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

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.

The interstitial lung disease multidisciplinary meeting: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

“…First, the combination of azathioprine, prednisolone and N‐acetylcysteine (NAC) is associated with inferior outcomes in IPF, making the distinction of IPF from other ILD for which immune suppression may be indicated critical . Second, two anti‐fibrotic agents—pirfenidone and nintedanib—have demonstrated efficacy in decreasing disease progression of IPF by approximately 50% and are now standard of care for mild‐moderate IPF . Their efficacies for other types of ILD have not yet been demonstrated and inappropriate use may be associated with a lack of effect, side effects and significant cost.…”

“…Their efficacies for other types of ILD have not yet been demonstrated and inappropriate use may be associated with a lack of effect, side effects and significant cost. Prescription of anti‐fibrotic agents for IPF in Australia requires that the diagnosis has been confirmed at an MDM while in New Zealand, although an MDM consensus diagnosis is not required, only respiratory specialists are able to prescribe pirfenidone …”

Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary

“…Suggested treatment algorithm for idiopathic pulmonary fibrosis (IPF)∗ FVC = forced vital capacity; ILD = interstitial lung disease; MDM = multidisciplinary meeting. ∗ Adapted from Jo et al 11 …”

“…CT = computed tomography; DL co = diffusion capacity of the lungs for carbon monoxide; FEV 1 = forced expiratory volume in 1 second; FVC = forced vital capacity; IPF = idiopathic pulmonary fibrosis. PBS = Pharmaceutical Benefits Scheme; PHARMAC = Pharmaceutical Management Agency; * Adapted from Jo et al 11 † Has only approved pirfenidone.…”

“…This article summarises two recently published position statements from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, outlining the diagnosis 10 and treatment of IPF 11 . They do not represent guidelines per se but are based on current evidence as well as expert opinion.…”