Sun Xin, Zhu Wenyan, Zhou Chunsheng, Xue Peijun, Li Zongru, Zhang Weihong, Zhao Jiuliang, Zhang Ting, Peng Min, Shi Juhong, Wang Chen
Department of Respiratory and Critical Care Medicine, Dongcheng District, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, No. 1 Shuai Fu Yuan, Beijing, 100730, China.
Department of Geriatrics, Peking University First Hospital, Beijing, China.
BMC Pulm Med. 2025 May 29;25(1):271. doi: 10.1186/s12890-025-03736-4.
Patients with anti-neutrophil cytoplasmic antibody (ANCA)-positive interstitial lung disease (ILD) but without evidence of systemic vasculitis have been reported in studies and are classified as isolated ANCA-positive idiopathic interstitial pneumonia (IIP). However, the clinical significance of ANCA, particularly myeloperoxidase (MPO) -ANCA in IIP remains poorly understood. This study aims to investigate the differences between ANCA-positive and ANCA-negative IIP patients and further explore the impact of MPO-ANCA on clinical manifestations and prognostic outcomes.
We reviewed 408 ILD patients with available ANCA results from January 2012 to September 2021. 61 patients diagnosed with microscopic polyangiitis-associated ILD were not included in the analysis. A comparative analysis was performed between 61 isolated ANCA-positive IIP patients (ANCA-IIP group) and 286 ANCA-negative IIP patients (IIP group). We further conducted subgroup analyses based on the status of MPO-ANCA.
Baseline clinical characteristics, pulmonary function tests, radiological features and all-cause mortality were similar between ANCA-IIP and IIP groups. When comparing the MPO-ANCA-IIP group with the IIP group and the non-MPO-ANCA-IIP group separately, a higher proportion of fibrotic features was observed on imaging (P = 0.004 vs IIP group; P = 0.031 vs non-MPO-ANCA-IIP group). After one year of treatment, the MPO-ANCA-IIP group showed a significantly greater decline in pulmonary function parameters compared to both the IIP group and the non-MPO-ANCA-IIP group. The frequency of pulmonary function decline was significantly higher in the MPO-ANCA-IIP group compared to the non-MPO-ANCA-IIP group (P = 0.026). Additionally, MPO-ANCA was not found to be statistically associated with mortality among patients with IIP.
ANCA-IIP patients had similar clinical characteristics and prognoses with IIP patients. MPO-ANCA-IIP patients had more prominent fibrosis on imaging and a greater decline in pulmonary function following treatment. Special attention should be paid to MPO-ANCA positivity during the diagnosis and treatment of IIP patients.
ClinicalTrials.gov: NCT04413149, May 2020.
研究报道了抗中性粒细胞胞浆抗体（ANCA）阳性的间质性肺疾病（ILD）患者，但无系统性血管炎证据，这些患者被归类为孤立性ANCA阳性特发性间质性肺炎（IIP）。然而，ANCA尤其是髓过氧化物酶（MPO）-ANCA在IIP中的临床意义仍知之甚少。本研究旨在调查ANCA阳性和ANCA阴性IIP患者之间的差异，并进一步探讨MPO-ANCA对临床表现和预后结果的影响。
我们回顾了2012年1月至2021年9月有ANCA检测结果的408例ILD患者。61例诊断为显微镜下多血管炎相关ILD的患者未纳入分析。对61例孤立性ANCA阳性IIP患者（ANCA-IIP组）和286例ANCA阴性IIP患者（IIP组）进行了对比分析。我们还根据MPO-ANCA状态进行了亚组分析。
ANCA-IIP组和IIP组在基线临床特征、肺功能测试、影像学特征和全因死亡率方面相似。将MPO-ANCA-IIP组分别与IIP组和非MPO-ANCA-IIP组比较时，影像学上观察到纤维化特征的比例更高（与IIP组相比，P = 0.004；与非MPO-ANCA-IIP组相比，P = 0.031）。治疗一年后，MPO-ANCA-IIP组的肺功能参数下降幅度明显大于IIP组和非MPO-ANCA-IIP组。MPO-ANCA-IIP组肺功能下降的频率明显高于非MPO-ANCA-IIP组（P = 0.026）。此外，未发现MPO-ANCA与IIP患者的死亡率有统计学关联。
ANCA-IIP患者与IIP患者具有相似的临床特征和预后。MPO-ANCA-IIP患者在影像学上纤维化更明显，治疗后肺功能下降更大。在IIP患者的诊断和治疗过程中应特别关注MPO-ANCA阳性情况。
ClinicalTrials.gov：NCT04413149，2020年5月。
