IgG4-related disease (IgG4-RD) and its pathogenesis-Crosstalk between Innate and Acquired immunity

• International Immunology
• Advance Access
• 10.1093/intimm/dxu074

1. Hisanori Umehara¹,²⁾,^*,^**, 
2. Akio Nakajima¹⁾, 
3. Takuji Nakamura¹⁾, 
4. Takafumi Kawanami¹⁾,
5. Masao Tanaka¹⁾, 
6. Lingli Dong³⁾ and 
7. Mitsuhiro Kawano⁴⁾

+Author Affiliations

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

1. ↵^* Corresponding author: Hisanori Umehara Contact email: umehara606@gmail.comDepartment of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kawahara-cho 54 Shougoin, Sakyo-ku, Kyoto 606-8501, Japan, Tel: +81-75-751-4379, FAX: +81-75-751-4338
2. ↵^** Present address: Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501

• Received October 26, 2013.

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 elevated serum IgG4 concentration and tumefaction by IgG4-positive plasma cells. Although the clinical manifestations in various organs have been established, the pathogenesis of IgG4-RD is still unknown. Recently, many reports in aberrant acquired immunity such as Th2-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 crosstalk between innate and acquired immunity.

Key words

•    Toll-like receptors
 
• regulatory T cell
 
• Th2
 
• IgG4

This Article

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

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