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International Journal of Hepatology
Volume 2012 (2012), Article ID 472376, 6 pages
Review Article

IgG4 Cholangiopathy

1Institute of Liver Studies, King’s College Hospital, Denmark Hill, London SE5 9RS, UK
2Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa 920-8640, Japan

Received 25 March 2011; Accepted 19 June 2011

Academic Editor: A. J. Demetris

Copyright © 2012 Yoh Zen and Yasuni Nakanuma. 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 cholangiopathy can involve any level of the biliary tree which exhibits sclerosing cholangitis or pseudotumorous hilar lesions. Most cases are associated with autoimmune pancreatitis, an important diagnostic clue. Without autoimmune pancreatitis, however, the diagnosis of IgG4-cholangiopathy is challenging. Indeed such cases have been treated surgically. IgG4-cholangiopathy should be diagnosed based on serological examinations including serum IgG4 concentrations, radiological features, and histological evidence of IgG4+ plasma cell infiltration. Steroid therapy is very effective even at disease relapse. A Th2-dominant immune response or the activation of regulatory T cells seems to be involved in the underlying immune reaction. It is still unknown why IgG4 levels are specifically elevated in patients with this disease. IgG4 might be secondarily overexpressed by Th2 or regulatory cytokines given the lack of evidence that IgG4 is an autoantibody.