A novel clinical entity, IgG4-relateddisease (IgG4RD): General concept and details

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 KojimaShigeo Nakamura, Dai Inoue, Keita Fujikawa, Hideaki Hamano, Keiji Kubo, Hiroshi Yamamoto, Mitsuyosi Hirokawa, Kunihiko Itoh, Terumi Kamisawa, Daisuke Kawabata, Morio Matsumoto, Seijiro Minamoto, Kayoko Murayama, Susumu Nishiyama, Yoko Ogawa, Tomoki Origuchi, Norihide Oyama, Yasuharu Sato, Masao Seto, Susumu Sugai, Norifumi Tsukamoto, Masayuki Takahira, Hiroki Takahashi, Hiroto Tsuboi, Yuko Waseda, Kazuko Kitagawa, Takayuki Nojima, Hitoshi Yokoyama, Hisao Tonami, Toshihiro Fukushima, Masao Tanaka, Yoshimasa Fujita, Toshioki Sawaki, Takafumi Kawanami, Miyuki Miki, Haruka Iwao, Akio Nakajima, Takuji Nakamura

Research output: Contribution to journalReview article

498 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1-14
Number of pages14
JournalModern rheumatology
Volume22
Issue number1
DOIs
Publication statusPublished - 2012 Feb 1

    Fingerprint

Keywords

  • Autoimmune pancreatitis
  • Castleman's disease
  • IgG4-related diseases
  • Mikulicz's disease
  • Sjögren's syndrome

ASJC Scopus subject areas

  • Rheumatology

Cite this

Umehara, H., Okazaki, K., Masaki, Y., Kawano, M., Yamamoto, M., Saeki, T., Matsui, S., Sumida, T., Mimori, T., Tanaka, Y., Tsubota, K., Yoshino, T., Kawa, S., Suzuki, R., Takegami, T., Tomosugi, N., Kurose, N., Ishigaki, Y., Azumi, A., ... Nakamura, T. (2012). A novel clinical entity, IgG4-relateddisease (IgG4RD): General concept and details. Modern rheumatology, 22(1), 1-14. https://doi.org/10.1007/s10165-011-0508-6