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Canadian Journal of Gastroenterology
Volume 26, Issue 5, Pages 261-268
http://dx.doi.org/10.1155/2012/426430
Review

Management of Primary Sclerosing Cholangitis: Conventions and Controversies

Natasha Chandok1 and Gideon M Hirschfield2,3

1Division of Gastroenterology, Western University, London, Canada
2Department of Medicine, University of Toronto, Toronto, Ontario, Canada
3Centre for Liver Research, University of Birmingham, Birmingham, UK

Received 9 March 2011; Accepted 29 July 2011

Copyright © 2012 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary sclerosing cholangitis (PSC) is a chronic inflammatory cholangiopathy that results in fibrotic strictures and dilations of the intra- and extrahepatic bile ducts. PSC is uncommon, occurs predominantly in males and has a strong association with inflammatory bowel disease. While the pathogenesis of PSC has not been fully elucidated, emerging evidence supports roles for the innate and adaptive immune systems, and genome-wide analyses have identified several genetic associations. Using the best available evidence, the present review summarizes the current understanding of the diagnosis, pathogenesis and management of PSC. Despite its rarity, there is an urgent need for collaborative research efforts to advance therapeutic options for PSC beyond liver transplantation.