About us

Funded 2023-2028 by the Australian National Health and Medical Research Council (NHMRC), this Centre of Research Excellence (CRE) aims to transform and extend the lives of people with interstitial lung disease through individualised care. We are achieving this through a comprehensive and cutting-edge bench to bedside research, education and capacity-building program that directly addresses patient-identified priorities and translates best practice care to people with interstitial lung disease across Australia.

Figure: CRE-PF bench to bedside program approach

The interstitial lung diseases (ILDs) are a complex group of more than 200 debilitating lung disorders. All ILDs are characterised by inflammation and fibrosis of the pulmonary tissue, leading to progressive breathlessness, reduction in exercise capacity and, often, rapid death. Until recently, the focus of ILD research has been on one form, idiopathic pulmonary fibrosis (IPF), a progressive and fatal lung disorder with a median survival of only 2-5 years from diagnosis, worse than many cancers. IPF is a specific diagnosis that only encompasses 30% of the spectrum of ILD in Australia and world-wide. However, it is now clear that many of the recent advances in understanding the causes, treatment options and factors influencing outcome in IPF, apply more broadly across the spectrum of all ILDs. With our successful prior CRE (2017-2023) our team is at the forefront of international research and several key developments which now prompt a new and individualised approach to care, transforming the lives of patients with ILD. 

Impact

Our Opportunity: Individualised Care. While the advent of anti-fibrotic therapy was a major breakthrough, these drugs only slow inexorable disease progression, and are fraught with frequent complications. These limitations reflect their non-specific mode of action, targeting the final, fibrotic step of the pathogenic cascade. Guided by an ever-improving understanding of pathogenesis, drug development has evolved across multiple disease categories including cancer and cystic fibrosis to become more precise, culminating in the development of truly disease modifying therapies targeting fundamental pathogenic mechanisms. ILD treatment is poised to undergo a similar transformation, with CRE investigators at the cutting edge globally. Recent findings of the roles of aberrant telomere biology, other genetic mutations, and alveolar macrophage dysfunction in ILD pathogenesis are driving precise and truly disease modifying drug discoveries, on the cusp of completely transforming the management and prognosis for people living with ILD. 

With the relatively recent Australian regulatory approval for the antifibrotic, nintedanib (November 2021) for progressive ILD, beyond IPF, this CRE is perfectly timed to capitalise on these recent scientific breakthroughs, transforming the diagnosis and management of ILD through Individualised Care for better outcomes for ILD patients. 

Governance

The CRE-PF is led by a Steering Committee, chaired by Prof Tamera Corte, and encompasses four subcommittees: Translation, EDucation and Support (TEDS); CRE Advanced Training Environment (CREATE); Pulmonary fibrosis Australasian Clinical Trials (PACT) network; and the Australasian Interstitial Lung Disease Registry (AILDR). The CRE-PF Steering Committee makes decisions regarding the prioritisation and implementation of research, including ensuring there is no duplication of research efforts, as well as the administration of CRE-PF funds.

The CRE-PF Project Managers, Dr Alison Hey-Cunningham, Ms Jennifer Walsh and Ms Sacha Macansh, provide administrative support through coordination of day-to-day activities to facilitate collaboration between CR-PF members and the implementation and delivery of CRE-PF’s research and communication strategy.

Support the CRE-PF

You can support the work of the CRE-PF by donating through the University of Sydney: Give.sydney.edu.au/pulmonaryfibrosis