Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Semin Arthritis Rheum. 2024 Oct;68:152484. doi: 10.1016/j.semarthrit.2024.152484. Epub 2024 May 30.
This study explored the development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and its risk factors in patients with idiopathic interstitial pneumonia (IIP) and positive ANCA results.
Data of patients diagnosed with IIP with positive ANCA results at a single tertiary center in South Korea were retrospectively reviewed from January 2013 to August 2023. Cox regression analysis was performed to identify variables associated with AAV occurrence following IIP diagnosis. Kaplan-Meier curves were employed to investigate the relationship between autoantibodies and the occurrence of AAV.
In a cohort of 154 IIP-diagnosed patients with positive ANCA results but without AAV, 10.4 % of them eventually developed AAV. The AAV and non-AAV groups did not significantly differ by sex, age, smoking status, urinalysis, or chest computed tomography findings. All the patients who subsequently developed AAV were anti-myeloperoxidase (MPO) positive, while 48.8 % of the non-AAV patients were anti-MPO positive (P < 0.001). Rheumatoid factor (RF) positivity differed significantly (62.5 % vs. 29.2 %, P = 0.007) between the AAV and non-AAV groups. Multivariate Cox regression and Kaplan-Meier analyses revealed RF (HR 4.02; P = 0.004) and anti-MPO (HR 38.10; P < 0.001) positivity as risk factors associated with AAV occurrence.
Approximately 10 % of ANCA-positive IIP patients developed AAV after an IIP diagnosis. Anti-MPO or co-occurring positive RF poses a significant risk for subsequent AAV occurrence. This emphasizes the importance of careful monitoring in patients with high-risk antibody profiles, even if the complete features of AAV are not present at IIP diagnosis.
本研究旨在探讨特发性间质性肺炎（IIP）伴抗中性粒细胞胞质抗体（ANCA）阳性患者中抗中性粒细胞胞质抗体相关性血管炎（AAV）的发病情况及其相关危险因素。
回顾性分析 2013 年 1 月至 2023 年 8 月在韩国某三级中心诊断为 IIP 且 ANCA 阳性的患者数据。采用 Cox 回归分析确定与 IIP 诊断后 AAV 发生相关的变量。采用 Kaplan-Meier 曲线分析自身抗体与 AAV 发生的关系。
在 154 例诊断为 IIP 且 ANCA 阳性但无 AAV 的患者队列中，有 10.4%的患者最终发展为 AAV。AAV 组和非 AAV 组在性别、年龄、吸烟状况、尿液分析或胸部计算机断层扫描结果方面无显著差异。所有随后发生 AAV 的患者均为抗髓过氧化物酶（MPO）阳性，而非 AAV 患者中 48.8%为抗 MPO 阳性（P<0.001）。RF 阳性在 AAV 组和非 AAV 组之间差异有统计学意义（62.5% vs. 29.2%，P=0.007）。多变量 Cox 回归和 Kaplan-Meier 分析显示 RF（HR 4.02；P=0.004）和抗 MPO（HR 38.10；P<0.001）阳性是与 AAV 发生相关的危险因素。
约 10%的 ANCA 阳性 IIP 患者在 IIP 诊断后发生 AAV。抗 MPO 或同时存在阳性 RF 是随后发生 AAV 的显著危险因素。这强调了在具有高危抗体谱的患者中进行仔细监测的重要性，即使在 IIP 诊断时不具备完整的 AAV 特征。
