A Real-Life Multicenter National Study on Nintedanib in Severe Idiopathic Pulmonary Fibrosis

• Published in: Respiration Vol. 95; no. 6; pp. 433 - 440
• Main Authors: Harari, Sergio, Caminati, Antonella, Poletti, Venerino, Confalonieri, Marco, Gasparini, Stefano, Lacedonia, Donato, Luppi, Fabrizio, Pesci, Alberto, Sebastiani, Alfredo, Spagnolo, Paolo, Vancheri, Carlo, Balestro, Elisabetta, Bonifazi, Martina, Cerri, Stefania, De Giacomi, Federica, Della Porta, Rossana, Foschino Barbaro, Maria Pia, Fui, Annalisa, Pasquinelli, Patrizio, Rosso, Roberta, Tomassetti, Sara, Specchia, Claudia, Rottoli, Paola
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 01.01.2018
• Subjects: Aged; Analysis; Carbon monoxide; Care and treatment; Clinical Investigations; Enzyme Inhibitors - therapeutic use; Female; Fibrosis; Health aspects; Humans; Idiopathic Pulmonary Fibrosis - drug therapy; Indoles - therapeutic use; Lung volume measurement; Male; Middle Aged; Nintedanib; Pirfenidone; Pulmonary fibrosis; Respiratory Function Tests; Respiratory tract diseases; Retrospective Studies; Severe idiopathic pulmonary fibrosis; Nintedanib; Therapy; Pirfenidone
• ISSN: 0025-7931; 1423-0356
• DOI: 10.1159/000487711

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 (D LCO ) ≤35% predicted at the start of nintedanib treatment were enrolled. At the 6-month follow-up, the decline of D LCO (both absolute and % predicted) was significantly reduced compared to the pretreatment period (absolute D LCO at the –6-month, T 0 , and +6-month time points (5.48, 4.50, and 5.03 mmol/min/kPa, respectively, p = 0.03; D LCO % 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 D LCO but does not have significant impact on FVC or other lung parameters.