title: A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details.
abstract: 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. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz's disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor. Although IgG4RD forms a distinct, clinically independent disease category and is attracting strong attention as a new clinical entity, many questions and problems still remain to be elucidated, including its pathogenesis, the establishment of diagnostic criteria, and the role of IgG4. Here we describe the concept of IgG4RD and up-to-date information on this emerging disease entity.
authors: Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun 920-0293, Ishikawa, Japan. umehara@kanazawa-med.ac.jp
authors: Hisanori
authors: H
authors: Umehara
authors: Kazuichi
authors: K
authors: Okazaki
authors: Yasufumi
authors: Y
authors: Masaki
authors: Mitsuhiro
authors: M
authors: Kawano
authors: Motohisa
authors: M
authors: Yamamoto
authors: Takako
authors: T
authors: Saeki
authors: Shoko
authors: S
authors: Matsui
authors: Takayuki
authors: T
authors: Sumida
authors: Tsuneyo
authors: T
authors: Mimori
authors: Yoshiya
authors: Y
authors: Tanaka
authors: Kazuo
authors: K
authors: Tsubota
authors: Tadashi
authors: T
authors: Yoshino
authors: Shigeyuki
authors: S
authors: Kawa
authors: Ritsuro
authors: R
authors: Suzuki
authors: Tsutomu
authors: T
authors: Takegami
authors: Naohisa
authors: N
authors: Tomosugi
authors: Nozomu
authors: N
authors: Kurose
authors: Yasuhito
authors: Y
authors: Ishigaki
authors: Atsushi
authors: A
authors: Azumi
authors: Masaru
authors: M
authors: Kojima
authors: Shigeo
authors: S
authors: Nakamura
authors: Dai
authors: D
authors: Inoue
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood* / Q000175:diagnosis / Q000276:immunology; D005260:Female; D017408:Guidelines as Topic; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008882:Mikulicz' Disease / Q000097:blood / Q000276:immunology / Q000473:pathology; D009928:Organ Specificity; D010195:Pancreatitis / Q000097:blood / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D009626:Terminology as Topic
chemical_list: D007074:Immunoglobulin G