Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease that often is associated with inflammatory bowel disease (IBD) and thats leads to destruction of the medium and large bile ducts, with a median time from diagnosis to liver transplantation or death of 20 years. In the setting of IBD, the typical cholangiographic features of segmental strictures and dilatations are nearly pathognomonic for PSC. In the absence of IBD, the diagnosis requires the exclusion of secondary causes of sclerosing cholangitis. In addition to liver failure, patients with PSC are at high risk of cholangiocarcinoma, gallbladder cancer, and, in the setting of IBD, colon cancer. Although ursodeoxycholic acid is frequently used to treat PSC, its benefit or the benefit of any other medical therapy in PSC has not been established.