Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia.

2017-10

Jo, Helen E

Troy, Lauren K

Keir, Gregory

Chambers, Daniel C

Holland, Anne

Goh, Nicole S L

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

nintedanib, pirfenidone

idiopathic pulmonary fibrosis

interstitial lung disease

treatment

Journal Article

0000-0003-1183-2729

0000-0002-5118-2890

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10.1111/resp.13146

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.

link

Respirology (Carlton, Vic.) 2017; 22(7): 1436-1458

Respirology (Carlton, Vic.)