Article thumbnail

A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details

By Hisanori Umehara, Kazuichi Okazaki, Yasufumi Masaki, Mitsuhiro Kawano, Motohisa Yamamoto, Takako Saeki, Shoko Matsui, Takayuki Sumida, Tsuneyo Mimori, Yoshiya Tanaka, Kazuo Tsubota, Tadashi Yoshino, Shigeyuki Kawa, Ritsuro Suzuki, Tsutomu Takegami, Naohisa Tomosugi, Nozomu Kurose, Yasuhito Ishigaki, Atsushi Azumi, Masaru Kojima, Shigeo Nakamura and Dai Inoue


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

Topics: Review Article
Publisher: Springer Japan
OAI identifier:
Provided by: PubMed Central

Suggested articles


  1. (1982). Uber Eine Eigenartige Symmetrishe Erkrankung Der Tranen Und Mundspeicheldrusen. Stuttgart: BeitrzChirFesrschrf Theodor Billroth;

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.