Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, No. 1 Shuai Fu Yuan Street, Dongcheng-Qu, Beijing, 100730, China.
Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, No. 1 Shuai Fu Yuan Street, Dongcheng-Qu, Beijing, 100730, China.
Respir Med. 2024 May;226:107613. doi: 10.1016/j.rmed.2024.107613. Epub 2024 Mar 26.
Patients with microscopic polyangiitis (MPA) and positive myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) may present with various abnormalities in chest computed tomography (CT). This study aimed to identify subphenotypes using latent class analysis (LCA) and to explore the relationship between the subphenotypes and clinical patterns, as well as compare the clinical characteristics of these subphenotypes in patients with MPO-ANCA-positive MPA (MPO-MPA).
The study identified subphenotypes using LCA based on chest CT findings in 178 patients with MPO-MPA and pulmonary involvement from June 2014 to August 2022.
LCA identified 27 participants (15.2%) in class 1, 43 (24.1%) in class 2, 35 (19.7%) in class 3, and 73 (41.0%) in class 4. Class 1 was characterized by prominent inflammatory exudation, class 2 by fibrosis and architectural distortion, class 3 by predominantly bronchiectasis, and class 4 by lesions mixed with inflammation and fibrosis. Class 1 had the highest level of extrapulmonary disease activity, with 77.8% of patients experiencing diffuse alveolar hemorrhage. Class 2 had the lowest level of extrapulmonary disease activity, with 41.9% of patients showing usual interstitial pneumonia. Class 3 patients were more likely to have complications involving the ear, nose, and throat, as well as pulmonary infections before treatment, and they exhibited the best outcomes. The characteristics and outcomes of class 4 were intermediate among the four classes.
These findings suggest that bronchiectasis may represent a unique pattern of pulmonary involvement in MPO-MPA, highlighting the importance of screening for bronchiectasis in MPO-MPA and identifying optimal management strategies.
抗髓过氧化物酶抗体阳性的显微镜下多血管炎(MPA)患者在胸部计算机断层扫描(CT)中可能会出现各种异常。本研究旨在使用潜在类别分析(LCA)确定亚表型,并探讨亚表型与临床模式之间的关系,以及比较这些抗髓过氧化物酶抗体阳性 MPA(MPO-MPA)患者亚表型的临床特征。
本研究基于 2014 年 6 月至 2022 年 8 月 178 例 MPO-MPA 合并肺部受累患者的胸部 CT 结果,采用 LCA 确定亚表型。
LCA 确定了 1 类 27 例(15.2%)、2 类 43 例(24.1%)、3 类 35 例(19.7%)和 4 类 73 例(41.0%)。1 类表现为明显的炎症渗出,2 类为纤维化和结构扭曲,3 类主要为支气管扩张,4 类为炎症和纤维化混合病变。1 类患者的肺外疾病活动度最高,77.8%的患者发生弥漫性肺泡出血。2 类患者的肺外疾病活动度最低,41.9%的患者表现为寻常性间质性肺炎。3 类患者在治疗前更易出现耳、鼻、喉并发症和肺部感染,且预后最佳。4 类患者的特征和预后处于 4 类中。
这些发现表明支气管扩张可能代表 MPO-MPA 肺部受累的一种独特模式,强调了在 MPO-MPA 中筛查支气管扩张的重要性,并确定了最佳的管理策略。