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Introduction Chronic cholestatic syndrome characterized by fibrosing inflammation in the intrahepatic and extrahepatic bile duct onset usually insidious leads to narrowing and, eventually, obliteration of the bile ducts Cause of primary sclerosing cholangitis (PSC) is unknown PSC occurs most often in young men ( 50% no urine urobilinogen low urine urobilin Endoscopic retrograde cholangiography (ERCP) give the biliary tree an irregular beaded appearance Treatment Liver transplantation prior to liver failure is a possibility in selected cases Prognosis, Prevention, and Complications Prognosis is poor May result in liver failure, cirrhosis, cholangiocarcinoma