Background: Although phase 3 trials showed significant efficacy and acceptable safety profiles for pirfenidone in mild-to-moderate idiopathic pulmonary fibrosis (IPF), data on advanced IPF are limited.
Objectives: The study aimed to evaluate the efficacy and safety of pirfenidone in advanced IPF patients.
Methods: The clinical data of 138 IPF patients (advanced group: 27%) treated with pirfenidone were retrospectively reviewed and compared between advanced and non-advanced groups. Advanced IPF was defined as (1) forced vital capacity (FVC) < 50% predicted or (2) diffusing capacity for carbon monoxide < 30% predicted.
Results: The mean treatment duration was 51.3 weeks, and lung function analysis was performed in 81 patients (17 in the advanced group). Changes in FVC and total lung capacity (TLC) were significantly reduced at 6 months after treatment in both the advanced (ΔFVC [6 months]: -6.3 [before] vs. 0.7% predicted [after]; ΔTLC: -5.3 vs. 0.8) and non-advanced (ΔFVC: -3.4 vs. 0.5; ΔTLC: -3.1 vs. -0.9) groups. The rate of decline in FVC and TLC was significant before treatment, but not after treatment in the advanced (FVC: -1.27 [before] vs. 0.21% predicted/month [after]; TLC: -0.89 vs. -0.15) and non-advanced (FVC: -0.60 vs. -0.20; TLC: -0.54 vs. -0.17) groups. The advanced group showed a similar rate of adverse events (AEs) (78.4 vs. 88.1%, p = 0.270), but more serious AEs (40.5 vs. 10.9%, p < 0.001) including death (24.3 vs. 5.0%, p = 0.002).
Conclusions: In advanced IPF, pirfenidone showed similar efficacy and safety to non-advanced IPF except for serious AEs, which may be due to the advanced status itself.
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