Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011
Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011
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DOI：
10.1007/s10165-011-0571-z
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发表时间：
2012-02-01
影响因子：
2.2
通讯作者：
Ohara, Hirotaka
中科院分区：
文献类型：
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作者：
Umehara, Hisanori;Okazaki, Kazuichi;Ohara, Hirotaka
IgG 4相关疾病（IgG 4-RD）是一种以血清IgG 4浓度升高和IgG 4+浆细胞肿胀或组织浸润为特征的新型临床疾病。尽管IgG 4-RD并不罕见且具有临床重要性，但其临床诊断标准尚未建立。IgG 4-RD的综合诊断标准，包括各种器官的参与，旨在为普通医生和非专业人员的实际使用。两个IgG 4-RD研究小组，梅原和冈崎小组，由日本厚生劳动省组织。由于IgG 4-RD包含多种疾病，这些小组由日本各地的各个学科的医生和研究人员组成，包括风湿病学、血液学、胃肠病学、肾脏病学、肺病学、眼科学、牙科学、病理学、统计学以及基础和分子免疫学，梅原和冈崎团队分别有66名和56名成员。两个研究小组的合作包括对IgG 4-RD患者的临床症状、实验室检查结果和活检标本进行详细分析，从而建立了IgG 4-RD的综合诊断标准。尽管许多IgG 4-RD患者同时或异时出现多个器官病变，且每个器官的病理特征不同，对于IgG 4 RD的两个诊断标准已经达成共识：（1）血清IgG 4浓度> 135 mg/dl，和（2）> 40%的IgG+浆细胞是IgG 4+且> 10个细胞/活检样品的高倍视野。虽然综合诊断标准对于诊断1型IgG 4相关自身免疫性胰腺炎（IgG 4相关AIP）不够敏感，但对于IgG 4相关Mikulicz病（MD）和肾脏疾病（KD）具有足够的敏感性。此外，综合诊断标准，结合器官特异性诊断标准，提高了诊断的灵敏度为100%的IgG 4相关的MD，KD和AIP。我们的IgG 4-RD的综合诊断标准是实用的一般医生和非专业人士。
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.