Primary sclerosing cholangitis is a rare progressive biliary disease. It often affects young men. The disease is often associated with inflammatory bowel disease, especially ulcerative colitis. Its onset is subtle. Symptoms include progressively worsening fatigue, itching and jaundice. Some patients may have signs of enlarged spleen or enlarged liver, or cirrhosis. Eventually it leads to biliary obstruction, biliary cirrhosis and liver failure. The prognosis is poor. There is no effective treatment yet. The purpose of surgical treatment is draining bile to reduce biliary pressure in order to control liver damage.
How is Primary sclerosing cholangitis treated in practice?