An update on emerging drugs for the treatment of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis is a chronic, progressive fibrotic lung disease of unknown etiology associated with a high morbidity and mortality. The hallmark of the disease is impaired healing after alveolar epithelial injury in the setting of a genetic predisposition. Development of two new drugs has changed the landscape of the treatment of IPF but more work is needed to improve outcomes and improve survival. Areas covered: The development of two antifibrotic agents, nintedanib and pirfenidone has been an exciting landmark in the treatment of IPF. Current research efforts are focused on developing new drugs, as well as combination of new agents with currently available therapies. New molecules in development target not only the deposition of extracellular matrix, but also upstream pathways including those mediated by immunity. Expert opinion: IPF is a complex and a heterogeneous disease involving several different pathways culminating in fibrosis. Efforts are underway to develop drugs targeting the different pathways. The key to the successful treatment of IPF will require identification of better end-points for research as well as precision medicine involving the use of multidrug therapy personalized to specific patients based on endomolecular genotyping.

Keywords: IPF; IPF trials; nintedanib; novel agents; pirfenidone.