Primary Sclerosing Cholangitis -- Approach to Diagnosis

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


April 25, 2007


The best approach to diagnosing PSC involves a systematic investigation including historical examination, laboratory studies, and cholangiographic evaluation. Particular attention should be paid to a history of IBD, autoimmune disease, or active cholangitis.

Laboratory testing should be directed at ruling out secondary causes of sclerosing cholangitis as well as looking for autoimmune markers, which can be more indicative of PSC. Thus, once initial evaluation with a hepatic panel has suggested cholestasis, further testing with serum autoantibodies and immunoglobulins is recommended.

As shown in Table 1 , the sensitivity and specificity of MRCP in facilities with radiologists experienced in biliary tract diseases approaches that of ERCP. Given the improved safety profile of MRCP compared with ERCP, we anticipate that MRCP will soon be considered as a first-line imaging investigation in centers with skilled radiologists. Once signs of obstruction, dominant strictures, or inconclusive findings are described, the clinician may proceed with an ERCP for diagnostic and therapeutic maneuvers. If the patient's presentation is suggestive of cholangitis, an ERCP should be the procedure of choice.

Finally, liver biopsy should be reserved for cases in which other studies are inconclusive or when a diagnosis of small-duct PSC or an overlap disorder is being considered.


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