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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Veröffentlicht in:	Journal of hepatology Jg. 67; H. 6; S. 1298 - 1323
Hauptverfasser:	Karlsen, Tom H., Folseraas, Trine, Thorburn, Douglas, Vesterhus, Mette
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	Netherlands Elsevier B.V 01.12.2017 Elsevier Science Ltd
Schlagworte:	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-Zugang:	Volltext
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Abstract	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.
AbstractList	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. 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.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.
Author	Folseraas, Trine Vesterhus, Mette Karlsen, Tom H. Thorburn, Douglas
Author_xml	– sequence: 1 givenname: Tom H. surname: Karlsen fullname: Karlsen, Tom H. email: t.h.karlsen@medisin.uio.no organization: Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway – sequence: 2 givenname: Trine surname: Folseraas fullname: Folseraas, Trine organization: Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway – sequence: 3 givenname: Douglas surname: Thorburn fullname: Thorburn, Douglas organization: UCL Institute for Liver and Digestive Health, Division of Medicine, University College London, UK – sequence: 4 givenname: Mette surname: Vesterhus fullname: Vesterhus, Mette organization: Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway
BackLink	https://www.ncbi.nlm.nih.gov/pubmed/28802875$$D View this record in MEDLINE/PubMed
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Copyright	2017 European Association for the Study of the Liver Copyright © 2017 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. Copyright Elsevier Science Ltd. Dec 2017
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Issue	6
Keywords	Primary sclerosing cholangitis Cholangiocarcinoma IgG4-associated cholangitis
Language	English
License	This is an open access article under the CC BY-NC-ND license. Copyright © 2017 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
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Snippet	Primary sclerosing cholangitis (PSC) is a rare disorder characterised by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel...
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SubjectTerms	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
Title	Primary sclerosing cholangitis – a comprehensive review
URI	https://www.clinicalkey.com/#!/content/1-s2.0-S0168827817321967 https://dx.doi.org/10.1016/j.jhep.2017.07.022 https://www.ncbi.nlm.nih.gov/pubmed/28802875 https://www.proquest.com/docview/2080987700 https://www.proquest.com/docview/1928781224
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