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.

KEYWORDS:

• Idiopathic pulmonary fibrosis
• interstitial lung disease
• nintedanib
• pirfenidone
• molecular targets
• therapeutic approach

Financial & competing interests disclosure

L Richeldi has received grants and personal fees from Boheringer Ingelheim and Intermune, and personal fees from Biogen-Idec, Immune works, Medimmune, Roche, Sanofi-Aventis, Shionogi and Takeda outside of the submitted work. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.