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Expert Rev Respir Med. 2019 Jan 11. doi: 10.1080/17476348.2019.1568244. [Epub ahead of print]

Existing and emerging treatments for idiopathic pulmonary fibrosis.

Author information

1
a 7th Department of Pneumonology , Hospital for Diseases of the Chest, "Sotiria" , Athens , Greece.
2
b 2nd Department of respiratory Medicine , National and Kapodistrian University of Athens, Attikon Hospital , Athens , Greece.
3
c First Academic Department of Pneumonology , Interstitial Lung Diseases Unit, Hospital for Diseases of the Chest, "Sotiria", Medical School, National and Kapodistrian University of Athens , Athens , Greece.

Abstract

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.

KEYWORDS:

anti-acid treatment; antifibrotic; combination therapy; idiopathic pulmonary fibrosis; mesenchymal stem cells; microbiome; novel pharmacologic compounds; pathogenetic mechanisms; pharmacologic therapy; usual interstitial pneumonia

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