Effectiveness Of Pirfenidone For Idiopathic Pulmonary Fibrosis Associated With Pleuroparenchymal Fibroelastosis-Like Lesions And Nonspecific Interstitial Pneumonia

Background Pirfenidone treatment can slow decline in forced vital capacity (FVC) in idiopathic pulmonary fibrosis (IPF). However, its effects for usual interstitial pneumonia (UIP) with pleuroparenchymal fibroelastosis-like lesions (UIP+PPFELL) and UIP with nonspecific interstitial pneumonia (UIP+NSIP) are unclear.Objectives The aim of study is to assess pirfenidone effectiveness for UIP+PPFELL and UIP+NSIP.Methods We retrospectively analysed data from 58 IPF patients treated with pirfenidone more than 6 months. The outcomes of interest were 6-month follow-up pulmonary function test results, progression-free survival (PFS) and overall survival (OS). Treatment was considered effective if FVC decline was <5% during the 6-month period. We compared clinical characteristics, effectiveness, PFS and OS between patients with typical IPF (n = 32), UIP+PPFELL (n = 12) and UIP+NSIP (n = 14).Results Data from 58 IPF patients were analysed. At the 6-month follow-up examination, treatment was deemed effective for 9 of 14 (64%) UIP+NSIP patients, 6 of 12 (50%) UIP+PPFELL patients and 14 of 32 (44%) patients with typical IPF. The 6-month decline in FVC before treatment was greater than that after starting treatment in the UIP+NSIP (-210 vs. -57 mL; P = 0.09), UIP+PPFELL (-370 vs. -89 mL; P = 0.001) and typical IPF (-172 vs. -85 mL; P = 0.37). PFS did not significantly differ between the three groups. OS was significantly shorter for UIP+PPFELL (312 days) than for UIP+NSIP (545 days) and typical IPF (661 days).Conclusions Pirfenidone decreased the decline in FVC in patients with UIP+PPFELL and UIP+NSIP, as well as in those with typical IPF. However, outcomes were worse for patients with UIP+PPFELL.