Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry
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 wa...
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BMC
2025-07-01
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| Series: | Respiratory Research |
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| Online Access: | https://doi.org/10.1186/s12931-025-03311-9 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Maria Asuncion Nieto |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-18e8d2fe9cde4664a4117691060d72a6 |
| institution | DOAJ |
| issn | 1465-993X |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMC |
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| series | Respiratory Research |
| spelling | doaj-art-18e8d2fe9cde4664a4117691060d72a62025-08-20T03:03:29ZengBMCRespiratory Research1465-993X2025-07-0126111510.1186/s12931-025-03311-9Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registryMaria Asuncion Nieto0Cristina Vadillo1Olga Sanchez Pernaute2Fredeswinda Romero-Bueno3María Jesús Rodriguez-Nieto4Rosalia Laporta5Hilda Godoy6Jesús Loarce7Juan Rigual8Leticia Leon9Lydia Abasolo10Pneumology Department, Hospital Clínico San Carlos, Instituto de Investigacion Sanitaria San Carlos (IdISSC)Rheumatology Department, Hospital Clínico San CarlosRheumatology Department, Hospital Fundación Jiménez DíazRheumatology Department, Hospital Fundación Jiménez DíazPneumology Department, Hospital Fundación Jiménez DíazPneumology Department, Hospital Puerta de HierroRheumatology Department, Hospital Puerta de HierroRheumatology Department, Hospital Ramon y CajalPneumology Department, Hospital Ramon y CajalFaculty of Health Sciences -HM Hospitals, University Camilo José CelaMusculoskeletal Pathology Group, Rheumatology Department, IdISSC, Hospital Clínico San CarlosAbstract 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.https://doi.org/10.1186/s12931-025-03311-9Interstitial lung diseaseObservational studyRisk factors, prognosis |
| spellingShingle | 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 Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry Respiratory Research Interstitial lung disease Observational study Risk factors, prognosis |
| title | Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry |
| title_full | Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry |
| title_fullStr | Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry |
| title_full_unstemmed | Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry |
| title_short | Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: multicenter study from NEREA registry |
| title_sort | role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases multicenter study from nerea registry |
| topic | Interstitial lung disease Observational study Risk factors, prognosis |
| url | https://doi.org/10.1186/s12931-025-03311-9 |
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