Idiopathic pulmonary fibrosis (IPF) is an incurable lung disease, and patients have very few options once the disease seriously progresses. Now, a study from the University of Turin, Italy, suggests that administration of Esbriet (pirfenidone), an oral antifibrotic agent used to treat IPF, can lead to a significant delay in disease progression, particularly in patients with more preserved lung function. The most suitable strategy to preserve lung capacity and extend life, the finding suggests, may be to intervene early in the development of the disease.
The study, conducted by Carlo Albera, MD, and colleagues, is titled “Efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis with more preserved lung function” and published in the European Respiratory Journal.
“The objectives of this analysis were to better understand the differences in disease progression and to determine whether treatment with pirfenidone is beneficial regardless of baseline disease stage,” the authors wrote.
Researchers conducted a posthoc analysis of 1,247 patients treated with pirfenidone 2403 mg/day or placebo in three Phase 3 controlled trials, CAPACITY (PIPF-004 and PIPF-006 studies) and ASCEND (PIPF-016 study). Two separate methods were used to distinguish between patients with more preserved and less preserved lung function at baseline: the forced vital capacity (FVC) and GAP (gender, age and physiology). Patients with baseline FVC ⩾80% predicted or GAP stage I were considered to have more preserved lung function, while those with baseline FVC <80% predicted or GAP stage II–III were considered to have less preserved lung function (GAP stage II and III patients were grouped together, given the reduced number of patients at stage III). The assessment of the efficacy and safety of the treatment was performed every three months over a period of one year.
Study results showed that pirfenidone treatment significantly delayed disease progression in patients regardless of their level of lung function at baseline, but was particularly effective in those with more preserved lung function.
Although the number of patients with the most severe disease progression (stage III) was lower than those at earlier stages of disease onset (stage I and stage II), the authors believe that the results obtained with pirfenidone in these more “advanced” patients also provide a good basis for predicting a decrease in future mortality, as they had better preserved lung function after the treatment. However, the authors suggest that the sooner the treatment starts, the better it will be to maintain quality life and improve mortality rates.