OBJECTIVE This study aimed to compare the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria with the European Medicines Agency (EMA) algorithm for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS All consecutive, newly diagnosed patients with AAV according to the 2012 Chapel Hill Consensus Conference who visited Keio University Hospital between March 2012 and May 2022 were retrospectively reviewed. Patients were reclassified according to the EMA algorithm and the 2022 ACR/EULAR criteria, and their clinical characteristics were statistically analyzed. RESULTS A total of 114 patients with AAV were included in the analyses. Using the EMA algorithm as a reference, reclassification of the patients revealed sensitivity and specificity of the 2022 ACR/EULAR criteria of 100% and 96% for eosinophilic granulomatosis with polyangiitis, 40% and 97% for granulomatosis with polyangiitis (GPA), and 90% and 49% for microscopic polyangiitis (MPA), respectively. Approximately half of patients classified as EMA-GPA or EMA-unclassifiable were reclassified as 2022-MPA; these patients were older, were more disposed to be positive for myeloperoxidase (MPO)-ANCA, and had interstitial lung disease (ILD) more frequently than patients with 2022-GPA or non-2022-MPA. Further, some patients positive for MPO-ANCA with biopsy-proven granulomatous inflammation were also reclassified from EMA-GPA to 2022-MPA. Over the mean observation period of 4.0 years, 16 patients died. Overall survival for each classification group differed significantly from the 2022 ACR/EULAR criteria (P = 0.02), but not with the EMA algorithm (P = 0.21). CONCLUSION Among the patients classified as EMA-GPA or EMA-unclassifiable, older patients with MPO-ANCA and ILD tended to be reclassified as 2022-MPA. The 2022 ACR/EULAR criteria were more useful in prognostic prediction than the EMA algorithm.

中文翻译：

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2022 年 ACR/EULAR 分类标准与欧洲药品管理局算法在抗中性粒细胞胞浆抗体相关血管炎中的表现比较。


目的 本研究旨在比较 2022 年美国风湿病学会 （ACR）/欧洲风湿病协会联盟 （EULAR） 分类标准与欧洲药品管理局 （EMA） 抗中性粒细胞胞质抗体 （ANCA） 相关血管炎 （AAV） 算法。方法 回顾性回顾了 2012 年 3 月至 2022 年 5 月期间访问庆应义塾大学医院的所有根据 2012 年教堂山共识会议连续新诊断的 AAV 患者。根据 EMA 算法和 2022 年 ACR/EULAR 标准对患者进行重新分类，并对其临床特征进行统计分析。结果 共纳入 114 例 AAV 患者。以 EMA 算法为参考，对患者进行重新分类揭示了 2022 年 ACR/EULAR 标准对嗜酸性肉芽肿性多血管炎的敏感性和特异性分别为 100% 和 96%，对肉芽肿性多血管炎 （GPA） 的敏感性和 97%，对显微镜下多血管炎 （MPA） 的敏感性和特异性分别为 90% 和 49%。大约一半被归类为 EMA-GPA 或 EMA 无法分类的患者被重新归类为 2022-MPA;这些患者年龄较大，髓过氧化物酶 （MPO）-ANCA 更易呈阳性，并且比 2022-GPA 或非 2022-MPA 患者更频繁地患有间质性肺病 （ILD）。此外，一些 MPO-ANCA 阳性且活检证实为肉芽肿性炎症的患者也从 EMA-GPA 重新分类为 2022-MPA。在 4.0 年的平均观察期内，16 名患者死亡。每个分类组的总生存期与 2022 年 ACR/EULAR 标准 （P = 0.02） 显著不同，但与 EMA 算法不同 （P = 0.21）。 结论 在归类为 EMA-GPA 或 EMA 无法分类的患者中，患有 MPO-ANCA 和 ILD 的老年患者往往被重新归类为 2022-MPA。2022 年 ACR/EULAR 标准在预后预测方面比 EMA 算法更有用。