Assessing the Effectiveness of Pirfenidone in Idiopathic Pulmonary Fibrosis: Long-Term, Real-World Data from European IPF Registry (eurIPFreg) — Krauss 2020
Background: The prevalence of idiopathic pulmonary fibrosis (IPF), a chronic, progressive, fibrotic lung disease, is increasing. In this study, IPF patients enrolled in the European registry for idiopathic pulmonary fibrosis were evaluated for the efficacy of pirfenidone using longitudinal changes in individual slope of forced vital capacity (FVC) before and after pirfenidone medication (eurIPFreg). The Borg dyspnea scale, the six-minute walking distance (6MWD), the individual slope of the lungs' carbon monoxide diffusion capacity (DLco), and survival analyses were considered secondary variables. Results: Slope-changes were used to evaluate the data of 122 eurIPFreg patients who had at least two pulmonary function tests (PFTs) before to or while receiving pirfenidone medication. The overall analysis showed a reduction in DLco and an increase in 6MWD (both p 0.0001), as well as a flattening of the Borg dyspnea scale (p = 0.02). This average slope change after treatment started was +1.48 0.28 (% per annum (p.a)), reflecting an approximate 50% reduction in the annual FVC decline under pirfenidone. 4.82 years was the median survival time during treatment. FVC decline >10% pred. p.a.), past smokers, patients >60 years of age, and patients with more restrictive illness (FVC 80% pred.) appeared to benefit more from pirfenidone treatment. Conclusions: In a European IPF cohort with outcome data spanning up to 9 years, we report on the efficacy of pirfenidone. Global evaluations showed that pirfenidone has a protective effect against the deterioration of lung function over time. Gender-Age-Physiology (GAP) score and age at the start of treatment both had an impact on survival.
Krauss E, Tello S, Wilhelm J, Schmidt J, Stoehr M, Seeger W, Dartsch RC, Crestani B, Guenther A. Assessing the Effectiveness of Pirfenidone in Idiopathic Pulmonary Fibrosis: Long-Term, Real-World Data from European IPF Registry (eurIPFreg). J Clin Med. 2020 Nov 22;9(11):3763. doi: 10.3390/jcm9113763. PMID: 33266405; PMCID: PMC7700641.
