[Primary sclerosing cholangitis]. / Cholangite sclérosante primitive.

Primary sclerosing cholangitis is an uncommon disease, characterized by the progressive destruction of intra and extrahepatic bile ducts. The disease mainly affects males below 40 years of age. Main consequences of the disease are cholestasis, cholangitis, intrahepatic lithiasis, and secondary biliary cirrhosis. In 5-10% of the patients, cholangiocellular carcinoma develops. Diagnosis is based on retrograde cholangiography. There is no specific medical treatment. Liver transplantation must be considered in the end-stage of the disease.