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Pirfenidone in the treatment of idiopathic pulmonary fibrosis: an evidence-based review of its place in therapy

Authors Margaritopoulos G, Vasarmidi E, Antoniou K

Received 24 August 2015

Accepted for publication 21 March 2016

Published 1 July 2016 Volume 2016:11 Pages 11—22


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 6

Editor who approved publication: Professor Garry Walsh

George A Margaritopoulos,1 Eirini Vasarmidi,2 Katerina M Antoniou2

1Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK; 2Department of Thoracic Medicine and Laboratory of Molecular and Cellular Pneumonology, Interstitial Lung Disease Unit, University Hospital of Heraklion, Heraklion, Greece

Abstract: The landscape of idiopathic pulmonary fibrosis (IPF) has changed. The significant progress regarding our knowledge on the pathogenesis of the disease together with the experience achieved after a series of negative trials has led to the development of two drugs for the treatment of IPF. Both pirfenidone and nintedanib can slow significantly the rate of disease progression. They are safe with side effects that can be either prevented by close collaboration between health care professionals and patients or treated successfully when they occur, rarely leading to treatment discontinuation. However, there are still few unanswered questions regarding the application of the beneficial results of pharmaceutical trials in the general population of IPF patients. Long-term “real-life” studies are being undertaken to answer these questions. In this article, we focus on the advances that have led to the development of the antifibrotic agents with particular focus on pirfenidone.

Keywords: antifibrotic, pirfenidone, photosensitivity, nausea

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