Primary sclerosing cholangitis
© 2015 Elsevier Ltd. 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, leading ultimately 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 co-existing PSC. Clinical symptoms include fatigue, intermittent jaundice, weight loss, right upper-quadrant abdominal pain and pruritus. The clinical course of PSC is variable. Serum biochemical tests usually indicate cholestasis; the diagnosis is established by cholangiography. In symptomatic patients, median survival from presentation to death or liver transplantation is about 12 years. About 75% of asymptomatic patients survive 20 years or more. Median overall survival is 23 years. Overall, 37% of patients die from hepatic failure, while approximately 44% die from cancer - PSC is a premalignant condition. The commonest malignancy is hepatobiliary in origin, usually bile duct carcinoma, which is often aggressive. Patients with associated inflammatory bowel disease may die from colonic cancer or complications of colitis. PSC has no curative treatment. Medical treatment with the bile acid, ursodeoxycholic acid, may slow progression of the disease and act 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.