Autoimmune cholangitis with features of autoimmune hepatitis: Successful treatment with immunosuppressive agents and ursodeoxycholic acid

We report a 42-year-old Chinese female with elevated serum levels of liver aminotransferases, alkaline phosphatase, gamma-glutamyl transpeptidase, cho lesterol and immunoglobulin M. Serum antimitochondrial antibody was negativ e, but antinuclear antibody was strongly positive. Liver histology showed f eatures of both autoimmune cholangitis and autoimmune hepatitis. Combinatio n therapy with immunosuppressive (prednisone and azathioprine) and choleure tic agents (ursodeoxycholic acid) was given. Serum aminotransferases and bi liary enzymes showed much improvement after treatment. A follow-up liver bi opsy showed improvement of both hepatic necroinflammation and bile duct dam age. Biliary enzymes rose after withdrawal of the immunosuppressive agents and declined again with reinstitution of prednisone. This case demonstrates that a combination of immunosuppressive agents and ursodeoxycholic acid ma y effectively treat patients with features of both autoimmune cholangitis a nd autoimmune hepatitis. (C) 2000 Blackwell Science Asia Pty Ltd.