ALEXANDRIA, Va. — Pirfenidone (Esbriet) can improve life expectancy compared with best supportive care and lower the risk of lung function decline for patients with idiopathic pulmonary fibrosis, an irreversible and unpredictable disease of unknown etiology that makes breathing difficult and causes permanent scarring damage to the lungs, according to two studies published in the March issue of the Journal of Managed Care & Specialty Pharmacy.
IPF is has no known cause and is fatal, often resulting in death in two to five years after diagnosis. It affects approximately 100,000 Americans and typically occurs in people older than 50. Historically, more men than women have been diagnosed with IPF. Common symptoms include a persistent dry cough; shortness of breath, sometimes even during normal daily activities; and “Velcro-like” crackles at the bases of the lungs, which a doctor can hear with a stethoscope. IPF is often initially misdiagnosed with other more common diseases such as asthma or chronic obstructive pulmonary disease. It can take months or even years to accurately diagnose IPF because the symptoms of IPF are very non-specific.
The first study, “Predicting Life Expectancy for Pirfenidone in Idiopathic Pulmonary Fibrosis,” revealed the Food and Drug Administration-approved drug, manufactured by Genenetech, can increase life expectancy in patients by 2.47 years compared to best supportive care. This study was funded by InterMune International AG, a wholly owned Roche subsidiary since 2014.
The second study, “Systematic Review and Network Meta-analysis of Idiopathic Pulmonary Fibrosis Treatments,” concluded pirfenidone may reduce the odds of experiencing a decline in percent predicted FVC of ≥10% compared with placebo in the first year of treatment. The results of the analysis also suggest that pirfenidone improves survival. FVC refers to Forced Vital Capacity, which is a measure of breath a person can exhale.