Primary Sclerosing Cholangitis

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Abstract
PRIMARY sclerosing cholangitis, an uncommonly diagnosed syndrome of unknown cause, is characterized by chronic fibrosing inflammation of bile ducts, usually affecting both the extrahepatic and intrahepatic biliary ductal systems.1 , 2 The disease leads to obliteration of intrahepatic bile ducts and to biliary cirrhosis. It can occur alone or in association with inflammatory bowel disease.3 It must be distinguished from bile-duct abnormalities resulting from biliary surgery, choledocholithiasis, bile-duct carcinoma, and congenital malformations. The course is generally one of slow progression to cirrhosis, portal hypertension, and death from liver failure; however, the time course of this progression is unpredictable. The syndrome is only . . .