Vol.22 No.1

Review Article

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

Authors

Hisanori Umehara1 , Kazuichi Okazaki2 , Yasufumi Masaki1 , Mitsuhiro Kawano3 , Motohisa Yamamoto4 , Takako Saeki5 , Shoko Matsui6 , Takayuki Sumida7 , Tsuneyo Mimori8 , Yoshiya Tanaka9 , Kazuo Tsubota10 , Tadashi Yoshino11, Shigeyuki Kawa12, Ritsuro Suzuki13, Tsutomu Takegami14, Naohisa Tomosugi15, Nozomu Kurose16, Yasuhito Ishigaki17, Atsushi Azumi18, Masaru Kojima19, Shigeo Nakamura20, Dai Inoue21

  • Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, 920-0293, Ishikawa, Japan
  • Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan
  • Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan
  • The First Department of Internal Medicine, Sapporo Medical University, Sapporo, Hokkaido, Japan
  • Department of Internal Medicine, Nagaoka Red Cross Hospital, Niigata, Japan
  • Health Administration Center, Sugitani Campus, University of Toyama, Toyama, Japan
  • Department of Clinical Immunology, Doctoral Programs in Clinical Science, Graduate School of Comprehensive Human Science, University of Tuskuba, Ibaraki, Japan
  • Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan
  • First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Fukuoka, Japan
  • Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan
  • Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
  • Center for Health, Safety and Environmental Management, Shinshu University, Nagano, Japan
  • Department of HSCT Data Management and Biostatistics, Graduate School of Medicine, Nagoya University, Nagoya, Japan
  • Division of Molecular Oncology and Virology, Medical Research Institute, Kanazawa Medical University, Ishikawa, Japan
  • Department of Advanced Medicine, Medical Research Institute, Kanazawa Medical University, Ishikawa, Japan
  • Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Ishikawa, Japan
  • Division of Core Facility, Medical Research Institute, Kanazawa Medical University, Ishikawa, Japan
  • Ophthalmology Department, Kobe Kaisei Hospital, Hyogo, Japan
  • Department of Anatomic and Diagnostic Pathology, Dokkyo University School of Medicine, Mibu, Japan
  • Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan
  • Department of Radiology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan
Received:

17 May 2011

Accepted:

28 July 2011

Published online:

1 September 2011

Full Text

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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.

Key words

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