IgG4 cholangiopathy: current concept, diagnosis, and pathogenesis.

Abstract

IgG4 related cholangiopathy, a distinctive type of cholangitis of unknown origin, is characterized by increased serum levels of IgG4, massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis in the thickened bile duct wall, and good response to steroids. Patients with IgG4-cholangiopathy are frequently associated with autoimmune pancreatitis; IgG4-cholangiopathy is recognized as a biliary manifestation of IgG4-related disease. This condition can be diagnosed by a combination of imaging, serology, histopathology, and steroid responsiveness; however, cholangiographic features are often difficult to differentiate from primary sclerosing cholangitis, pancreatic cancer, or cholangiocarcinoma. The Japanese clinical diagnostic criteria for IgG4-related sclerosing cholangitis established in 2012 are useful in the diagnosis of IgG4-cholangiopathy. Although the precise pathogenic mechanism remains unclear, the development of IgG4-cholangiopathy may involve: susceptible genetic factors, abnormal innate and acquired immunity, decreased naïve regulatory T cells, and specific B cell responses. Further studies on genetic backgrounds, disease specific antigens, and the role of IgG4 are necessary to clarify the pathogenesis.

• Authors: Kazuichi Okazaki, Kazushige Uchida, Masanori Koyabu, Hideaki Miyoshi, Tsukasa Ikeura, Makoto Takaoka
• Journal: Journal of hepatology (J Hepatol) Vol. 61 Issue 3 Pg. 690-5 (Sep 2014) ISSN: 1600-0641 [Electronic] Netherlands
• PMID: 24768756 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2014. Published by Elsevier B.V.

Chemical References

• Immunoglobulin G

Topics

• Adaptive Immunity (physiology)
• Bile Duct Diseases (diagnosis, etiology, immunology)
• Humans
• Immunity, Innate (physiology)
• Immunoglobulin G (blood)
• Liver (immunology, pathology)
• Plasma Cells (immunology, pathology)