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...
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2022-08-01
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| Online Access: | https://www.mdpi.com/2227-9059/10/8/1973 |
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| author | Paolo Cameli Valerio Alonzi Miriana d’Alessandro Laura Bergantini Elena Pordon Marco Guerrieri Rosa Metella Refini Piersante Sestini Elena Bargagli |
| author_facet | Paolo Cameli Valerio Alonzi Miriana d’Alessandro Laura Bergantini Elena Pordon Marco Guerrieri Rosa Metella Refini Piersante Sestini Elena Bargagli |
| author_sort | Paolo Cameli |
| collection | DOAJ |
| 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. |
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| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-09T10:00:18Z |
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| spelling | doaj.art-43befbd2cc1542c7a21a6fd4eb74f27a2023-12-01T23:28:17ZengMDPI AGBiomedicines2227-90592022-08-01108197310.3390/biomedicines10081973The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World StudyPaolo Cameli0Valerio Alonzi1Miriana d’Alessandro2Laura Bergantini3Elena Pordon4Marco Guerrieri5Rosa Metella Refini6Piersante Sestini7Elena Bargagli8Respiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyRespiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, 53100 Siena, ItalyBackground: 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.https://www.mdpi.com/2227-9059/10/8/1973nintedanibreal-world effectivenessidiopathic pulmonary fibrosisfamilial pulmonary fibrosismortality |
| spellingShingle | Paolo Cameli Valerio Alonzi Miriana d’Alessandro Laura Bergantini Elena Pordon Marco Guerrieri Rosa Metella Refini Piersante Sestini Elena Bargagli The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study Biomedicines nintedanib real-world effectiveness idiopathic pulmonary fibrosis familial pulmonary fibrosis mortality |
| 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 | nintedanib real-world effectiveness idiopathic pulmonary fibrosis familial pulmonary fibrosis mortality |
| url | https://www.mdpi.com/2227-9059/10/8/1973 |
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