ABSTRACT
Primary biliary cirrhosis and autoimmune hepatitis are the two main immune-mediated liver diseases. They are generally differentiated easily on the basis of clinical, biochemical, serological, and histological findings. Occasionally, the normally distinctive features overlap and classification of a given patient's chronic liver disease is difficult. Series of such patients with Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome were reported. We report a case of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome proven by clinicopathology. The patient showed hepatitic features including easy fatigability, icteric sclera. Markers of hepatitis B and C virus were absent. The liver biopsy showed chronic hepatitis with piecemeal necrosis, infiltration of inflammatory cell surrounding bile duct. The clinical data of high titers of antinuclear antibodies and antimitochondral antibodies, elevated serum IgM, suggest mixed features of primary biliary cirrhosis-autoimmune hepatitis. The patient as given only ursodeoxycholic acid (600 mg/day), then was observed follow-up regularly.