Primary sclerosing cholangitis – a comprehensive review

Primary sclerosing cholangitis (PSC) is a rare disorder characterised by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. Most patients ultimately require liver transpla...

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Bibliographic Details
Published in:Journal of hepatology Vol. 67; no. 6; pp. 1298 - 1323
Main Authors: Karlsen, Tom H., Folseraas, Trine, Thorburn, Douglas, Vesterhus, Mette
Format: Journal Article
Language:English
Published: Netherlands Elsevier B.V 01.12.2017
Elsevier Science Ltd
Subjects:
Bile ducts
Biopsy
Cholangiocarcinoma
Cholangitis
Cholangitis, Sclerosing - diagnosis
Cholangitis, Sclerosing - etiology
Cholangitis, Sclerosing - genetics
Cholangitis, Sclerosing - therapy
Colorectal cancer
Colorectal carcinoma
Genetic Predisposition to Disease
Humans
IgG4-associated cholangitis
Inflammatory bowel diseases
Inflammatory diseases
Intestine
Liver - pathology
Liver diseases
Liver Transplantation
Pathogenesis
Primary sclerosing cholangitis
Quality of Life
Risk factors
Stricture
Transplants & implants
Primary sclerosing cholangitis
Cholangiocarcinoma
IgG4-associated cholangitis
ISSN:0168-8278, 1600-0641, 1600-0641
Online Access:Get full text
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Summary:Primary sclerosing cholangitis (PSC) is a rare disorder characterised by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. Most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and a lack of proven surveillance strategies, patients currently have significant unmet needs. In the present seminar, we provide a comprehensive review of the status of the field. We emphasise developments related to patient stratification and disease behaviour, and provide an overview of management options from a practical, patient-centered perspective. We survey advances made in the understanding of PSC pathogenesis and summarise the ongoing efforts to develop an effective therapy based on these insights.
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ISSN:0168-8278
1600-0641
1600-0641
DOI:10.1016/j.jhep.2017.07.022