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.
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