Pirfenidone in Idiopathic Pulmonary Fibrosis: Real-World Observation on Efficacy and Safety, Focus on Patients Undergoing Antithrombotic and Anticoagulant

Abstract

Idiopathic pulmonary fibrosis (IPF) is a rare and progressive interstitial lung disease characterized by irreversible distortion of lung architecture and subsequent loss of pulmonary function. Pirfenidone is an antifibrotic agent associated with increased progression-free survival and overall survival rates, but it carries multiple side effects. The aim of the study was to examine the efficacy and safety profile of pirfenidone in a real-life context, with a focus on the concomitant use of antithrombotic and/or anticoagulant treatments. The clinical and functional data (forced vital capacity [FVC], forced expiratory volume in 1 s [FEV1], diffusing lung capacity for carbon monoxide [DLCO], and 6 min walking test distance [6MWD]) of all IPF patients treated with pirfenidone and referred to our two centers between 2019 and 2022 were retrospectively analyzed at baseline, 6 and 12 months after the start of treatment. A total of 55 IPF subjects undergoing pirfenidone treatment were included in the analysis (45.5% females, median [IQR] age at disease onset 68.0 [10.0] years, median [IQR] age at baseline 69.0 [10.8] years). Compared to baseline, at 12 months, FVC (86.0% vs. 80.0%; p = 0.023) and DLCO (44.0% vs. 40.0%; p = 0.002) were significantly reduced, while FEV1 (p = 0.304) and 6MWD (p = 0.276) remained stable; no significant change was recorded at 6 months. Most of the reported adverse events were mild or moderate. Gastrointestinal intolerance (9.1%) was the main cause of treatment discontinuation. A total of 5% of patients reported at least one minor bleeding event, although all episodes occurred in those receiving concomitant antithrombotic or anticoagulant. Overall, this real-life experience confirms the efficacy and safety profile of pirfenidone in the case of the concomitant use of antithrombotic and/or anticoagulant drugs.

Keywords: anticoagulants; antithrombotic treatment; idiopathic pulmonary fibrosis (IPF); interstitial lung disease (ILD); pirfenidone; pulmonary function test (PFT).

Figure 1

Comparison of lung function test measurements at baseline (green color) and 6 months (orange color). The two graphs represent (A) FVC, (B) FEV1, (C) DLCO, and (D) 6MWD. In the left part, each dot represents a patient, and each line reveals the same patient’s evolution over time. In the right part, data are presented as bar plots. Comparisons between pulmonary function test measurements and 6MWD at different time points were conducted using the paired t-test.

Figure 2

Comparison of lung function test measurements at baseline (green color) and 12 months (orange color). The two graphs represent (A) FVC, (B) FEV1, (C) DLCO, and (D) 6MWD. In the left part, each dot represents a patient, and each line reveals the same patient’s evolution over time. In the right part, data are presented as bar plots. Comparisons between pulmonary function test measurements and 6MWD at different time points were conducted using the paired t-test.

Figure 3

Kaplan–Meier estimates of the probability of survival at 12 months.