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Roles of Regulatory T and B Cells in IgG4-Related Disease

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IgG4-Related Disease

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 401))

Abstract

Immunoglobulin G4 (IgG4) -related disease (RD) is a newly recognized systemic disease. Although there are several forms of IgG4-RD reported under various names, depending on the target organ and characteristics, patients with IgG4-RD manifest several immunologic and histologic abnormalities including increased levels of serum IgG4 and storiform fibrosis with infiltration of lymphocytes and IgG4-positive plasmacytes in the involved organs. However, the pathophysiology remains unclear. Regulatory immune cells play an important role in several immune-related diseases. In particular, abnormalities in regulatory T cell (Treg) and regulatory B cell (Breg) numbers and function are implicated in several immune-related (include autoimmune) conditions, and their roles in IgG4-RD have recently begun to be investigated. We provide an overview of the research conducted to date on Tregs and Bregs in IgG4-RD. We highlight the basic functions of these cells, their changes in patients with various forms of IgG4-RD, and insight gained from animal models of the disease. Based on the evidence accumulated thus far, we proposed a hypothesis for the pathophysiological mechanism of IgG4-RD with respect to the roles regulatory immune cells, and highlight the questions and venues of research deserving of further attenuation, Over all, we demonstrate that Tregs and Bregs have a clear impact on IgG4-RD, and further exploration of this field is expected to lead to a better mechanistic understanding of the disease, hopefully resulting in the in the discovery of new therapeutic targets.

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Acknowledgments

This study was partially supported by (1) JSPS KAKENHI Grant Numbers 26461038, 15K09052, and (2) a Grant-in-Aid for “Research for Intractable Diseases” Program from the Ministry of Labor and Welfare of Japan.

Figure 1 was reprinted from Pancreas, Okazaki K, Uchida K. Autoimmune Pancreatitis: The Past, Present, and Future. 1006–1613, 2015, with permission from Wolters Kluwer Health Inc.

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  1. Department of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, 573-1197, Osaka, Japan

    Kazushige Uchida

  2. Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Japan

    Kazushige Uchida & Kazuichi Okazaki

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  1. Kazushige Uchida
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  2. Kazuichi Okazaki
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Correspondence to Kazushige Uchida .

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  1. Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Osaka, Japan

    Kazuichi Okazaki

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Uchida, K., Okazaki, K. (2016). Roles of Regulatory T and B Cells in IgG4-Related Disease. In: Okazaki, K. (eds) IgG4-Related Disease. Current Topics in Microbiology and Immunology, vol 401. Springer, Cham. https://doi.org/10.1007/82_2016_41

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