Development of autoimmune hepatitis in primary biliary cirrhosis
Article first published online: 16 AUG 2007
Volume 27, Issue 8, pages 1086–1090, October 2007
How to Cite
Gossard, A. A. and Lindor, K. D. (2007), Development of autoimmune hepatitis in primary biliary cirrhosis. Liver International, 27: 1086–1090. doi: 10.1111/j.1478-3231.2007.01538.x
- Issue published online: 16 AUG 2007
- Article first published online: 16 AUG 2007
- Received 17 January 2007accepted 15 May 2007
- autoimmune hepatitis superimposed on primary biliary cirrhosis;
- sequential development of autoimmune hepatitis in PBC;
- treatment of autoimmune hepatitis developing in patients with PBC
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.