Manganis CD, Chapman RW, Culver EL. Review of primary sclerosing cholangitis with increased IgG4 levels. World J Gastroenterol 2020; 26(23): 3126-3144 [PMID: 32684731 DOI: 10.3748/wjg.v26.i23.3126]
Corresponding Author of This Article
Emma L Culver, BSc, MBChB, DPhil, MRCP Gastro, Consultant Hepatologist and Senior Lecturer, Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, United Kingdom. emma.culver@nhs.net
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Review
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastroenterol. Jun 21, 2020; 26(23): 3126-3144 Published online Jun 21, 2020. doi: 10.3748/wjg.v26.i23.3126
Review of primary sclerosing cholangitis with increased IgG4 levels
Charis D Manganis, Roger W Chapman, Emma L Culver
Charis D Manganis, Roger W Chapman, Emma L Culver, Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom
Author contributions: Manganis CD and Culver EL performed the literature review and drafted the original manuscript; Chapman RW and Culver EL were responsible for conception and design of the study, critical revision and editing of the manuscript; all authors approved the final manuscript.
Supported bythe National Institute of Health Research Biomedical Research Centre, based at Oxford University Hospitals Trust; Oxfordshire Health Service Research Committee as part of Oxford Hospitals Charity, Oxford.
Conflict-of-interest statement: There are no commercial or financial conflicts of interest related to this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Emma L Culver, BSc, MBChB, DPhil, MRCP Gastro, Consultant Hepatologist and Senior Lecturer, Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, United Kingdom. emma.culver@nhs.net
Received: December 23, 2019 Peer-review started: December 23, 2019 First decision: March 23, 2020 Revised: April 7, 2020 Accepted: June 5, 2020 Article in press: June 5, 2020 Published online: June 21, 2020 Processing time: 180 Days and 23.2 Hours
Core Tip
Core tip: Elevated serum IgG4 titres and/or abundant IgG4-positive plasma cell infiltrates in the liver and bile ducts are present in up-to one-quarter of patients with primary sclerosing cholangitis (PSC). It should be distinguished from IgG4-related sclerosing cholangitis. PSC with high IgG4 have a different clinical phenotype to those with normal IgG4, with higher bilirubin and alkaline phosphatase, higher PSC Mayo Risk Score, more advanced liver disease, shorter time to liver transplantation, more aggressive colitis and shorter time to colectomy. Corticosteroids may provide biochemical improvement but rarely results in cholangiographic resolution. Concerns include steroid toxicity, infection and disease progression in those with advanced cirrhosis.
