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J Hepatol. 2017 Dec;67(6):1298-1323. doi: 10.1016/j.jhep.2017.07.022. Epub 2017 Aug 10.

Primary sclerosing cholangitis - a comprehensive review.

Author information

1
Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Norway; Research Institute of Internal Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway. Electronic address: t.h.karlsen@medisin.uio.no.
2
Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; Research Institute of Internal Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway.
3
UCL Institute for Liver and Digestive Health, Division of Medicine, University College London, UK; Sheila Sherlock Liver Centre, Royal Free London NHS Foundation Trust, London, UK.
4
Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; National Centre for Ultrasound in Gastroenterology, Haukeland University Hospital, Bergen, Norway.

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.

KEYWORDS:

Cholangiocarcinoma; IgG4-associated cholangitis; Primary sclerosing cholangitis

PMID:
28802875
DOI:
10.1016/j.jhep.2017.07.022
[Indexed for MEDLINE]
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