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

被引：1492

作者：

Umehara, Hisanori ^{[1
,2
]}

Okazaki, Kazuichi ^{[3
]}

Masaki, Yasufumi ^{[2
]}

Kawano, Mitsuhiro ^{[4
]}

Yamamoto, Motohisa ^{[5
]}

Saeki, Takako ^{[6
]}

Matsui, Shoko ^{[7
]}

Yoshino, Tadashi ^{[8
]}

Nakamura, Shigeo

Kawa, Shigeyuki ^{[9
]}

Hamano, Hideaki ^{[10
]}

Kamisawa, Terumi ^{[11
]}

Shimosegawa, Toru ^{[12
]}

Shimatsu, Akira ^{[13
]}

Nakamura, Seiji

Ito, Tetsuhide ^{[14
]}

Notohara, Kenji ^{[15
,16
]}

Sumida, Takayuki ^{[17
]}

Tanaka, Yoshiya ^{[18
]}

Mimori, Tsuneyo ^{[19
]}

Chiba, Tsutomu ^{[20
]}

Mishima, Michiaki ^{[21
]}

Hibi, Toshifumi ^{[22
]}

Tsubouchi, Hirohito ^{[23
]}

Inui, Kazuo ^{[24
]}

Ohara, Hirotaka ^{[25
]}

机构：

[1] Kanazawa Med Univ, Div Hematol & Immunol, Dept Internal Med, Kanazawa, Ishikawa 9200293, Japan

[2] Kanazawa Med Univ, Dept Hematol & Immunol, Kanazawa, Ishikawa 9200293, Japan

[3] Kansai Med Univ, Div Gastroenterol & Hepatol, Dept Internal Med 3, Hirakata, Osaka 5731191, Japan

[4] Kanazawa Univ, Div Rheumatol, Dept Internal Med, Grad Sch Med Sci, Kanazawa, Ishikawa, Japan

[5] Sapporo Med Univ, Dept Internal Med 1, Sapporo, Hokkaido, Japan

[6] Nagaoka Red Cross Hosp, Dept Internal Med, Nagaoka, Niigata, Japan

[7] Toyama Univ, Hlth Adm Ctr, Toyama 930, Japan

[8] Okayama Univ, Dept Pathol, Grad Sch Med Dent & Pharmaceut Sci, Okayama 7008530, Japan

[9] Shinshu Univ, Ctr Hlth Safety & Environm Management, Matsumoto, Nagano 390, Japan

[10] Shinshu Univ, Sch Med, Dept Gastroenterol, Matsumoto, Nagano 390, Japan

[11] Tokyo Metropolitan Komagome Hosp, Dept Internal Med, Tokyo, Japan

[12] Tohoku Univ, Div Gastroenterol, Grad Sch Med, Sendai, Miyagi 980, Japan

[13] Natl Hosp Org, Clin Res Inst, Kyoto Med Ctr, Kyoto, Japan

[14] Kyushu Univ, Dept Med & Bioregulatory Sci, Grad Sch Med Sci, Fukuoka 812, Japan

[15] Kurashiki Cent Hosp, Dept Anat Pathol, Kurashiki, Okayama, Japan

[16] Kanazawa Med Univ, Dept Pathol & Lab Med, Kanazawa, Ishikawa 9200293, Japan

[17] Univ Tsukuba, Doctoral Programs Clin Sci, Dept Clin Immunol, Grad Sch Comprehens Human Sci, Tsukuba, Ibaraki, Japan

[18] Univ Occupat & Environm Hlth, Dept Internal Med 1, Sch Med, Fukuoka, Japan

[19] Kyoto Univ, Dept Rheumatol & Clin Immunol, Grad Sch Med, Kyoto, Japan

[20] Kyoto Univ, Dept Gastroenterol & Hepatol, Grad Sch Med, Kyoto, Japan

[21] Kyoto Univ, Dept Resp Med, Grad Sch Med, Kyoto, Japan

[22] Keio Univ, Div Gastroenterol & Hepatol, Dept Internal Med, Sch Med, Tokyo, Japan

[23] Kagoshima Univ, Div Digest & Lifestyle Dis, Dept Human & Environm Sci, Grad Sch Med & Dent Sci, Kagoshima 890, Japan

[24] Fujita Hlth Univ, Dept Internal Med, Teaching Hosp 2, Toyoake, Aichi, Japan

[25] Nagoya City Univ, Dept Internal Med & Bioregulat, Grad Sch Med Sci, Nagoya, Aichi, Japan

来源：

MODERN RHEUMATOLOGY | 2012年 / 22卷 / 01期

关键词：

IgG4-related disease; Criteria; Mikulicz's disease; Autoimmune pancreatitis; Interstitial nephritis; RETROPERITONEAL FIBROSIS; AUTOIMMUNE PANCREATITIS; SCLEROSING PANCREATITIS; SYSTEMIC-DISEASE; SERUM; CHOLANGITIS; LUNG;

D O I：

10.1007/s10165-011-0571-z

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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. 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. 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. Our comprehensive diagnostic criteria for IgG4-RD are practically useful for general physicians and nonspecialists.

引用

页码：21 / 30

页数：10

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