Management of Primary Sclerosing Cholangitis: Conventions and Controversies

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 pathogenes...

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Main Authors: Natasha Chandok, Gideon M Hirschfield
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Canadian Journal of Gastroenterology
Online Access:http://dx.doi.org/10.1155/2012/426430
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author Natasha Chandok
Gideon M Hirschfield
author_facet Natasha Chandok
Gideon M Hirschfield
author_sort Natasha Chandok
collection DOAJ
description 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.
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spelling doaj-art-cad0db5f178c48bab922183f9417837c2025-02-03T05:50:56ZengWileyCanadian Journal of Gastroenterology0835-79002012-01-0126526126810.1155/2012/426430Management of Primary Sclerosing Cholangitis: Conventions and ControversiesNatasha Chandok0Gideon M Hirschfield1Division of Gastroenterology, Western University, London, CanadaDepartment of Medicine, University of Toronto, Toronto, Ontario, CanadaPrimary 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.http://dx.doi.org/10.1155/2012/426430
spellingShingle Natasha Chandok
Gideon M Hirschfield
Management of Primary Sclerosing Cholangitis: Conventions and Controversies
Canadian Journal of Gastroenterology
title Management of Primary Sclerosing Cholangitis: Conventions and Controversies
title_full Management of Primary Sclerosing Cholangitis: Conventions and Controversies
title_fullStr Management of Primary Sclerosing Cholangitis: Conventions and Controversies
title_full_unstemmed Management of Primary Sclerosing Cholangitis: Conventions and Controversies
title_short Management of Primary Sclerosing Cholangitis: Conventions and Controversies
title_sort management of primary sclerosing cholangitis conventions and controversies
url http://dx.doi.org/10.1155/2012/426430
work_keys_str_mv AT natashachandok managementofprimarysclerosingcholangitisconventionsandcontroversies
AT gideonmhirschfield managementofprimarysclerosingcholangitisconventionsandcontroversies