An unspecific inflammatory fibrosis of the intermediate and large bile ducts leads to irregular stenosis and ectasia of the intra- and extrahepatic bile ducts. This often remains completely asymptomatic and is only diagnosed because of increased
levels of alkaline phosphatase (AP), although chronic fatigue, stomach pain and intermittent jaundice may also result.
Typically, primary sclerosing cholangitis predominates among male patients in their fifth decade of life.
The clinical course can be variable, with many patients dying due to progressive hepatic insufficiency. The only curative treatment is liver transplantation. About 10% of all patients with primary sclerosing cholangitis subsequently develop cholangiocarcinoma or HCC.
An association with chronic inflammatory bowel disease (such as Colitis ulcerosa) has also been reported. Primary sclerosing cholangitis has to be distinguished from secondary types of sclerosing cholangitis, such as those induced by surgical intervention, cholelithiasis and even cholangiocarcinoma.
tumor of the liver HCC