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Primary sclerosing cholangitis: diagnostic and management challenges

Authors Sirpal S, Chandok N

Received 21 February 2017

Accepted for publication 14 August 2017

Published 6 November 2017 Volume 2017:10 Pages 265—273


Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Amy Norman

Peer reviewer comments 3

Editor who approved publication: Professor Andreas M Kaiser

Sanjeev Sirpal,1 Natasha Chandok2

1Department of Medicine, Centre Hospitalier de l’Université de Montréal (CHUM), University of Montreal, Montreal, QC, 2Department of Medicine, University of Western Ontario, London, ON, Canada

Abstract: Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease affecting intra- and extrahepatic bile ducts, primarily the large biliary ducts. Clinical manifestations are broad, and the spectrum encompasses asymptomatic cholestasis, icteric cholangitis with pruritis, cirrhosis, and cholangiocarcinoma. Though rare, PSC has a propensity to affect young to middle-aged males and is strongly associated with inflammatory bowel disease. There is an unmet need for effective medical treatments for PSC, and to date, the only curative therapy is liver transplantation reserved for those with end-stage liver disease. This article addresses the diagnostic and management challenges of PSC, with a succinct analysis of existing therapies, their limitations, and a glimpse into the future of the management of this multifaceted pathologic entity.

Keywords: primary sclerosing cholangitis, management, PSC

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