TY - JOUR
T1 - Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011
AU - Umehara, Hisanori
AU - Okazaki, Kazuichi
AU - Masaki, Yasufumi
AU - Kawano, Mitsuhiro
AU - Yamamoto, Motohisa
AU - Saeki, Takako
AU - Matsui, Shoko
AU - Yoshino, Tadashi
AU - Nakamura, Shigeo
AU - Kawa, Shigeyuki
AU - Hamano, Hideaki
AU - Kamisawa, Terumi
AU - Shimosegawa, Toru
AU - Shimatsu, Akira
AU - Nakamura, Seiji
AU - Ito, Tetsuhide
AU - Notohara, Kenji
AU - Sumida, Takayuki
AU - Tanaka, Yoshiya
AU - Mimori, Tsuneyo
AU - Chiba, Tsutomu
AU - Mishima, Michiaki
AU - Hibi, Toshifumi
AU - Tsubouchi, Hirohito
AU - Inui, Kazuo
AU - Ohara, Hirotaka
N1 - Funding Information:
Acknowledgments This work was supported by Intractable Diseases, the Health and Labor Sciences Research Grants from Ministry of Health, Labor and Welfare, Japan. We sincerely thank the many contributing researchers and collaborators who participated in the All Japan IgG4 team.
PY - 2012/2
Y1 - 2012/2
N2 - 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 involveddetailed 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.
AB - 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 involveddetailed 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.
KW - Autoimmune pancreatitis
KW - Criteria
KW - IgG4-related disease
KW - Interstitial nephritis
KW - Mikulicz's disease
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U2 - 10.1007/s10165-011-0571-z
DO - 10.1007/s10165-011-0571-z
M3 - Article
C2 - 22218969
AN - SCOPUS:84861483241
SN - 1439-7595
VL - 22
SP - 21
EP - 30
JO - Japanese Journal of Rheumatology
JF - Japanese Journal of Rheumatology
IS - 1
ER -