Broad Therapeutic Efficacy of Nintedanib in Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a variably progressive disease that carries a poor prognosis. Recent international multicenter phase III clinical trials testing the efficacy of nintedanib (1) and pirfenidone (2) have shown that medical therapy, specifically antifibrotic agents, can reduce the rate of lung function decline in patients with IPF. In the United States, these groundbreaking findings were quickly followed by U.S. Food and Drug Administration fast-track priority review, orphan product designation, and drug approval for both pirfenidone and nintedanib (3). Nintedanib, a small-molecule tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor, fibroblast growth factor receptor, and platelet-derived growth factor receptor, was tested in two simultaneous phase III trials (INPULSIS-1 and INPULSIS-2) (1). Compared with placebo, nintedanib consistently slowed disease progression by significantly reducing the annual rate of decline in forced vital capacity (FVC) in both INPULSIS trials (1). Comparable reductions in disease progression were reported for pirfenidone, raising the possibility that overlapping mechanisms mediate these remarkably similar antifibrotic effects.