Auckland District Health Board, Auckland, New Zealand.
Centre for Health and Clinical Research, University of the West of England and University Hospitals and Weston NHS Foundation Trust, Bristol, UK.
Ann Rheum Dis. 2022 Mar;81(3):321-326. doi: 10.1136/annrheumdis-2021-221796. Epub 2022 Feb 2.
To develop and validate classification criteria for microscopic polyangiitis (MPA).
Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate 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 MPA consisted of 149 cases of MPA and 408 comparators. The validation set consisted of an additional 142 cases of MPA and 414 comparators. From 91 candidate items, regression analysis identified 10 items for MPA, 6 of which were retained. The final criteria and their weights were as follows: perinuclear antineutrophil cytoplasmic antibody (ANCA) or anti-myeloperoxidase-ANCA positivity (+6), pauci-immune glomerulonephritis (+3), lung fibrosis or interstitial lung disease (+3), sino-nasal symptoms or signs (-3), cytoplasmic ANCA or anti-proteinase 3 ANCA positivity (-1) and eosinophil count ≥1×10/L (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having MPA with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 91% (95% CI 85% to 95%) and the specificity was 94% (95% CI 92% to 96%).
The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for MPA are now validated for use in clinical research.
制定和验证显微镜下多血管炎（MPA）的分类标准。
将血管炎或对照疾病患者纳入国际队列。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生成基于点的风险评分，随后在独立病例和对照集中验证性能特征。
MPA 的开发集包括 149 例 MPA 和 408 例对照。验证集包括另外 142 例 MPA 和 414 例对照。从 91 项候选项目中，回归分析确定了 10 项 MPA 项目，其中 6 项被保留。最终的标准及其权重如下：核周抗中性粒细胞胞质抗体（ANCA）或抗髓过氧化物酶-ANCA 阳性（+6）、少免疫性肾小球肾炎（+3）、肺纤维化或间质性肺疾病（+3）、鼻旁窦症状或体征（-3）、细胞质 ANCA 或抗蛋白酶 3 ANCA 阳性（-1）和嗜酸性粒细胞计数≥1×10/L（-4）。排除血管炎的模拟物后，具有小或中血管炎诊断的患者可以通过累积评分≥5 分来诊断为 MPA。当这些标准在验证数据集上进行测试时，敏感性为 91%（95%CI 85%至 95%），特异性为 94%（95%CI 92%至 96%）。
2022 年美国风湿病学会/欧洲风湿病联盟 MPA 分类标准现已验证可用于临床研究。
