OBJECTIVES: Data on the effectiveness of pirfenidone in real-world patients with IPF are limited; this study aimed at assessing the effectiveness and tolerability of pirfenidone in a large cohort of IPF Italian patients.

METHODS: IRENE was an observational, retrospective cohort study. Data were collected from clinical charts to evaluate change from baseline in FVC mL, FVC% predicted forced vital capacity (%FVC) and percentage of patients with ≥10% decline in %FVC at Month 12. Adverse events (AE) were also collected and analyzed.

RESULTS: The 18 centers participating to the study enrolled a cohort of 379 eligible consecutive patients. At Month 12, mean change from baseline in FVC mL was -81.82 mL. (SD: 419,81 mL; P =0.002), in %FVC was −0.82% (SD: 10.64%; paired t-test, P=0.199), and 16% of patients (95% CI: 12.2–20.9%) had ≥10% decline in %FVC. 211 AEs occurred in 149 patients (39.3%); 112 AEs in 95 patients (25.1%) were deemed related to pirfenidone treatment. AEs led to pirfenidone discontinuation in 2.4% of patients. The most frequent AEs were skin- (14% of patients) and gastrointestinal-related (12.4%).

CONCLUSIONS: The results from this real-world nationwide IPF study demonstrated that the effectiveness and tolerability of pirfenidone were comparable to phase 3 clinical trial and other real-world data.