Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal interstitial lung disease. After many drugs failed in clinical trials, improvements in the understanding of the pathogenesis of IPF led to the approval of two drugs that slow the progression of the disease. However, the prognosis for patients with IPF remains poor and the search continues for drugs that inhibit the pathogenic pathways active in IPF to reduce or even halt the progression of the disease. In this article, we review the mechanisms of action of the two approved therapies for IPF (nintedanib and pirfenidone) and of the investigational compounds that are in Phase II trials and discuss the potential for combination therapy in the treatment of IPF.
Keywords:
Idiopathic pulmonary fibrosis.
Copyright © 2017 Elsevier Ltd. All rights reserved.
Publication types
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Review
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Research Support, Non-U.S. Gov't
MeSH terms
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Anti-Inflammatory Agents, Non-Steroidal / therapeutic use*
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Disease Progression
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Drug Therapy, Combination
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Enzyme Inhibitors / therapeutic use*
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Humans
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Idiopathic Pulmonary Fibrosis / drug therapy*
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Idiopathic Pulmonary Fibrosis / metabolism
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Idiopathic Pulmonary Fibrosis / physiopathology
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Indoles / therapeutic use*
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Molecular Targeted Therapy*
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Pyridones / therapeutic use*
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Receptors, Fibroblast Growth Factor / antagonists & inhibitors
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Receptors, Platelet-Derived Growth Factor / antagonists & inhibitors
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Receptors, Vascular Endothelial Growth Factor / antagonists & inhibitors
Substances
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Anti-Inflammatory Agents, Non-Steroidal
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Enzyme Inhibitors
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Indoles
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Pyridones
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Receptors, Fibroblast Growth Factor
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pirfenidone
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Receptors, Platelet-Derived Growth Factor
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Receptors, Vascular Endothelial Growth Factor
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nintedanib