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：
10.1002/art.41120
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发表时间：
2019-12-02
影响因子：
13.3
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
目的igg4相关疾病（IgG4-RD）可引起几乎所有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。方法由美国风湿病学会（ACR）和欧洲抗风湿病联盟（EULAR）组成的国际多专科组86名医生。研究者使用共识练习、现有文献、衍生和验证1,879名受试者（1,086例病例，793名模仿者）的队列，以及多标准决策分析来确定、加权和测试潜在的分类标准。纳入两个独立验证队列。结果建立了三步分类方法。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。其次，必须采用由临床、血清学、放射学和病理学共32项组成的排除标准；任何这些标准的存在都排除了患者的IgG4-RD分类。第三，应用了8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%置信区间[95% CI] 97.2-99.8%)，敏感性为85.5% （95% CI 81.9-88.5%）。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。结论IgG4-RD的ACR/EULAR分类标准已经建立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，并将对未来的临床、流行病学和基础科学调查做出重大贡献。
Objective IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods 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 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2-99.8%) and a sensitivity of 85.5% (95% CI 81.9-88.5%). In the second, the specificity was 97.8% (95% CI 93.7-99.2%) and the sensitivity was 82.0% (95% CI 77.0-86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion 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, epidemiologic, and basic science investigations.