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Review
. 2017 Nov;9(11):4700-4707.
doi: 10.21037/jtd.2017.10.111.

Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age

Jonathon Shaw  1 , Tracey Marshall  1 , Helen Morris  1 , Conal Hayton  1 , Nazia Chaudhuri  1
Affiliations
Review

Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age

Jonathon Shaw et al. J Thorac Dis. 2017 Nov.

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common cause of interstitial lung disease (ILD) and carries a worse prognosis than many cancers. Until recently, there were no active treatment options available for patients with IPF, meaning palliation or lung transplantation in selected patients were the only options. The management of IPF has changed dramatically over the last decade with the advent of two antifibrotic agents; pirfenidone and nintedanib. These new agents have been shown to reduce decline in lung function and pirfenidone has been shown to reduce mortality. The changing landscape of IPF diagnosis and management present a number of issues that may be encountered including management of side effects related to antifibrotic therapy. This article aims to give an overview of the holistic approach to the management of patients with IPF, including antifibrotic management, symptom management and the invaluable role of the ILD specialist nurse.

Keywords: Idiopathic pulmonary fibrosis (IPF); cough; dyspnea; interstitial lung diseases (ILDs); therapeutics.

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Conflict of interest statement

Conflicts of Interest: N Chaudhuri has received educational funding and project grants from Boehringer Ingelheim, Intermune and Roche. These funds are not related to this publication. The other authors have no conflicts of interest to declare.

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