Primary Sclerosing Cholangitis -- Approach to Diagnosis

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


April 25, 2007

Clinical Presentation

Case 1 illustrates the classic patient with PSC: a young male with history of ulcerative colitis who is found to have a cholestatic pattern on serum liver biochemistry measurement. Indeed, up to 70% of patients with PSC are male, with the mean age of 40 years at time of diagnosis.[2,9,10]

Case 2 demonstrates the diagnostic challenge of PSC because of its varying clinical presentation. Approximately 15% to 40% of patients with PSC are asymptomatic.[6,7,8,11] Despite the lack of symptoms, these patients have a decreased life expectancy compared with the general population.[12,13] This may be due to the fact that up to 17% of asymptomatic patients are found to have cirrhosis at diagnosis.[8]

When present, symptoms of PSC are vague and nonspecific, and usually are of little benefit in making the diagnosis. Typically, patients will go through successive periods of exacerbation and remission, with the most common complaints consisting of fatigue, right upper quadrant abdominal pain, and pruritus.[14,15] Also, particular attention should be paid to clinical signs of infection, because these patients are at increased risk for the development of bacterial cholangitis secondary to biliary obstruction. Jaundice may appear during these episodes of acute cholangitis, but unrelieved jaundice usually means progression of the underlying disease. In general, symptomatic patients are more likely to have advanced liver disease as well as a decreased survival free of liver transplantation. Finally, although unusual, PSC can present as acute liver failure. This diagnosis should especially be considered in individuals with IBD who present with acute liver failure of indeterminate etiology.[16]


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