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Int Immunol. 2014 Nov;26(11):585-95. doi: 10.1093/intimm/dxu074. Epub 2014 Jul 14.

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

Author information

1
Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan Present address: Department of Clinical Immunology, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto 606-8501, Japan umehara606@gmail.com.
2
Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan.
3
Department of Hematology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China.
4
Department of Internal Medicine, Division of Rheumatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa 920-8641, Japan.

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 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 cross-talk between innate and acquired immunity.

KEYWORDS:

IgG4; Th2; regulatory T cell; toll-like receptor

PMID:
25024397
PMCID:
PMC4201844
DOI:
10.1093/intimm/dxu074
[Indexed for MEDLINE]
Free PMC Article

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