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J Thorac Dis. 2013 Feb;5(1):48-73. doi: 10.3978/j.issn.2072-1439.2012.12.07.

A review of current and novel therapies for idiopathic pulmonary fibrosis.

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1
Division of Pulmonary, Critical Care, and Sleep Medicine, University of California, Davis, School of Medicine and VA Northern California Health Care System, Sacramento, California, USA;

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic interstitial lung disease that is associated with a median survival of 2-3 years from initial diagnosis. To date, there is no treatment approved for IPF in the United States, and only one pharmacological agent has been approved outside of the United States. Nevertheless, research over the past 10 years has provided us with a wealth of information on its histopathology, diagnostic work-up, and a greater understanding of its pathophysiology. Specifically, IPF is no longer thought to be a predominantly pro-inflammatory disorder. Rather, the fibrosis in IPF is increasingly understood to be the result of a fibroproliferative and aberrant wound healing cascade. The development of therapeutic targets has shifted in accord with this paradigm change. This review highlights the current understanding of IPF, and the recent as well as novel therapeutics being explored in clinical trials for the treatment of this devastating disease.

KEYWORDS:

Idiopathic pulmonary fibrosis/drug therapy; clinical trials; idiopathic pulmonary fibrosis/pathology; molecular targeted therapy

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