National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Maryland, USA.
Hospital de Santa Maria, Centro Hospitalar Universitario Lisboa Norte, Universidade de Lisboa, Lisboa, Portugal.
Ann Rheum Dis. 2022 Mar;81(3):309-314. doi: 10.1136/annrheumdis-2021-221794. Epub 2022 Feb 2.
To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).
Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.
The development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1×10/L (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3-ANCA positivity (-3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1) and haematuria (-1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%).
The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.
制定并验证嗜酸性肉芽肿性多血管炎（EGPA）的修订分类标准。
血管炎或对照疾病患者被纳入国际队列。该研究分为五个阶段：（1）使用共识方法确定候选标准项目，（2）前瞻性收集诊断时存在的候选项目，（3）基于数据减少候选项目数量，（4）专家小组审查病例以定义参考诊断，（5）使用最小绝对收缩和选择算子逻辑回归在开发集中为疾病分类开发基于点的风险评分，随后在独立病例和对照集中验证性能特征。
EGPA 的开发集包括 107 例 EGPA 和 450 例对照。验证集包括另外 119 例 EGPA 和 437 例对照。从 91 个候选项目中，回归分析确定了 11 个用于 EGPA 的项目，其中 7 个保留。最终标准及其权重如下：最大嗜酸性粒细胞计数≥1×10/L（+5）、气道阻塞性疾病（+3）、鼻息肉（+3）、细胞质抗中性粒细胞胞浆抗体（ANCA）或抗蛋白酶 3-ANCA 阳性（-3）、血管外嗜酸性粒细胞为主的炎症（+2）、多发性单神经病/运动神经病非神经根病所致（+1）和血尿（-1）。排除血管炎的模拟物后，如果小或中血管炎患者的累积评分≥6 分，则可诊断为 EGPA。当这些标准在验证数据集进行测试时，敏感性为 85%（95%CI 77%至 91%），特异性为 99%（95%CI 98%至 100%）。
2022 年美国风湿病学会/欧洲风湿病联盟嗜酸性肉芽肿性多血管炎分类标准具有较强的性能特征，并经过验证可用于研究。
