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.

中文翻译：

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2019年美国风湿病学院/欧洲风湿病联盟针对IgG4相关疾病的分类标准。

目的IgG4相关疾病（IgG4-RD）可以在几乎任何器官中引起纤维炎性病变。诊断需要临床，血清学，放射学和病理学数据之间的相关性。开展这项工作是为了开发和验证IgG4-RD的国际分类标准集。方法由美国风湿病学院（ACR）和欧洲抗风湿病联盟（EULAR）组成的由86名医师组成的国际性多专业小组。研究人员使用共识演习，现有文献，1879名受试者（1,086例，793名模仿者）的推导和验证队列以及多标准决策分析来确定，衡量和测试潜在的分类标准。包括两个独立的验证队列。结果开发了一个三步分类过程。第一的，必须证明，潜在的IgG4-RD病例以与IgG4-RD一致的方式累及了11个可能器官中的至少1个。其次，必须采用排除标准，该排除标准包括总共32个临床，血清学，放射学和病理学项目；这些标准中的任何一项都会使患者脱离IgG4-RD分类。第三，应用8个加权纳入标准域，这些域涉及临床发现，血清学结果，放射学评估和病理学解释。在第一个验证队列中，阈值20分的特异性为99.2％（95％置信区间[95％CI] 97.2-99.8％），灵敏度为85.5％（95％CI 81.9-88.5％）。第二种方法的特异性为97.8％（95％CI 93.7-99.2％），敏感性为82.0％（95％CI 77.0-86.1％）。该标准在各种阈值范围内均具有强大的测试特性。结论IgG4-RD的ACR / EULAR分类标准已经得到开发，并在大量患者中得到了验证。这些标准证明了优异的测试性能，并应为将来的临床，流行病学和基础科学研究做出重大贡献。