Existing and emerging treatments for idiopathic pulmonary fibrosis

ABSTRACT

Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive and invariable fatal interstitial lung disease. Current antifibrotic treatment halts disease progression but does not cure the disease itself. In the last decade, a substantial understanding of disease pathobiological mechanisms led to the development of numerous clinical trials testing promising pharmacologic agents.

Areas covered: In this review, we summarize and discuss current and emerging pharmacological therapies for IPF and highlight the potential of different promising investigational compounds in phase II-IV trials with positive or pending results.

Expert commentary: Existing therapies for IPF slow disease progression and recent advances in understanding IPF complex pathogenesis unfolded multiple new possible therapeutic targets. A relevant number of promising clinical trials targeted specific biologic pathways, are ongoing or upcoming, but we are far away from a definitive cure of IPF soon. An ‘oncologic approach’ via tailoring medicine could be a realistic therapeutic intervention that may improve expectancy and quality of life in IPF.

KEYWORDS:

• Idiopathic pulmonary fibrosis
• usual interstitial pneumonia
• pathogenetic mechanisms

Declaration of interest

Two authors have received lecture feeds, department grants and travel support from Boehringer Ingelheim and Roche. The remaining authors have no 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. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewers Disclosure

Peer reviewers on this manuscript have no relevant financial relationships or otherwise to disclose.

Additional information

Funding

This paper was not funded.