Primary sclerosing cholangitis - Clinic and therapy

Primary sclerosing cholangitis (PSC) leads to a chronic fibrosis of the intra- or extrahepatic bile ducts. The morphological characteristics with dilatations and strictures of the bile ducts are shown by endoscopic retrograde cholangiography. The etiology of the disease, which mainly affects young male patients, is not clear. Moreover, there is a close correlation to inflammatory bowel diseases. Pruritus and jaundice are the characteristic clinical signs. The progressive course of the disease leads to a secondary biliary cirrhosis. Ursodeoxycholic acid (UDCA) is the drug of choice for the medical treatment. Controlled clinical trials have shown the positive effects on clinical symptoms, laboratory tests and histological changes. However, liver transplantation is required for the progressive stages of PSC.