Consecutive occurrence of primary biliary cirrhosis and autoimmune hepatitis: a case report and review of the literature.

Autoimmune cholangiopathy is a term that describes a subset of patients with overlapping features of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AH). These patients typically have cholestasis, negative antimitochondrial antibody (AMA), liver histology suggestive of PBC, and may respond clinically to corticosteroid therapy. We describe a patient who presented with typical AMA-positive, biopsy-proven PBC who responded to ursodeoxycholic acid therapy both clinically and biochemically. Approximately 3 yr later, she developed elevated transaminases with biopsy-proven antinuclear antibody (ANA) negative AH. The AMA was negative at this time. After responding to steroid therapy for the AH and after discontinuing the ursodeoxycholic acid, the patient had a clinical recurrence of PBC with renewed AMA positivity.