The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis

Abstract

Background: The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis is an extremely rare condition that has been reported in only six published cases so far.

Methods: Here we report two cases showing the clinical manifestations of both primary biliary cirrhosis and primary sclerosing cholangitis.

Results: In one case the overlap condition was associated with psoriatric arthritis, and the patient successfully underwent dual treatment with ursodeoxycholic acid and the anti-tumour necrosis factor-alpha agent adalimumab. In the second case, the predominant condition was, initially, an antimitochondrial antibody-negative primary biliary cirrhosis with progressive course towards end-stage liver disease; the patient then developed either antimitochondrial antibody positivity or changes in the biliary tree compatible with primary sclerosing cholangitis.

Conclusions: These two cases add information on a controversial issue in the literature, and indicate the importance of recognizing a possible overlap syndrome to optimize treatment.

Keywords: Overlap syndrome; Primary biliary cirrhosis; Primary sclerosing cholangitis.