Treatment of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis: A position statement from the Thoracic Society of Australia and New Zealand 2023 revision

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive disease leading to significant morbidity and mortality. In 2017 the Thoracic Society of Australia and New Zealand (TSANZ) and Lung Foundation Australia (LFA) published a position statement on the treatment of IPF. Since that time, subsidized anti-fibrotic therapy in the form of pirfenidone and nintedanib is now available in both Australia and New Zealand. More recently, evidence has been published in support of nintedanib for non-IPF progressive pulmonary fibrosis (PPF). Additionally, there have been numerous publications relating to the non-pharmacologic management of IPF and PPF. This 2023 update to the position statement for treatment of IPF summarizes developments since 2017 and reaffirms the importance of a multi-faceted approach to the management of IPF and progressive pulmonary fibrosis.

Keywords: idiopathic pulmonary fibrosis; interstitial lung disease; progressive pulmonary fibrosis; pulmonary fibrosis; treatment.

FIGURE 1

Suggested algorithm for the management of IPF and PPF. *Subsidized prescribing criteria will vary depending on jurisdiction. Observation of fibrotic disease progression in an ILD other than IPF should prompt review of the disease specific management approach, consideration of re‐evaluation of the diagnosis at an ILD‐MDM and consideration of nintedanib should it be accessible. Enrolment in clinical trials should be considered for all patients with IPF and PPF. Alongside pharmacologic therapies, all ILD patients should be considered for supportive therapies as well as lung transplantation and palliative care where relevant. ILD, interstitial lung disease; MDM, multi‐disciplinary meeting; PPF, progressive pulmonary fibrosis.

FIGURE 2

Pathogenic pathways of IPF and potential clinical trial targets. Current (blue) and proposed (green) molecules assessed in clinical trials for the treatment of idiopathic pulmonary fibrosis. Modulators (→) and inhibitors (┬) are shown directed at different components of the fibrosis pathway. Created with Biorender.com.

FIGURE 3

Transplant window and the unpredictable natural history of IPF necessitating referral early in the disease course. Given the unpredictable nature of IPF, transplant referral should be strongly considered at the time of diagnosis. (A) Rapidly deteriorating patient, whose diagnosis and transplant window occurs synchronously with a subsequent very narrow transplant window. (B) *Acute‐exacerbation of IPF prompting rapid deterioration. Outcomes of transplantation in this context are worse. (C) Linear progression, enabling easier assessment of when transplant window opens. (D) Step‐wise deterioration, a period of stability may give a false sense of reassurance. (E) Slowly progressive disease that may never come to transplant. Figure adapted from Ley et al.