Sclerosing cholangitis: Primary and secondary

Abstract

Sclerosing cholangitis comprises a spectrum of chronic cholestatic disease of the hepatobiliary system characterized by hepatic inflammation, biliary strictures, and fibrosis. The best studied form is primary sclerosing cholangitis (PSC), which is a slowly progressive disorder eventually resulting in concentric obliterative fibrosis of the bile ducts, biliary cirrhosis, and, in approximately 30% of patients, cholangiocarcinoma. There is a strong association between PSC and inflammatory bowel disease (IBD), with between 75 and 80% of PSC patients of northern European origin having underlying IBD (1,2). Ulcerative colitis is the most common form of IBD associated with PSC, and, interestingly, when considering the pathogenesis of the disease, those PSC patients who have Crohn's disease almost invariably have disease predominantly affecting the colon. © 2007 Humana Press Inc.