| Literature DB >> 28845557 |
Helen E Jo1,2, Lauren K Troy1,2, Gregory Keir3, Daniel C Chambers4, Anne Holland5, Nicole Goh4,6, Margaret Wilsher7, Sally de Boer7, Yuben Moodley8, Christopher Grainge9, Helen Whitford10, Sally Chapman11, Paul N Reynolds11, Ian Glaspole10, David Beatson12, Leonie Jones9, Peter Hopkins4, Tamera J Corte1,2.
Abstract
Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.Entities:
Keywords: nintedanib, pirfenidone; idiopathic pulmonary fibrosis; interstitial lung disease; treatment
Mesh:
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Year: 2017 PMID: 28845557 DOI: 10.1111/resp.13146
Source DB: PubMed Journal: Respirology ISSN: 1323-7799 Impact factor: 6.424