Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology. Characteristic features are multifocal strictures and dilatations of the bile ducts. In 60-80 % of cases the PSC is strongly associated with chronic inflammatory bowel disease, mostly in the form of pancolitis. The diagnosis is established based on detection of typical cholangiographic lesions of the bile ducts and exclusion of secondary causes of sclerosing cholangitis. There is no approved medical treatment, but in Germany ursodeoxycholic acid is frequently used. Clinically relevant stenoses can be successfully treated by interventional endoscopy. Patients with PSC suffer from a greatly increased risk of hepatobiliary malignancies, especially with respect to cholangiocarcinoma and colorectal cancer and therefore require regular surveillance and screening. Liver transplantation is currently the only curative treatment option.
Keywords: Cholangiocarcinoma; Cholangiopancreatography, endoscopic retrograde; Inflammatory bowel diseases; Liver transplantation; Ursodeoxycholic acid.