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Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. The progressive pathological process obliterates intrahepatic and extrahepatic bile ducts, ultimately leading to biliary cirrhosis, portal hypertension and hepatic failure. The cause is unknown but it is closely associated with inflammatory bowel disease, particularly ulcerative colitis which occurs in about 70% of cases. Approximately 5-10% of patients with total ulcerative colitis will have coexisting PSC. Clinical symptoms include fatigue, intermittent jaundice, weight loss, right upper quadrant abdominal pain and pruritus. The clinical course of PSC is variable In symptomatic patients, median survival from presentation to death or liver transplantation is about 12 years. About 75% of asymptomatic patients survive 15 years or more. Patients die in hepatic failure following deepening cholestatic jaundice. About 30% die from bile duct carcinoma, which is often aggressive. The remainder die from colonic cancer or complications of colitis. Serum biochemical tests usually indicate cholestasis; the diagnosis is established by cholangiography. PSC has no curative treatment. Medical treatment with the bile acid ursodeoxycholic acid (UDCA) probably slows progression of the disease and acts as a chemoprotective agent against colonic dysplasia. Liver transplantation is the only option in young patients with PSC and advanced liver disease; 5-year survival is 80-90% in most centres. The disease will recur in the donor liver in 30% of patients after 5 years. © 2006.

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