroid and cytotoxic (cell-destroying) therapy was given, and the patient responded well, although later she developed bone weakness. This case is the first described in which dermatomyositis and Evans' syndrome co-occurred in a patient. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 1990
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Detection of anti-ADAM 10 antibody in serum of a patient with pulmonary fibrosis associated with dermatomyositis
Article Abstract:
Some collagen vascular disorders may produce autoantibodies against lung tissue. Researchers detected such an antibody in one patient with dermatomyositis and pulmonary fibrosis. The antibody was directed against an enzyme called A disintegrin and metalloprotease (ADAM) 10.
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 1999
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Sequential changes of KL-6 in sera of patients with interstitial pneumonia associated with polymyositis/dermatomyositis
Article Abstract:
A glycoprotein named KL-6 can be used as a biological marker for lung disease in patients with polymyositis or dermatomyositis. In a study of 16 such patients, KL-6 levels were higher in those who had pneumonia. The levels also changed as the pneumonia progressed.
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 2000
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