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Functions of IgG4, and pathogenesis of IgG4-related disease (IgG4RD) Hisanori Umehara 1 1Department of Hematology and Immunology, Kanazawa Medical University Keyword: IgG4関連疾患 , シェーグレン症候群 , ミクリッツ病 pp.731-735
Published Date 2011/8/15
DOI https://doi.org/10.11477/mf.1542102691
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IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. Many papers concerning IgG4RD have been reported from Japan. IgG4 has a unique characteristic structure and shows Fab exchange involving a pair of heavy and light chains binding another pair. Thus, IgG4 functions as an inhibitor of cellular immunity and allergy. In lesions of IgG4RD, regulatory T cells are increased and Th2 type cytokines such as IL-10 and TGF-β are dominant. These cytokines may contribute to the class switch of immunoglobulin to IgG4 and IgE, and fibrogenesis.


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電子版ISSN 1882-1367 印刷版ISSN 0485-1420 医学書院

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