Development of autoimmune hepatitis in primary biliary cirrhosis


Keith D. Lindor, MD, Mayo Clinic, 200 First St. SW, Rochester, MN 55901, USA
Tel: +507 284 2969
Fax: +507 266 4531


Aim/Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown aetiology. Up to 10% of patients with typical features of PBC will have additional features of autoimmune hepatitis (AIH). A subset, however, have no such features but go on to develop a ‘sequential’ AIH overlap syndrome.

Objectives: Describe our experience with eight patients who developed AIH after the diagnosis of PBC was made.

Methods: We reviewed the charts of all PBC patients over a 9-year period (from 1996 to 2005). Only PBC patients with no features of AIH were included.

Results: There were 1476 patients with PBC. Of these, eight patients developed features of AIH overlap syndrome based on biochemical and histological parameters. Treatment included prednisone and azathioprine for 24 or more months. The majority of patients remained on ursodeoxycholic acid (UDCA) throughout treatment. Response to therapy was defined by improvement in enzymes, and was rapid for all patients. One patient was able to discontinue treatment with prednisone and azathioprine, while seven have continued on therapy to date.

Conclusions: A ‘sequential’ overlap syndrome of AIH with PBC can occur. Treatment with prednisone and azathioprine may lead to a rapid improvement in aminotransferase levels.