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KMID : 0388219970040020168
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Journal of the Korean Rheumatism Association
1997 Volume.4 No. 2 p.168 ~ p.172
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A Case of Diffuse Scleroderma Associated with Primary Biliary Cirrhosis and Isolated Pulmonary Hypertension
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장현규/Chang, Hyun Kyu
정상식/정행섭/이성희/허환/Chung, Sang Sig/Chung R., Haning Sub/Lee, Sung Hee/Herr, Hwan
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Abstract
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Primary biliary cirrhosis is frequently associated with a variety of disorders presumed to be autoimmune in nature, such as Sjogren s syndrome, scleroderma, rheumatoid arthritis, systemic lupus erythematosus, and autoimmune thyroiditis. Scleroderma has been recognized in association with primary biliary cirrhosis. Most cases present as the CREST (calcinosis cutis, Raynaucf s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome.
Isolated pulmonary hypertension develops in a small proportion of patients, nearly all of whom have limited cutaneous involvement. We report a case who has diffuse scleroderma associated with primary biliary cirrhosis and isolated pulmonary hypertension.
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KEYWORD
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