IgG4-related disease and its pathogenesis— cross-talk between innate and acquired immunity

Hisanori Umehara1,2,5, Akio Nakajima1, Takuji Nakamura1, Takafumi Kawanami1, Masao Tanaka1, Lingli Dong3 and Mitsuhiro Kawano4

+Author Affiliations

1. ¹ Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan
2. ² Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan
3. ³ Department of Hematology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China
4. ⁴ Department of Internal Medicine, Division of Rheumatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa 920-8641, Japan
5. ⁵Present address: Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan

1. Correspondence to: H. Umehara; E-mail: umehara606@gmail.com

• Received October 26, 2013.
• Revision received July 2, 2014.
• Accepted July 3, 2014.

Abstract

IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world. The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4⁺ plasma cells. Although the clinical manifestations in various organs have been established, the pathogenesis of IgG4-RD is still unknown. Recently, many reports of aberrant acquired immunity such as T_h2-diminated immune responses have been published. However, many questions still remain, including questions about the pathogenesis of IgG4-RD and the roles of IgG4. In this review, we discuss the pathogenesis of IgG4-RD by focusing on the cross-talk between innate and acquired immunity.

Key words

• • 
    
• IgG4
 
• regulatory T cell
 
• T_h2
 
• toll-like receptor

This Article

1. 
   
2. Int. Immunol. (2014)doi: 10.1093/intimm/dxu074First published online: July 14, 2014

1. This article is Open Access
3. 
   
4. Full Text (HTML)Free
5. Full Text (PDF)Free
6. All Versions of this Article:

   1. dxu074v1
   2. dxu074v2 most recent

© The Author 2014. Published by Oxford University Press on behalf of The Japanese Society for Immunology.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com