Real-life experience with pirfenidone in idiopathic pulmonary fibrosis (IPF) in a single centre in Italy. A retrospective study

Aim: To assess the safety of pirfenidone and its effectiveness in slowing down IPF progression in a real-life setting in Italy. Patients and Methods: Between Apr 2008 and Dec 2016, 68 IPF patients were treated. Side effects and reason for therapy discontinuation were recorded. Functional (FVC and DLCO) decline in the first 11±2 months (mean±SD) of treatment was assessed in the 22 patients for whom the data were available. A historical cohort of 26 patients enrolled prior to pirfenidone approval was used for comparison. Results: Treatment was discontinued in 3 patients (4%) due to malaise (1) and significant weight loss (2), and the dose reduced in 4 patients (6%) due to skin reactions (2) and epigastralgia (2). Baseline functional characteristics were not different between the two groups (unpaired t-test). DLCO % predicted decreased significantly during the follow-up period in untreated patients; in contrast, no significant decline was observed in treated patients (paired t-test; fig. 1A). No significant decline in FVC was observed for either group. Time to functional decline, defined as a 10- or 15-percentage point reduction for FVC (not shown) and DLCO (fig. 1B) respectively, was not different between treated and untreated patients. Conclusions: Pirfenidone treatment was well tolerated by the majority of patients and prevented DLCO decline over a 12-month follow-up.