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IgG4-Related Disease pp 115–128Cite as

IgG4-Related Disease and Innate Immunity

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

Abstract

An increased number of clinicopathological studies on autoimmune pancreatitis, cholangitis, and sialoadenitis have led to the recognition of immunoglobulin G4-related disease (IgG4-RD) as a novel disorder, characterized by elevated levels of serum IgG4 and infiltration of IgG4-expressing plasma cells in the affected organs. Although the immunological background associated with the development of IgG4-RD remains poorly understood, recent studies have suggested involvement of the innate immune response in its pathogenesis. Peripheral blood innate immune cells, such as plasmacytoid dendritic cells and monocytes isolated from patients with IgG4-RD, promote IgG4 production by B cells. Activation of the innate immune response by microbe- and/or damage-associated molecular patterns stimulates production of type I interferon and B cell-activating factor by innate immune cells and results in IgG4 production by B cells. Elucidation of the innate immune response associated with IgG4-RD may help identify a new therapeutic target for this immune disorder.

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Acknowledgments

This work was supported by Grant-in-Aid for Scientific Research (25293172, 15K15370) from the Japan Society for the Promotion of Science; the Kato Memorial Trust for Nambyo Research; and the Health and Labor Sciences Research Grants for Research on Intractable Diseases from the Ministry of Health, Labor and Welfare, Japan.

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Authors and Affiliations

  1. Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2, Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan

    Tomohiro Watanabe & Masatoshi Kudo

  2. Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, 606-8507, Japan

    Kouhei Yamashita

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  1. Tomohiro Watanabe
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  2. Kouhei Yamashita
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  3. Masatoshi Kudo
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Correspondence to Tomohiro Watanabe .

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

    Kazuichi Okazaki

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Watanabe, T., Yamashita, K., Kudo, M. (2016). IgG4-Related Disease and Innate Immunity. In: Okazaki, K. (eds) IgG4-Related Disease. Current Topics in Microbiology and Immunology, vol 401. Springer, Cham. https://doi.org/10.1007/82_2016_42

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