Cholestatic jaundice as the predominant presentation in a patient with autoimmune hepatitis.

Autoimmune hepatitis is a heterogeneous liver disease of unknown etiology. It is predominant in females and characterized by elevation of transaminase, hypergammaglobulinemia and circulating autoantibodies. Interface hepatitis and plasma cell infiltration are the main findings on liver biopsy. However, deep cholestatic jaundice is rarely seen. We present here an unusual case of type 1 autoimmune hepatitis with hyperbilirubinemia as the initial and predominant presentation. Although rare, autoimmune hepatitis should be considered in patients with cholestasis without history of drug or viral hepatitis.