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Primary biliary cirrhosis with autoimmune hepatitis (PCB/AIH) overlap is characterized by uncertain behavior and no standardized treatment. A 35 year-old-woman with vitiligo, jaundice and cholestasis fulfilled serological, biochemical and histological criteria for PBC/AIH overlap. Treatment was initiated with conventional doses of corticosteroid and ursodeoxycholic acid. Her condition worsened with poor biochemical hepatic response. The course of action was altered to institute high doses of ursodiol, azathioprine and corticosteroids for extended periods of time. This case illustrates how increased understanding of the overlap PBC/AIH leads to new interventions. Recognition of these variant forms is critical for institutional management of both disease entities.

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