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Respir Med. 2017 Oct;131:49-57. doi: 10.1016/j.rmed.2017.07.062. Epub 2017 Aug 1.

Therapeutic targets in idiopathic pulmonary fibrosis.

Author information

1
McMaster University, Hamilton, Ontario, Canada. Electronic address: kolbm@mcmaster.ca.
2
Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Essen, Germany.
3
Boehringer Ingelheim Pharma GmbH & Co, KG, Biberach an der Riss, Germany.

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

PMID:
28947042
DOI:
10.1016/j.rmed.2017.07.062
[Indexed for MEDLINE]

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