Primary sclerosing cholangitis (PSC) leads to a chronic fibrosis of th
e intra- or extrahepatic bile ducts. The morphological characteristics
with dilatations and strictures of the bile ducts are shown by endosc
opic retrograde cholangiography. The etiology of the disease, which ma
inly affects young male patients, is not clear. Moreover, there is a c
lose 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 clin
ical trials have shown the positive effects on clinical symptoms, labo
ratory tests and histological changes. However, liver transplantation
is required for the progressive stages of PSC.