Investigational approaches to therapies for idiopathic pulmonary fibrosis — gomer 2011

Background: Idiopathic pulmonary fibrosis (IPF), a lung condition that is chronic, progressive, and fibrotic, is becoming more common. The effectiveness of pirfenidone was assessed in this study using longitudinal changes in individual slope of forced vital capacity (FVC) before and after pirfenidone treatment in IPF patients registered in the European registry for idiopathic pulmonary fibrosis (eurIPFreg). Secondary factors included the Borg dyspnea scale, the six-minute walking distance (6MWD), the individual slope of carbon monoxide diffusion capacity (DLco), and survival analyses. Results: The data of 122 eurIPFreg patients who underwent at least two pulmonary function tests (PFTs) prior to or while using pirfenidone were evaluated using slope-changes. The Borg dyspnea scale flattened (p = 0.02), and the total analysis revealed a decrease in DLco and an increase in 6MWD (both p 0.0001). The annual FVC drop under pirfenidone was reduced by around 50% on average with a slope shift of +1.48 0.28 (% per annum (p.a)) after treatment began. The median survival time during treatment was 4.82 years. Patients with more severe illnesses (FVC decline > 10% pred. p.a.), previous smokers, patients older than 60, and patients with FVC decline > 80% pred.) seemed to benefit more from pirfenidone treatment. Conclusions: We discuss the effectiveness of pirfenidone using outcome data from a European IPF cohort that has been followed for up to 9 years. Global analyses revealed that pirfenidone has a preventive effect against the progressive decline in lung function. Age at the beginning of treatment and Gender-Age-Physiology (GAP) score both had an effect on survival.

Gomer RH, Lupher ML Jr. Investigational approaches to therapies for idiopathic pulmonary fibrosis. Expert Opin Investig Drugs. 2010 Jun;19(6):737-45. doi: 10.1517/13543784.2010.484018. PMID: 20443753; PMCID: PMC2881476.