Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry.

IF 5.8 2区医学 Q1 Medicine

Respiratory Research Pub Date : 2025-07-02 DOI:10.1186/s12931-025-03311-9

Maria Asuncion Nieto, Cristina Vadillo, Olga Sanchez Pernaute, Fredeswinda Romero-Bueno, María Jesús Rodriguez-Nieto, Rosalia Laporta, Hilda Godoy, Jesús Loarce, Juan Rigual, Leticia Leon, Lydia Abasolo

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引用次数: 0

Abstract

Background: 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.

Methods: 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.

Main outcome: 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.

Independent variable: 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]).

Results: 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.

Conclusions: 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.

Clinical trial number: Not applicable.

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抗纤维化药物在自身免疫性疾病相关的进行性肺纤维化中的作用：来自NEREA注册的多中心研究

背景：评估自身免疫性间质性肺疾病（ILD）、开始进行性肺纤维化（PPF）的功能性呼吸损害发生率，并评估抗纤维化药物和其他变量的有效性。方法：从2006年到2023年，在马德里进行了一项纵向多中心研究，研究对象是自身免疫性ILD（自身免疫性风湿性疾病、IPAF和无法分类的自身免疫性ILD），并随访至2024年9月。患者是在NEREA[肺炎风湿病自身免疫]登记的符合PPF标准的患者。主要转归：功能性呼吸障碍（预测用力肺活量（FVC%）一年内绝对下降≥5%）。在基线和每6-12个月评估一次肺功能。自变量：抗纤维化药物。协变量：社会人口统计学，临床，其他治疗。生存技术用于估计功能性呼吸障碍的发病率（IR）和[95%CI]（每100例患者-年）。采用Cox多元回归模型检验抗纤维化药物及其他协变量对主要结局的影响（结果以危险比（HR）和[95%CI]表示）。结果：在150例患者中，21例在基线时使用抗纤维化药物，随访期间增加到52例。118例患者发生功能性呼吸损害，292例事件（IR 57.4[51.2-64.4]）。多因素分析：抗纤维化药物降低功能性呼吸损伤风险（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。结论：自身免疫性PPF-ILD的恶化率相当高。这两种抗纤维化药物降低了这些患者的功能性呼吸损伤风险，支持先前的临床试验。确定了其他危险因素。临床试验号：不适用。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Respiratory Research RESPIRATORY SYSTEM-

CiteScore

9.70

自引率

1.70%

发文量

314

审稿时长

4-8 weeks

期刊介绍： Respiratory Research publishes high-quality clinical and basic research, review and commentary articles on all aspects of respiratory medicine and related diseases. As the leading fully open access journal in the field, Respiratory Research provides an essential resource for pulmonologists, allergists, immunologists and other physicians, researchers, healthcare workers and medical students with worldwide dissemination of articles resulting in high visibility and generating international discussion. Topics of specific interest include asthma, chronic obstructive pulmonary disease, cystic fibrosis, genetics, infectious diseases, interstitial lung diseases, lung development, lung tumors, occupational and environmental factors, pulmonary circulation, pulmonary pharmacology and therapeutics, respiratory immunology, respiratory physiology, and sleep-related respiratory problems.