A 28-year-old woman was admitted to our hospital in August 1998, because of high grade fever, anorexia, and general fatigue. She had been diagnosed as having mixed connective tissue disease (MCTD) because of Raynaud's phenomenon, sausage like fingers, positive anti U 1-RNP antibody, multiple arthralgia, and sclerodactyly. The laboratory examination revealed the marked elevation of serum GOT (3, 712IU/L), GPT (2, 246IU/L), and LDH (5, 907IU/L). The antibodies suggestive of participation of viral hepatitis, such as serum IgM class of anti-hepatitis A, hepatitis B antigen, and anti-hepatitis C were negative. The liver biopsy showed cellularly enlarged portal areas and damaged interlobular bile ducts with altered biliary epithelium. This is the report of a rare case with MCTD associated with chronic non-suppurative cholangitis probably induced by autoimmune mechanism.
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