Nieto Maria Asuncion, Vadillo Cristina, Pernaute Olga Sanchez, Romero-Bueno Fredeswinda, Rodriguez-Nieto María Jesús, Laporta Rosalia, Godoy Hilda, Loarce Jesús, Rigual Juan, Leon Leticia, Abasolo Lydia
Pneumology Department, Hospital Clínico San Carlos, Instituto de Investigacion Sanitaria San Carlos (IdISSC), Madrid, Spain.
Medicine Department, Universidad Complutense de Madrid, Madrid, Spain.
Respir Res. 2025 Jul 2;26(1):234. doi: 10.1186/s12931-025-03311-9.
To assess the incidence of functional respiratory impairment in interstitial lung disease (ILD) of autoimmune origin, starting progressive pulmonary fibrosis (PPF), and to evaluate the effectiveness of antifibrotics and other variables.
A longitudinal multicenter study was conducted in ILD of autoimmune origin (ILD with autoimmune rheumatic diseases, IPAF, and unclassifiable autoimmune ILD) from 2006 to 2023 and followed until September 2024 in Madrid. Patients were those enrolled in NEREA [pNEumology RhEumatology Autoinmune] registry who met PPF criteria.
functional respiratory impairment (≥ 5% absolute decline in predicted forced vital capacity (FVC%) within a year). Pulmonary function was assessed at baseline and every 6-12 months.
antifibrotics. Covariates: sociodemographics, clinical, other treatments. Survival techniques were used to estimate the incidence rate (IR) and [95%CI] of functional respiratory impairment, (per 100 patients-year). Cox multivariate regression models were run to examine the influence of antifibrotics and other covariates on, main outcome (results expressed as hazard ratio (HR) and [95%CI]).
Among 150 patients, 21 were on antifibrotics at baseline, increasing to 52 during follow-up. Functional respiratory impairment occurred in 118 patients with 292 events (IR 57.4 [51.2-64.4]). Regarding multivariate analysis: antifibrotics lowered functional respiratory impairment risk (nintedanib: HR 0.58 [0.39-0.85], pirfenidone: HR 0.68 [0.5-0.94]). Emphysema (HR 1.32 [1.04-1.68]), smoking (HR 1.40 [1.06-1.84]), and cardiovascular risk (HR 1.02 [1.02-1.63]) increased the risk.
The rate of worsening in PPF-ILD of autoimmune origin was considerable. Both antifibrotics reduced functional respiratory impairment risk in these patients, supporting prior clinical trials. Additional risk factors were identified.
Not applicable.
评估自身免疫性间质性肺病（ILD）、起始进行性肺纤维化（PPF）中功能性呼吸功能障碍的发生率，并评估抗纤维化药物及其他变量的有效性。
2006年至2023年在马德里开展了一项针对自身免疫性ILD（伴有自身免疫性风湿病的ILD、特发性间质性肺炎纤维化（IPAF）以及无法分类的自身免疫性ILD）的纵向多中心研究，并随访至2024年9月。患者为纳入NEREA[肺病与风湿病自身免疫性疾病]登记处且符合PPF标准的患者。
功能性呼吸功能障碍（一年内预测用力肺活量（FVC%）绝对下降≥5%）。在基线及每6 - 12个月评估一次肺功能。
抗纤维化药物。协变量：社会人口统计学、临床、其他治疗。采用生存技术估计功能性呼吸功能障碍的发病率（IR）及[95%置信区间]（每100患者 - 年）。运行Cox多变量回归模型以检验抗纤维化药物及其他协变量对主要结局的影响（结果以风险比（HR）及[95%置信区间]表示）。
150例患者中，21例在基线时使用抗纤维化药物，随访期间增至52例。118例患者发生功能性呼吸功能障碍，共292次事件（IR 57.4[51.2 - 64.4]）。关于多变量分析：抗纤维化药物降低了功能性呼吸功能障碍风险（尼达尼布：HR 0.58[0.39 - 0.85]，吡非尼酮：HR 0.68[0.5 - 0.94]）。肺气肿（HR 1.32[1.04 - 1.68]）、吸烟（HR 1.40[1.06 - 1.84]）和心血管风险（HR 1.02[1.02 - 1.63]）增加了风险。
自身免疫性PPF - ILD的恶化率相当高。两种抗纤维化药物均降低了这些患者的功能性呼吸功能障碍风险，支持先前的临床试验。还确定了其他风险因素。
不适用。
