Cargando…
The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study
Background: Nintedanib is an oral multitarget tyrosine kinase inhibitor approved for the treatment of patients with idiopathic pulmonary fibrosis (IPF). Recent evidence demonstrated that nintedanib reduced functional disease progression also in subjects with non-IPF progressive fibrosing interstitia...
Autores principales: | , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9405731/ https://www.ncbi.nlm.nih.gov/pubmed/36009520 http://dx.doi.org/10.3390/biomedicines10081973 |
_version_ | 1784773949183229952 |
---|---|
author | Cameli, Paolo Alonzi, Valerio d’Alessandro, Miriana Bergantini, Laura Pordon, Elena Guerrieri, Marco Refini, Rosa Metella Sestini, Piersante Bargagli, Elena |
author_facet | Cameli, Paolo Alonzi, Valerio d’Alessandro, Miriana Bergantini, Laura Pordon, Elena Guerrieri, Marco Refini, Rosa Metella Sestini, Piersante Bargagli, Elena |
author_sort | Cameli, Paolo |
collection | PubMed |
description | Background: Nintedanib is an oral multitarget tyrosine kinase inhibitor approved for the treatment of patients with idiopathic pulmonary fibrosis (IPF). Recent evidence demonstrated that nintedanib reduced functional disease progression also in subjects with non-IPF progressive fibrosing interstitial lung disease (PF-ILD). However, real-life data on the effectiveness of nintedanib in PF-ILD and familial pulmonary fibrosis (FPF) are lacking. Methods: this retrospective monocentric study enrolled 197 patients affected with IPF, PF-ILD and FPF treated with nintedanib at the Referral Centre of Siena from 2014 to 2021. Pulmonary functional tests and survival data were collected throughout the observation period for the evaluation of mortality and disease progression outcomes. Results: nintedanib treatment significantly reduced the FVC decline rate in IPF and PF-ILD subgroups, but not in FPF subjects. No significant differences were observed among the subgroups in terms of survival, which appeared to be influenced by gender and impaired lung function (FVC < 70% of predicted value). Concerning disease progression rate, a diagnosis of FPF is associated with more pronounced FVC decline despite nintedanib treatment. Conclusions: our research studies the effectiveness and safety of nintedanib in reducing functional disease progression of IPF and PF-ILD. FPF appeared to be less responsive to nintedanib, even though no differences were observed in terms of survival. |
format | Online Article Text |
id | pubmed-9405731 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-94057312022-08-26 The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study Cameli, Paolo Alonzi, Valerio d’Alessandro, Miriana Bergantini, Laura Pordon, Elena Guerrieri, Marco Refini, Rosa Metella Sestini, Piersante Bargagli, Elena Biomedicines Article Background: Nintedanib is an oral multitarget tyrosine kinase inhibitor approved for the treatment of patients with idiopathic pulmonary fibrosis (IPF). Recent evidence demonstrated that nintedanib reduced functional disease progression also in subjects with non-IPF progressive fibrosing interstitial lung disease (PF-ILD). However, real-life data on the effectiveness of nintedanib in PF-ILD and familial pulmonary fibrosis (FPF) are lacking. Methods: this retrospective monocentric study enrolled 197 patients affected with IPF, PF-ILD and FPF treated with nintedanib at the Referral Centre of Siena from 2014 to 2021. Pulmonary functional tests and survival data were collected throughout the observation period for the evaluation of mortality and disease progression outcomes. Results: nintedanib treatment significantly reduced the FVC decline rate in IPF and PF-ILD subgroups, but not in FPF subjects. No significant differences were observed among the subgroups in terms of survival, which appeared to be influenced by gender and impaired lung function (FVC < 70% of predicted value). Concerning disease progression rate, a diagnosis of FPF is associated with more pronounced FVC decline despite nintedanib treatment. Conclusions: our research studies the effectiveness and safety of nintedanib in reducing functional disease progression of IPF and PF-ILD. FPF appeared to be less responsive to nintedanib, even though no differences were observed in terms of survival. MDPI 2022-08-15 /pmc/articles/PMC9405731/ /pubmed/36009520 http://dx.doi.org/10.3390/biomedicines10081973 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Cameli, Paolo Alonzi, Valerio d’Alessandro, Miriana Bergantini, Laura Pordon, Elena Guerrieri, Marco Refini, Rosa Metella Sestini, Piersante Bargagli, Elena The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study |
title | The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study |
title_full | The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study |
title_fullStr | The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study |
title_full_unstemmed | The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study |
title_short | The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study |
title_sort | effectiveness of nintedanib in patients with idiopathic pulmonary fibrosis, familial pulmonary fibrosis and progressive fibrosing interstitial lung diseases: a real-world study |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9405731/ https://www.ncbi.nlm.nih.gov/pubmed/36009520 http://dx.doi.org/10.3390/biomedicines10081973 |
work_keys_str_mv | AT camelipaolo theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT alonzivalerio theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT dalessandromiriana theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT bergantinilaura theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT pordonelena theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT guerrierimarco theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT refinirosametella theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT sestinipiersante theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT bargaglielena theeffectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT camelipaolo effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT alonzivalerio effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT dalessandromiriana effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT bergantinilaura effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT pordonelena effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT guerrierimarco effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT refinirosametella effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT sestinipiersante effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy AT bargaglielena effectivenessofnintedanibinpatientswithidiopathicpulmonaryfibrosisfamilialpulmonaryfibrosisandprogressivefibrosinginterstitiallungdiseasesarealworldstudy |