title: IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity.
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.
authors.affiliation: 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.
authors.forename: Hisanori
authors.initials: H
authors.lastname: Umehara
authors.affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan.
authors.forename: Akio
authors.initials: A
authors.lastname: Nakajima
authors.affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan.
authors.forename: Takuji
authors.initials: T
authors.lastname: Nakamura
authors.affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kawanami
authors.affiliation: Department of Internal Medicine, Division of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa 920-0293, Japan.
authors.forename: Masao
authors.initials: M
authors.lastname: Tanaka
authors.affiliation: Department of Hematology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei 430030, China.
authors.forename: Lingli
authors.initials: L
authors.lastname: Dong
authors.affiliation: Department of Internal Medicine, Division of Rheumatology, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa 920-8641, Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kawano
mesh_terms: D056704:Adaptive Immunity; D001402:B-Lymphocytes / Q000276:immunology / Q000378:metabolism; D002450:Cell Communication; D005355:Fibrosis; D006801:Humans; D007154:Immune System Diseases / Q000175:diagnosis / Q000209:etiology*; D007113:Immunity, Innate; D017578:Immunoglobulin Class Switching; D007074:Immunoglobulin G / Q000276:immunology*
chemical_list: D007074:Immunoglobulin G
keywords: IgG4; Th2; regulatory T cell; toll-like receptor