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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| Published in: | Journal of hepatology Vol. 67; no. 6; pp. 1298 - 1323 |
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| Main Authors: | , , , |
| Format: | Journal Article |
| Language: | English |
| Published: |
Netherlands
Elsevier B.V
01.12.2017
Elsevier Science Ltd |
| Subjects: | |
| ISSN: | 0168-8278, 1600-0641, 1600-0641 |
| Online Access: | Get full text |
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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. |
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| 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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| PublicationTitle | Journal of hepatology |
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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 |
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