Assessing the effectiveness of pirfenidone in changing the natural course of idiopathic pulmonary fibrosis: the data from European IPF Registry (eurIPFreg)

Background: Longitudinal response to treatment with pirfenidone is highly variable. This study evaluated the effectiveness of pirfenidone, as measured by longitutidal changes in FVC (% pred.), DLco (% pred), Borg dyspnoe scale, and six minute walking distance test, as well as survival analyses. Methods: Data were available for 122 eurIPFreg patients. Patients’ data from at least two subsequent records with/without antifibrotic treatment were analyzed by slope-changes in each individual patient, as well as in global analysis, where the data from all patients were used to fit a global segmented regression model. Results: The individual analysis showed a tremendous heterogeneity, and the mean FVC slope difference was not significantly different from baseline (p = 0.32). The global analysis revealed an average slope change of +1.48 (% p.a.) ± 0.28 (p Conclusions: We report the effectiveness of pirfenidone in European IPF cohort with outcome data extending up to 9 years. Global analyses demonstrated a positive effect of pirfenidone on the decline of the lung function over time.