Primary Sclerosing Cholangitis -- Approach to Diagnosis

Ian L. Steele, MD; Cynthia Levy, MD; Keith D. Lindor, MD


April 25, 2007


Primary sclerosing cholangitis (PSC) is a progressive disease of the biliary tract characterized by diffuse inflammation and fibrosis of both intra- and extrahepatic bile ducts. According to population-based studies, its prevalence is estimated at 1-10 per 100,000 persons in the United States.[1,2] Disease progression is slow and highly variable, but eventually culminates in cirrhosis and complications related to portal hypertension, with a 10-year survival of approximately 65%.[1,3,4] Problems more specific to PSC can also develop, such as recurrent ascending cholangitis and cholangiocarcinoma.

Although neither the etiology nor the pathogenesis of PSC is well understood, PSC is thought to be an immune-mediated process and is indeed associated with other autoimmune diseases.[5] The vast majority of PSC cases occur in the setting of inflammatory bowel disease (IBD) and its association with IBD is well recognized, especially ulcerative colitis. Although PSC occurs in approximately 5% of all patients with ulcerative colitis, up to 75% of those with PSC will have associated ulcerative colitis.[1,6,7,8]

It can be extremely difficult to diagnose PSC with confidence, particularly outside of tertiary care centers. This article uses case scenarios to illustrate the diagnostic process in PSC, and follows with a review of the current understanding of how clinical presentation, laboratory tests, endoscopy, radiology, and histology facilitate the diagnosis of PSC.

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