New targets in idiopathic pulmonary fibrosis: from inflammation and immunity to remodeling and repair

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic respiratory disease whose incidence and prevalence are increasing. Recent evidence has led to a change of paradigm regarding understanding of the underlying pathophysiology. In parallel, we have witnessed the development and rise of the first anti-fibrotic drugs, namely pirfenidone and nintedanib. However, with clinical results being below expectations there is a clear need for new medications in this field. Areas covered: After covering new mechanisms involved in IPF pathogenesis, the present review discusses current clinical trials and deciphers potential new targets in light of in vitro and in vivo experimental studies. Expert Opinion: All in all, we believe that future development will require (1) a significant improvement in experimental models, (2) a proper selection and characterization of patients, allowing us to foresee which will respond to a given treatment and (3) an evaluation of combined therapies, targeting different pathways.