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Primary Sclerosing Cholangitis

Sclerosing cholangitis is a progressive condition characterized by inflammation and fibrosis (fibrous cords and tissue) of the liver's biliary system. Over time, the condition destroys the internal and external bile ducts, ultimately leading to liver failure. While the cause is unknown, it is linked to immunoregulation dysfunction. There are several types of sclerosing cholangitis: primary, infectious and vascular. Of primary sclerosing cholangitis (PSC), males are more likely to be affected, generally between 25 and 45 years of age. Roughly 70 percent of patients with PSC also have ulcerative colitis although the association isn't clear. Symptoms include jaundice, pruritis (itching), weight loss, right upper quadrant pain, acute cholangitis, elevated serum transaminase levels, and malaise. Many patients are asymptomatic. PSC may present as chronic hepatitis, particularly in children. Bile duct lesions, copper deposits and necrosis may be evident with liver biopsy. Cholangiograms may show beading and strictures in the biliary tree. Treatment includes symptom management, fat-soluble vitamin replacement and antibiotics. Liver transplant is an option.