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

1. Hisanori Umehara¹,²,⁵, 
2. Akio Nakajima¹, 
3. Takuji Nakamura¹, 
4. Takafumi Kawanami¹,
5. Masao Tanaka¹, 
6. Lingli Dong³ and 
7. 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. Int. Immunol. (2014) 26 (11):585-595.doi: 10.1093/intimm/dxu074First published online: July 14, 2014
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• © The Author 2014. Published by Oxford University Press on behalf of The Japanese Society for Immunology.

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