The effect of nintedanib on lung functions and survival in idiopathic pulmonary fibrosis: real-life analysis of the Czech EMPIRE registry

Abstract

Introduction The antifibrotic drug nintedanib is used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the effect of nintedanib on antifibrotic treatment outcome in real-world cohorts of Czech EMPIRE registry. Patients/methods Data of 611 Czech IPF subjects, 430 (70%) treated with nintedanib (NIN group), 181 (30%) with no-antifibrotic treatment (NAF group) were analysed. The influence of nintedanib on overall survival (OS), pulmonary function parameters as forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO), as well as GAP score (gender, age, physiology) and and CPI (composite physiological index) were investigated. Results During 2 year follow-up we observed that nintedanib treated patients had longer OS, compared to those treated with no-antifibrotic drugs (p < 0.00001). Nintedanib reduces risk of mortality over no-antifibrotic treatment by 55% (p < 0.001). We have observed no significant difference in the rate of FVC and DLCO decline between the NIN and NAF group. Changes within 24 months from baseline in CPI were not significant between the groups (NAF and NIN). Conclusion Our real-practice study showed the benefit of nintedanib treatment on survival. There were no significant differences between NIN and NAF groups in changes from baseline in FVC %, DLCO % predicted and CPI.

Pirfenidone and Nintedanib in Pulmonary Fibrosis: Lights and Shadows

“…by a progressive distortion of airway architecture, leading to reduced lung volume and gradual loss of lung function, which can eventually result in respiratory failure [2][3][4][5][6][7][8][9].…”

“…Interstitial lung diseases (ILDs) comprise nearly a fifth of all lung diseases. ILDs are a heterogeneous group of disorders classified into distinct entities, each with specific clinical, radiological, and pathological characteristics [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 ]. Most ILDs are characterized by a progressive distortion of airway architecture, leading to reduced lung volume and gradual loss of lung function, which can eventually result in respiratory failure [ 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ].…”

“…Nintedanib acts as a triple tyrosine kinase inhibitor (TKI) targeting the receptor kinases of platelet-derived growth factor (PDGF), fibroblast growth factor (FGF), and vascular endothelial growth factor (VEGF). These receptors play a crucial role in IPF pathogenesis by inhibiting the proliferation and activation of human fibroblast, reducing the release of profibrotic and inflammatory mediators, decreasing extracellular matrix deposition, and inhibiting vessel proliferation [ 8 , 35 ]. Its antifibrotic and anti-inflammatory activities have been demonstrated in multiple in vitro assays and animal models of lung fibrosis [ 37 , 38 , 39 , 40 , 41 , 42 , 43 ].…”