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.

中文翻译：

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2019年美国风湿病学会/欧洲抗风湿病联盟IgG4相关疾病分类标准

IgG4 相关疾病 (IgG4-RD) 几乎可以在任何器官中引起纤维炎症病变。诊断需要临床、血清学、放射学和病理学数据之间的相关性。这项工作的目的是开发和验证一套国际 IgG4-RD 分类标准。美国风湿病学会 (ACR) 和欧洲抗风湿病联盟 (EULAR) 组建了一个由 86 名医生组成的国际多专业小组。调查人员采用共识练习；现有文献；1879 名受试者（1086 例，793 名模仿者）的推导和验证队列；多标准决策分析，以识别、衡量和测试潜在的分类标准。包括两个独立的验证队列。开发了三步分类过程。首先，必须证明潜在的 IgG4-RD 病例以与 IgG4-RD 一致的方式涉及 11 个可能器官中的至少一个。其次，必须采用总共32个临床、血清学、放射学和病理学项目组成的排除标准；任何这些标准的存在都会将患者排除在 IgG4-RD 分类之外。第三，应用了八个加权纳入标准领域，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，20 个点的阈值的特异性为 99.2%（95% CI 97.2% 至 99.8%），敏感性为 85.5%（95% CI 81.9% 至 88.5%）。第二个样本的特异性为 97.8%（95% CI 93.7% 至 99.2%），敏感性为 82.0%（95% CI 77.0% 至 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征。IgG4-RD 的 ACR/EULAR 分类标准已经制定并在大量患者中得到验证。这些标准展示了出色的测试性能，应该对未来的临床、流行病学和基础科学研究做出重大贡献。