title: Novel drug targets for idiopathic pulmonary fibrosis.
abstract: Idiopathic Pulmonary Fibrosis (IPF) is a progressive, fatal lung disorder of unknown cause with a highly variable and unpredictable clinical course. The advances made in deciphering IPF pathobiology over the last decades have led to the approval of two anti-fibrotic molecules, pirfenidone and nintedanib, that showed to be effective in significantly reducing the rate of progression of the disease. Such pharmacological breakthroughs represent a dramatic change in the management of these patients and are reflected in updated international guidelines. However, the need to find a cure for this devastating disease remains unmet and the development of novel therapeutic agents remains hurdled by several factors. Here, we review the latest insights into therapeutic approaches for IPF and the available evidence for the most promising novel compounds currently under development, and discuss the challenges and evolution of IPF clinical research over the next few years.
authors: a Southampton NIHR Respiratory Biomedical Research Unit , University Hospital Southampton , Southampton , UK.
authors: Giacomo
authors: G
authors: Sgalla
authors: b Department of Cardiologic, Thoracic and Vascular Sciences, Section of Respiratory Diseases , University of Padova , Padova , Italy.
authors: Elisabetta
authors: E
authors: Cocconcelli
authors: c Department of Respiratory Diseases , University Hospital of Modena , Modena , Italy.
authors: Roberto
authors: R
authors: Tonelli
authors: a Southampton NIHR Respiratory Biomedical Research Unit , University Hospital Southampton , Southampton , UK.|d Academic Unit of Clinical and Experimental Sciences, Faculty of Medicine , University Hospital Southampton , Southampton , UK.
authors: Luca
authors: L
authors: Richeldi
keywords: Idiopathic pulmonary fibrosis; interstitial lung disease; molecular targets; nintedanib; pirfenidone; therapeutic approach