Sergio Harari1, Antonella Caminati1, Venerino Poletti2, Marco Confalonieri3, Stefano Gasparini4, Donato Lacedonia5, Fabrizio Luppi6, Alberto Pesci7, Alfredo Sebastiani8, Paolo Spagnolo9, Carlo Vancheri10, Elisabetta Balestro9, Martina Bonifazi4, Stefania Cerri6, Federica De Giacomi7, Rossana Della Porta3, Maria Pia Foschino Barbaro5, Annalisa Fui11, Patrizio Pasquinelli8, Roberta Rosso10, Sara Tomassetti2, Claudia Specchia12,13, Paola Rottoli11. 1. U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, IRCCS MultiMedica, Milan, Italy. 2. U.O. di Pneumologia, Dipartimento dell'Apparato Respiratorio e del Torace, Ospedale G.P. Morgagni-L. Pierantoni, Forlì, Italy. 3. Department of Pulmonology, University Hospital of Cattinara, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" di Trieste, Trieste, Italy. 4. Pulmonology Unit, Department of Biomedical Sciences and Public Health, Università Politecnica delle Marche, Azienda Ospedaliera-Universitaria "Ospedali Riuniti", Ancona, Italy. 5. Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy. 6. Center for Rare Lung Diseases, University Hospital Policlinico di Modena, Modena, Italy. 7. Respiratory Unit, Department of Health Science, University of Milano-Bicocca, AO San Gerardo, Monza, Italy. 8. Department of Respiratory Diseases, S. Camillo-Forlanini Hospital, Rome, Italy. 9. Section of Respiratory Disease, Department of Cardiac, Thoracic, and Vascular Sciences, University of Padua, Padua, Italy. 10. Regional Referral Centre for Rare Lung Disease, University of Catania, A.O.U. Policlinico-Vittorio Emanuele, Catania, Italy. 11. Respiratory Diseases and Lung Transplant Unit, Department of Internal and Specialist Medicine, AOUS, Siena, Italy. 12. Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy. 13. IRCCS MultiMedica, Milan, Italy.
Abstract
BACKGROUND: Two therapeutic options are currently available for patients with mild-to-moderate idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. To date, there is still insufficient data on the efficacy of these 2 agents in patients with more severe disease. OBJECTIVES: This national, multicenter, retrospective real-life study was intended to determine the impact of nintedanib on the treatment of patients with severe IPF. METHODS: All patients included had severe IPF and had to have at least 6 months of follow-up before and at least 6 months of follow-up after starting nintedanib. The aim of the study was to compare the decline in lung function before and after treatment. Patient survival after 6 months of therapy with nintedanib was assessed. RESULTS: Forty-one patients with a forced vital capacity (FVC) ≤50% and/or a diffusing capacity of the lung for carbon monoxide (DLCO) ≤35% predicted at the start of nintedanib treatment were enrolled. At the 6-month follow-up, the decline of DLCO (both absolute and % predicted) was significantly reduced compared to the pretreatment period (absolute DLCO at the -6-month, T0, and +6-month time points (5.48, 4.50, and 5.03 mmol/min/kPa, respectively, p = 0.03; DLCO% predicted was 32.73, 26.54, and 29.23%, respectively, p = 0.04). No significant beneficial effect was observed in the other functional parameters analyzed. The 1-year survival in this population was 79%, calculated from month 6 of therapy with nintedanib. CONCLUSIONS: This nationwide multicenter experience in patients with severe IPF shows that nintedanib slows down the rate of decline of absolute and % predicted DLCO but does not have significant impact on FVC or other lung parameters.
BACKGROUND: Two therapeutic options are currently available for patients with mild-to-moderate idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. To date, there is still insufficient data on the efficacy of these 2 agents in patients with more severe disease. OBJECTIVES: This national, multicenter, retrospective real-life study was intended to determine the impact of nintedanib on the treatment of patients with severe IPF. METHODS: All patients included had severe IPF and had to have at least 6 months of follow-up before and at least 6 months of follow-up after starting nintedanib. The aim of the study was to compare the decline in lung function before and after treatment. Patient survival after 6 months of therapy with nintedanib was assessed. RESULTS: Forty-one patients with a forced vital capacity (FVC) ≤50% and/or a diffusing capacity of the lung for carbon monoxide (DLCO) ≤35% predicted at the start of nintedanib treatment were enrolled. At the 6-month follow-up, the decline of DLCO (both absolute and % predicted) was significantly reduced compared to the pretreatment period (absolute DLCO at the -6-month, T0, and +6-month time points (5.48, 4.50, and 5.03 mmol/min/kPa, respectively, p = 0.03; DLCO% predicted was 32.73, 26.54, and 29.23%, respectively, p = 0.04). No significant beneficial effect was observed in the other functional parameters analyzed. The 1-year survival in this population was 79%, calculated from month 6 of therapy with nintedanib. CONCLUSIONS: This nationwide multicenter experience in patients with severe IPF shows that nintedanib slows down the rate of decline of absolute and % predicted DLCO but does not have significant impact on FVC or other lung parameters.
Authors: Paola Faverio; Federica De Giacomi; Giulia Bonaiti; Anna Stainer; Luca Sardella; Giulia Pellegrino; Giuseppe Francesco Sferrazza Papa; Francesco Bini; Bruno Dino Bodini; Mauro Carone; Sara Annoni; Grazia Messinesi; Alberto Pesci Journal: Int J Med Sci Date: 2019-06-10 Impact factor: 3.738
Authors: Claudia Valenzuela; Sebastiano Emanuele Torrisi; Nicolas Kahn; Manuel Quaresma; Susanne Stowasser; Michael Kreuter Journal: Respir Res Date: 2020-01-06