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Multicenter Study
. 2025 Jul 2;26(1):234.
doi: 10.1186/s12931-025-03311-9.

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

Maria Asuncion Nieto  1   2 , Cristina Vadillo  3 , Olga Sanchez Pernaute  4 , Fredeswinda Romero-Bueno  4 , María Jesús Rodriguez-Nieto  5   6 , Rosalia Laporta  7 , Hilda Godoy  8 , Jesús Loarce  9 , Juan Rigual  10 , Leticia Leon  11   12 , Lydia Abasolo  13
Affiliations
Multicenter Study

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

Maria Asuncion Nieto et al. Respir Res. .

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.

Keywords: Interstitial lung disease; Observational study; Risk factors, prognosis.

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Conflict of interest statement

Declarations. Ethical approval: Data in this project were obtained during clinical practice, and patients provided signed informed consent. The study was conducted in accordance with the Declaration of Helsinki and Good Clinical Practices, and approved by the Hospital Clinico San Carlos Ethics Committee ( 24/072-E). Consent for publication: Not applicable. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Patients without any DMARD prescription during the follow up and stratified by GC and/or AF prescription
Fig. 2
Fig. 2
Kaplan-Meier survival estimate curve. Functional respiratory impairment over time
See this image and copyright information in PMC

References

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