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Abstract
Focal bile-duct dilatation proximal to areas of stricture produces a characteristic beaded appearance on cholangiography.5 ,6 Primary sclerosing cholangitis, although only about 1 percent as common as alcoholic liver disease,7 ,8 is the fourth leading indication for liver transplantation in adults in the United States.9 The disease progresses silently, but relentlessly, in most patients and leads to cirrhosis, portal hypertension, and liver failure.10 ,11 Seventy percent of patients with primary sclerosing cholangitis are men, and the mean age at diagnosis is 39 years.12 The disease typically occurs in patients with inflammatory bowel disease, but it may also occur alone or in association with retroperitoneal or mediastinal fibrosis.13 Of the approximately 75 percent of patients with primary sclerosing cholangitis who have inflammatory bowel disease, about 87 percent have ulcerative colitis and 13 percent Crohn's disease.14 -21 Seen another way, 2.5 to 7.5 percent of patients who present with ulcerative colitis have, or will have, primary sclerosing cholangitis.22 -24 The true frequency of primary sclerosing cholangitis in patients with ulcerative colitis may actually be higher.