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Treatment of idiopathic pulmonary fibrosis in A ustralia and N ew Zealand: A position statement from the T horacic S ociety of A ustralia and N ew Z ealand and the L ung F oundation A ustralia
Author(s) -
Jo Helen E.,
Troy Lauren K.,
Keir Gregory,
Chambers Daniel C.,
Holland Anne,
Goh Nicole,
Wilsher Margaret,
de Boer Sally,
Moodley Yuben,
Grainge Christopher,
Whitford Helen,
Chapman Sally,
Reynolds Paul N.,
Glaspole Ian,
Beatson David,
Jones Leonie,
Hopkins Peter,
Corte Tamera J.
Publication year - 2017
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.13146
Subject(s) - medicine , nintedanib , idiopathic pulmonary fibrosis , interstitial lung disease , pirfenidone , population , disease , clinical trial , pulmonary function testing , intensive care medicine , lung , environmental health
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.