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Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry

BACKGROUND: The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis...

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Autores principales: Nieto, Maria Asuncion, Sanchez-Pernaute, Olga, Vadillo, Cristina, Rodriguez-Nieto, Maria Jesus, Romero-Bueno, Fredeswinda, López-Muñiz, Belen, Cebrian, Laura, Rio-Ramirez, Maria Teresa, Laporta, Rosalia, Bonilla, Gema, Cobo, Tatiana, Leon, Leticia, Abasolo, Lydia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9847038/
https://www.ncbi.nlm.nih.gov/pubmed/36653833
http://dx.doi.org/10.1186/s12931-023-02317-5
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author Nieto, Maria Asuncion
Sanchez-Pernaute, Olga
Vadillo, Cristina
Rodriguez-Nieto, Maria Jesus
Romero-Bueno, Fredeswinda
López-Muñiz, Belen
Cebrian, Laura
Rio-Ramirez, Maria Teresa
Laporta, Rosalia
Bonilla, Gema
Cobo, Tatiana
Leon, Leticia
Abasolo, Lydia
author_facet Nieto, Maria Asuncion
Sanchez-Pernaute, Olga
Vadillo, Cristina
Rodriguez-Nieto, Maria Jesus
Romero-Bueno, Fredeswinda
López-Muñiz, Belen
Cebrian, Laura
Rio-Ramirez, Maria Teresa
Laporta, Rosalia
Bonilla, Gema
Cobo, Tatiana
Leon, Leticia
Abasolo, Lydia
author_sort Nieto, Maria Asuncion
collection PubMed
description BACKGROUND: The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients. METHODS: A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis. Main outcome: poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests. Statistics: Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval [CI]. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and [CI]). RESULTS: 79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 [19.9–28.8], and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 [0.34–0.83]), age (HR: 1.04 [1.01–1.07]), and Ro-antibodies (HR: 0.36 [0.19–0.65]) influenced the prognosis. CONCLUSIONS: IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF.
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spelling pubmed-98470382023-01-19 Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry Nieto, Maria Asuncion Sanchez-Pernaute, Olga Vadillo, Cristina Rodriguez-Nieto, Maria Jesus Romero-Bueno, Fredeswinda López-Muñiz, Belen Cebrian, Laura Rio-Ramirez, Maria Teresa Laporta, Rosalia Bonilla, Gema Cobo, Tatiana Leon, Leticia Abasolo, Lydia Respir Res Comment BACKGROUND: The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients. METHODS: A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis. Main outcome: poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests. Statistics: Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval [CI]. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and [CI]). RESULTS: 79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 [19.9–28.8], and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 [0.34–0.83]), age (HR: 1.04 [1.01–1.07]), and Ro-antibodies (HR: 0.36 [0.19–0.65]) influenced the prognosis. CONCLUSIONS: IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF. BioMed Central 2023-01-18 2023 /pmc/articles/PMC9847038/ /pubmed/36653833 http://dx.doi.org/10.1186/s12931-023-02317-5 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Comment
Nieto, Maria Asuncion
Sanchez-Pernaute, Olga
Vadillo, Cristina
Rodriguez-Nieto, Maria Jesus
Romero-Bueno, Fredeswinda
López-Muñiz, Belen
Cebrian, Laura
Rio-Ramirez, Maria Teresa
Laporta, Rosalia
Bonilla, Gema
Cobo, Tatiana
Leon, Leticia
Abasolo, Lydia
Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry
title Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry
title_full Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry
title_fullStr Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry
title_full_unstemmed Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry
title_short Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry
title_sort functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (ipaf): multicenter study from nerea registry
topic Comment
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9847038/
https://www.ncbi.nlm.nih.gov/pubmed/36653833
http://dx.doi.org/10.1186/s12931-023-02317-5
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