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Idiopathic Pulmonary Fibrosis: New and Emerging Treatment Options

  • Richard J. Hewitt
  • Toby M. MaherEmail author
Leading Article

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and debilitating, scarring lung disease with a worse prognosis than some cancers. The incidence of IPF is increasing and while current antifibrotic therapies slow disease progression, they do not offer a cure. The pathobiology of IPF is complex and is driven by aging-associated cellular dysfunction, epithelial injury, and an aberrant wound-healing response characterised by fibroblast activation and extracellular matrix accumulation (ECM) in the interstitium. As understanding of the underlying mechanisms has evolved, new targets for pharmacotherapy have emerged. Novel drugs currently in development for pulmonary fibrosis have diverse molecular properties and mechanisms of action, as well as different routes of administration. A shared primary goal of these agents is reduction of the profibrotic activity of fibroblasts and limitation of ECM deposition, which hinders gas exchange and ultimately leads to respiratory failure. This article provides an overview of some promising new therapeutic options for IPF and considers the challenges for future drug development.

Notes

Acknowledgements

Toby M. Maher is supported by a National Institute for Health Research (NIHR) Clinician Scientist Fellowship (NIHR Ref: CS-2013-13-017) and a British Lung Foundation Chair in Respiratory Research (C17-3).

Compliance with Ethical Standards

Conflict of Interest

Richard J. Hewitt has no conflicts of interest. Toby M. Maher has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB, and has received consultancy or speakers’ fees from Apellis, AstraZeneca, Bayer, Biogen Idec, Boehringer Ingelheim, Galapagos, GlaxoSmithKline R&D, Indalo, Pliant, ProMetic, Roche, Samumed and UCB.

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.National Heart and Lung InstituteImperial College LondonLondonUK
  2. 2.Interstitial Lung Disease UnitRoyal Brompton HospitalLondonUK

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