Vol.22 No.1

Original Article

Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011

Authors

Hisanori Umehara1,26 , Kazuichi Okazaki2 , Yasufumi Masaki1 , Mitsuhiro Kawano3 , Motohisa Yamamoto4 , Takako Saeki5 , Shoko Matsui6 , Tadashi Yoshino7 , Shigeo Nakamura8 , Shigeyuki Kawa9 , Hideaki Hamano10 , Terumi Kamisawa11, Toru Shimosegawa12, Akira Shimatsu13, Seiji Nakamura14, Tetsuhide Ito15, Kenji Notohara16,27, Takayuki Sumida17, Yoshiya Tanaka18, Tsuneyo Mimori19, Tsutomu Chiba20, Michiaki Mishima21, Toshifumi Hibi22, Hirohito Tsubouchi23, Kazuo Inui24, Hirotaka Ohara25

  • Department of Hematology and Immunology, Kanazawa Medical University, Kanazawa, Ishikawa, Japan
  • Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Hirakata, Osaka, 573-1191, Japan
  • Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medical Science, Kanazawa, Ishikawa, Japan
  • The First Department of Internal Medicine, Sapporo Medical University, Sapporo, Hokkaido, Japan
  • Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Niigata, Japan
  • Health Administration Center, Sugitani Campus, University of Toyama, Toyama, Japan
  • Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
  • Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan
  • Center for Health, Safety and Environmental Management, Shinshu University, Matsumoto, Japan
  • Department of Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan
  • Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
  • Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
  • Clinical Research Institute, National Hospital Organization Kyoto Medical Center, Kyoto, Japan
  • Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, Fukuoka, Japan
  • Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  • Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan
  • Doctoral Programs in Clinical Science, Department of Clinical Immunology, Graduate School of Comprehensive Human Science, University of Tsukuba, Tsukuba, Ibaraki, Japan
  • First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Fukuoka, Japan
  • Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
  • Department of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
  • Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
  • Division of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
  • Division of Digestive and Lifestyle Disease, Department of Human and Environmental Sciences, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan
  • Department of Internal Medicine, Second Teaching Hospital, Fujita Health University, Toyoake, Aichi, Japan
  • Department of Internal Medicine and Bioregulation, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan
  • Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, Kanazawa, Ishikawa, 920-0293, Japan
  • Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Kanazawa, Ishikawa, Japan
Received:

14 October 2011

Accepted:

19 November 2011

Published online:

5 January 2012

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Abstract

Background IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4+ plasma cells. Although IgG4-RD is not rare and is clinically important, its clinical diagnostic criteria have not been established. Comprehensive diagnostic criteria for IgG4-RD, including the involvement of various organs, are intended for the practical use of general physicians and nonspecialists.
Methods Two IgG4-RD study groups, the Umehara and Okazaki teams, were organized by the Ministry of Health, Labor and Welfare Japan. As IgG4-RD comprises a wide variety of diseases, these groups consist of physicians and researchers in various disciplines, including rheumatology, hematology, gastroenterology, nephrology, pulmonology, ophthalmology, odontology, pathology, statistics, and basic and molecular immunology throughout Japan, with 66 and 56 members of the Umehara and Okazaki teams, respectively. Collaborations of the two study groups involved detailed analyses of clinical symptoms, laboratory results, and biopsy specimens of patients with IgG4-RD, resulting in the establishment of comprehensive diagnostic criteria for IgG4-RD.
Results Although many patients with IgG4-RD have lesions in several organs, either synchronously or metachronously, and the pathological features of each organ differ, consensus has been reached on two diagnostic criteria for IgG4RD: (1) serum IgG4 concentration[135 mg/dl, and (2)>40% of IgG+ plasma cells being IgG4+ and >10 cells/high powered field of biopsy sample. Although the comprehensive diagnostic criteria are not sufficiently sensitive for the diagnosis of type 1 IgG4-related autoimmune pancreatitis (IgG4-related AIP), they are adequately sensitive for IgG4-related Mikulicz’s disease (MD) and kidney disease (KD). In addition, the comprehensive diagnostic criteria, combined with organ-specific diagnostic criteria, have increased the sensitivity of diagnosis to 100% for IgG4-related MD, KD, and AIP.
Conclusion Our comprehensive diagnostic criteria for IgG4-RD are practically useful for general physicians and nonspecialists.

Key words

IgG4-related disease - Criteria - Mikulicz’s disease - Autoimmune pancreatitis - Interstitial nephritis