Effects of Pirfenidone on Idiopathic Pulmonary Fibrosis Progression and Safety: Results of Multicenter Prospective Observational Study

SIMPLE SUMMARY: Idiopathic pulmonary fibrosis (IPF) commonly occurs in men in their 60s and results in replacement of the lungs parenchyma with fibrotic tissue. Usually, with such a diagnosis, patients die within 3–5 years. This disease is relatively rare, with approximately 1 case per 10,000 populations. Our study followed 55 patients who took pirfenidone, a medicine which can stop fatal changes in the lungs. This was a multicenter prospective study involving 11 expert centers in different parts of the country. Patients took the medicine and were examined in the usual way with regard to their real life conditions. For the efficacy assessment, we used not only the traditional outcomes (symptoms, pulmonary function, etc.), but also high-resolution computed tomography score and European Quality of Life 5-Dimension Questionnaire. In general, in 80–90% of patients, the disease did not progress. In the study, there was a very thorough evaluation of safety and tolerability of treatment with pirfenidone. Serious adverse effects were not registered when taking the medication. ABSTRACT: The aim of this study was to determine the effectiveness of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) seen in clinical practice. Fifty-five adults with IPF were enrolled in this multicenter, open-label, non-randomized, non-controlled, interventional clinical study. All patients received pirfenidone 2403 mg/day (three 267 mg capsules three times daily) for 26 weeks. After 26 weeks of treatment, the mean change in absolute forced vital capacity (FVC) was 128.8 mL (95% confidence interval [CI] −26.8, 284.4) and the mean change in relative predicted FVC was −0.10% (95% CI −3.18, 2.99). Stable disease (defined as improvement of ≥0% or a decline of <10% to 0% of the corresponding FVC value) was observed in most patients (relative FVC, 90.9%; absolute FVC, 83.6%). There was no statistically significant change in the mean high-resolution computed tomography fibrosis score or lung opacity score at week 26 compared with baseline. Treatment-emergent adverse events were reported in 80% of patients during the treatment period; most of them were mild or moderate in severity. No serious pirfenidone-related adverse events were observed during the study period. Pirfenidone was generally safe and effective for controlling functional decline and stabilizing disease in patients with IPF encountered in clinical practice in Russia.