Table of Contents
Advances in Hepatology
Volume 2014, Article ID 632320, 7 pages
Review Article

IgG4 Related Sclerosing Cholangitis

Department of Gastroenterology, University College London Hospital, 250 Euston Road, London NW1 2PG, UK

Received 7 August 2014; Revised 13 October 2014; Accepted 15 October 2014; Published 10 November 2014

Academic Editor: Sharon DeMorrow

Copyright © 2014 D. Joshi and G. J. M. Webster. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


IgG4 related disease (IgG4-RD) is a multisystemic disorder which has only recently been recognized. IgG4 related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the disease, often in association with autoimmune pancreatitis (AIP). In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC that need to be considered, namely, primary sclerosing cholangitis (PSC) and cholangiocarcinoma, and provide a management algorithm. Finally, we highlight future directions and unanswered questions which will provide new insights into this exciting and evolving disease entity.