Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive and invariable fatal interstitial lung disease. Current antifibrotic treatment halts disease progression but do not cure the disease itself. In the last decade, a substantial understanding in 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.