Overlap of Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis - a Rare Coincidence or a New Syndrome

Background: Overlap syndrome has been introduced to the field of hepatology to describe the coexistence of two or more autoimmune hepatic conditions in the same individual. This is a rare clinical case of a patient diagnosed with primary biliary cirrhosis (PBC) who later developed primary sclerosing cholangitis (PSC). This is a unique case as no other cases with a similar pattern have been reported. Overlap syndrome does not include the coexistence of PBC and PSC as a distinctive syndrome so far.

Case Report: A middle-aged woman suffering from PBC for 17 years got admitted with clinical and biochemical features of cholestatic syndrome. A provisional diagnosis of worsening PBC was proved wrong by magnetic resonance cholangiopancreatography, which revealed typical benign stricture and dilatation of common bile duct with typical beading appearance suggestive of PSC. The patient was stented and treated with an increased dose of ursodeoxycholic acid (UDCA) which improved the symptoms and the biochemical picture.

Conclusion: This is a clear overlap of PBC-PSC. It is very difficult to say whether it is a rare coincidence or a new overlap syndrome, but there are no clear guidelines for management of these patients. Currently, the treatment involves endoscopic duct dilatation, UDCA, and regular follow-ups to rule out hepato-biliary tumor occurrence.