Extraintestinal manifestations of inflammatory bowel disease: Primary sclerosing cholangitis

© Springer Science+Business Media, LLC 2012. All rights reserved. Primary sclerosing cholangitis (PSC) is a chronic and progressive disease of the biliary tree characterised by concentric, obliterative fibrosis leading to bile duct stricturing and eventually cirrhosis in the majority of cases. The disease course is highly variable between individuals but most patients reach the combined end-point of death or liver transplantation 12–17 years following their diagnosis [1]. One-third of the patients PSC will develop cholangiocarcinoma. Although the underlying aetiopathogenesis of PSC is not yet fully elucidated it is generally accepted to be a condition of immune dysregulation. Approximately three quarters of the Northern European PSC population have concomitant inflammatory bowel disease (IBD), with the predominant form of IBD being ulcerative colitis (UC). In 1874, only 7 years after PSC was defined, CH Thomas first recognised this association when he described a man who died of a “much enlarged, fatty liver in the presence of ulceration of the colon“ [2]. Caring for the patients PSC presents a number of challenges – Unfortunately medical therapy remains controversial and the only intervention with proven survival benefit is liver transplantation. Not only is the diagnostic work-up and management of PSC complex, but also treatment of associated conditions such as IBD and cholangiocarcinoma requires expertise that bridges the ever growling divide between hepatology and luminal gastroenterology.