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Primary sclerosing cholangitis in adults: Management

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-size ducts in the intrahepatic and extrahepatic biliary tree. The great majority of affected patients have underlying ulcerative colitis; the incidence may be as high as 90 percent when rectal and sigmoid biopsies are routinely obtained [1]. (See "Primary sclerosing cholangitis: Epidemiology and pathogenesis".)

PSC is associated with numerous complications, including cholestasis (with associated problems), dominant stricture formation, cholelithiasis and cholangitis, cholangiocarcinoma, and colon cancer (in patients with concurrent ulcerative colitis) [2,3]. In addition, PSC may follow a progressive course, resulting in portal hypertension and liver failure.

There are two major goals of treatment in PSC:

Retardation and reversal of the disease process

Management of progressive disease and its complications

                         

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Literature review current through: Nov 2016. | This topic last updated: Tue Jan 12 00:00:00 GMT 2016.
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References
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