The impact of antifibrotic therapy in the management of idiopathic pulmonary fibrosis and progressive fibrosing interstitial lung diseases: a real-world comparative study of efficacy between pirfenidone and nintedanib

Background: Idiopathic pulmonary fibrosis (IPF) is the most common and lethal among diffuse fibrosing interstitial lung diseases (ILD). Beyond lung transplantion, the therapeutic approach relies on antifibrotic treatment: pirfenidone and nintedanib are the only pharmaceutical drugs approved for IPF, since they have demonstrated to significantly reduce disease progression rate. Still, no solid data has been published to compare these two drugs as well as few studies have investigated their potential efficacy on familial pulmonary fibrosis (FPF) and progressive fibrosing ILD (PF-ILD) in a real-life setting. Methods: we collected clinical, functional and radiological data from all patients affected with IPF and PF-ILDs that have been treated with pirfenidone and nintedanib at Referral Centre of Siena from 2011 to 2020. The aim of the research was to compare effectiveness of the two drugs in terms of mortality and disease progression in our population. Results: no significant differences in mortality and progression-free survival were observed between pirfenidone and nintedanib subgroup. Both drugs significantly reduce FVC and DLCO decline rate in respect with pretreatment period. Similar data was observed in the PF-ILD subgroup, while FPF patients showed no significant benefit from antifibrotic treatment in terms of disease progression. Pirfenidone was more effective than nintedanib in preserving FVC in FPF subgroup. Conclusions: our research study, conducted in a large cohort through a almost decennial time of observation, confirmed the reliable and substantially similar efficacy of pirfenidone and nintedanib in improving life expectancy and progression-free survival of IPF patients. FPF appeared to be less responsive to antifibrotics, but pirfenidone showed a better performance than nintedanib on this field. PF-ILD patients showed a analogue clinical course of IPF subjects in our study: the effectiveness of pirfenidone and nintedanib was reliable and similar, supporting their future use in clinical practice.