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Primary sclerosing cholangitis in adults: Clinical manifestations and diagnosis

Author
Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
Section Editor
Keith D Lindor, MD
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF

INTRODUCTION

Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown etiology that is characterized by inflammation, fibrosis, and stricturing of medium and large ducts in the intrahepatic and/or extrahepatic biliary tree (picture 1) [1,2].

This topic will review the clinical manifestations and diagnosis of PSC. The treatment of PSC is discussed separately. (See "Primary sclerosing cholangitis in adults: Management".)

The approach to patients with PSC was reviewed in a 2015 guideline from the American College of Gastroenterology [3] and in a 2010 guideline from by the American Association for the Study of Liver diseases [4]. The discussion that follows is generally consistent with these guidelines.

NATURAL HISTORY

PSC is usually a progressive disorder that ultimately leads to complications of cholestasis and hepatic failure. Median survival without liver transplantation after diagnosis is 10 to 12 years [5-8]. Survival is significantly worse for patients who are symptomatic at the time of diagnosis [7]. (See 'Complications of primary sclerosing cholangitis' below and 'Prognosis' below.)

ASSOCIATION WITH INFLAMMATORY BOWEL DISEASE

The majority of patients with PSC have underlying ulcerative colitis (UC); the prevalence of ulcerative colitis may be as high as 90 percent when rectal and sigmoid biopsies are routinely obtained [9]. Because of this strong association, we evaluate patients diagnosed with PSC for inflammatory bowel disease (IBD). (See "Clinical manifestations, diagnosis, and prognosis of ulcerative colitis in adults", section on 'Evaluation'.)

                             

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Literature review current through: Nov 2016. | This topic last updated: Mon Jan 04 00:00:00 GMT+00:00 2016.
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