The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease
The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease
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DOI：
10.1136/annrheumdis-2019-216561
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发表时间：
2020-01-01
影响因子：
27.4
通讯作者：
Zhang, Wen
中科院分区：
文献类型：
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作者：
Wallace, Zachary S.;Naden, Ray P.;Zhang, Wen
igg4相关疾病（IgG4-RD）可引起几乎任何器官的纤维炎性病变。诊断需要临床、血清学、放射学和病理资料的相关性。开展这项工作是为了制定和验证IgG4-RD的一套国际分类标准。由美国风湿病学会（ACR）和欧洲抗风湿病联盟（EULAR）召集了一个由86名医生组成的国际多专业小组。调查人员使用共识练习；现有文献;衍生和验证队列1879名受试者（1086例，793名模仿者）；并对多准则决策分析进行识别、权重和测试潜力分类准则。纳入两个独立验证队列。开发了一个三步分类过程。首先，必须证明潜在的IgG4-RD病例以与IgG4-RD一致的方式累及了11个可能的器官中的至少一个。第二，必须采用临床、血清学、放射学和病理学共32项的排除标准；任何这些标准的存在都排除了患者的IgG4-RD分类。第三，采用八个加权纳入标准域，包括临床表现、血清学结果、放射学评估和病理解释。在第一个验证队列中，阈值为20点的特异性为99.2% (95% CI 97.2%至99.8%)，敏感性为85.5% （95% CI 81.9%至88.5%）。第二组特异性为97.8% (95% CI 93.7% ~ 99.2%)，敏感性为82.0% （95% CI 77.0% ~ 86.1%）。该标准被证明在广泛的阈值范围内具有稳健的测试特性。IgG4-RD的ACR/EULAR分类标准已经制定并在大量患者中得到验证。这些标准表现出优异的测试性能，应该对未来的临床、流行病学和基础科学调查做出重大贡献。
IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.