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Alison Hey-Cunningham +61 2 9515 6651    

Email
alison.hey-cunningham@sydney.edu.au

Address
Centre of Research Excellence in Pulmonary Fibrosis
Respiratory Medicine and Sleep Unit
Level 11, Royal Prince Alfred Hospital
Camperdown NSW 2050

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Projects

 

Assessing the burden and economic impact of PF in Australia

Investigators: Dr Ingrid Cox, Dr Qiang Zheng, Dr Barbara de Graaff, Prof Haydn Walters, Prof Andrew Palmer, A/Prof Tamera Corte and A/Prof Ian Glaspole.

The Health Economics and Epidemiology research group at the Menzies Institute for Medical Research at the University of Tasmania, led by Profs Andrew Palmer and Haydn Walters, have have made great progress in the understanding of the true epidemiological profile and economic impact of IPF in Australia. Prior to the CRE-PF, there were no published epidemiological studies estimating the burden of IPF in Australia.

The team has recently demonstrated Australian IPF prevalence and incidence (age standardised) rates to be 34.3 per 100,000 and 11.0 per 100,000 person years respectively – the first Australian estimates to date (Cox et al., 2021a). We have shown that IPF mortality in Australia has increased over 2000-2017, but since the advent of anti-fibrotic medications in 2017, there has been an increasing trend towards survival for IPF patients (Zheng et al., 2021). Our systematic review and meta-analysis of health-related quality of life of IPF patients confirmed that IPF negatively affects health-related quality of life, mostly impacting the physical health domains (Cox et al., 2020). In a study addressing health related quality of life in Australian IPF patients, we have demonstrated that IPF disease severity and comorbidities had the greatest impact on quality of life (Cox et al., 2021b).

In the first comprehensive cost of illness study for IPF in Australia, the team found that the highest costs were from antifibrotic medications, hospital admissions and medications for comorbidities. The average cost per person annually was approximately $32,000 (manuscript under review). In a recent review of antifibrotic use in Australia, we have shown that the associated total cost of antifibrotics for IPF was $131,377,951 over the period 2017-2020 in Australia (Cox et al., 2021c).

Much of this work is ongoing, including a study of the effect of ambient air pollutants (PM2.5 and NO2) on lung function, societal costs of IPF in Australia and mortality of persons living with IPF. 

 


Accurate clinical phenotyping of IPF patients, informing the development and application of relevant and individualised management strategies

Investigators: A/Prof Yuben Moodley, Prof Haydn Walters, A/Prof Tamera Corte and A/Prof Ian Glaspole.

Individuals affected with IPF may demonstrate gradually progressive disease, a stable disease course, or an accelerated decline, with or without acute respiratory worsening. These subgroups may represent distinct phenotypes of IPF or reflect other factors contributing to the disease.

We are conducting a large-scale Australian study in the IPF population to identify clinical and physiological factors, that when integrated with systems biology-based biomarker collations, may assist clinicians in predicting natural history for individual IPF patients. 

As part of clinically phenotyping PF patients, we have examined implications of the diagnostic criteria of IPF in clinical practice (Jo et al., 2018), exposures and associations with clinical phenotypes in hypersensitivity pneumonitis (Barnes et al., 2021) and baseline characteristics and survival of an Australian interstitial pneumonia with autoimmune features cohort (Jee et al., 2021).

 


Real-world data on anti-fibrotic medications

Investigators: A/Prof Tamera Corte, A/Prof Ian Glaspole, Dr Helen Jo and Dr Alan Teoh.

Two anti-fibrotic therapies, nintedanib and pirfenidone, are registered on the PBS in Australia. The true impact of these medications beyond the specific and narrow inclusion criteria of clinical trials was not understood, nor was their impact beyond the 52-week study period. We are therefore conducting real-world post-marketing studies into the beneficial and adverse effects in IPF patients to inform prescription practices and future guidelines for the use of these two medications. The team has reported trends in nintedanib and pirfenidone utilisation for IPF in Australia (Cox et al., 2021c), eligibility for anti-fibrotic treatment in IPF (Burgess et al., 2019) and a systematic review of prognosis of IPF without anti-fibrotic therapy (Khor et al., 2020).

We're also phenotyping patients receiving anti-fibrotic therapy and studied candidate biomarkers to predict both treatment response and failure.

 


Identification of an IPF molecular signature for the accurate diagnosis and phenotyping of PF Patients

Investigators: A/Prof Yuben Moodley, A/Prof Tamera Corte, Dr Dino Tan, Dr Britt Clynick, Prof Geoff Laurent and Prof Darryl Knight.

We are working to identify molecular signatures of IPF to: (1) improve and refine diagnostic accuracy; (2) allow accurate phenotyping specifically for IPF patients with rapid disease progression; and (3) study candidate proteins identified by systems proteomic analysis, in lung tissue, providing insights into pathogenesis of the disease.

The CRE-PF has extended the work of a previous NHMRC funded project, profiling known biomarkers in a subset of the AIPFR and providing novel, urgently needed information in relation to the temporal expression of biomarkers in IPF. Using 3 independent IPF cohorts including the Australian IPF Registry (AIPFR), Trent Lung Fibrosis (TLF; UK) and Prospective Observation of Fibrosis in the Lung Clinical Endpoints (PROFILE; UK), we identified a panel of circulatory biomarkers can provide potentially valuable clinical assistance in the prognosis of IPF patients. We described biomarker signatures for progressive IPF, with differential biomarker expression observed by ELISA and replicated for osteopontin, matrix metallopeptidase-7, intercellular adhesion molecule-1 and periostin for those with a progressor phenotype at one-year (Clynick et al., 2021). 

Using lipidomics and metabolomics technology, we were also the first to characterise abnormalities in lipid metabolism between IPF patients who progressed vs those who are stable (Nambiar et al., 2021a) and between IPF patients vs patients with other chronic lung conditions (Nambiar et al., 2021b). We have also published studies of unique circulating proteins in IPF (Moodley et al., 2019), circulating RNA in IPF stable and progressing patients (Clynick et al., 2020) and the impact of elevated monocyte count on survival of IPF patients (Teoh et al., 2020). 

The team’s novel research is ongoing, including work to investigate the science of early lung injury and fibrosis and PF progression. We look forward to seeing their innovative research continue, moving to benefit people living with PF, by improving the ability to predict disease progression, allowing a personalised approach to PF treatment.

 


Improving patient-centred outcomes in PF with pulmonary rehabilitation

Investigators: Prof Anne Holland, Dr Leona Dowman, Dr Anthony May, A/Prof Tamera Corte, Prof Dan Chambers, A/Prof Ian Glaspole

We are conducting a world-first randomised controlled trial of optimised pulmonary rehabilitation to improve quality of life in PF. Alongside this trial, we are conducting qualitative research to provide an in-depth understanding of the barriers and facilitators to the provision of pulmonary rehabilitation for people with pulmonary fibrosis, including exercise and non-exercise components.

The aim of this clinical trial is to test the effects of an optimised exercise training protocol in people with interstitial lung disease, using high intensity exercise training (HIIT). This is a randomised controlled trial, being conducted 4 sites (Austin Health, Alfred Health, Royal Prince Alfred Hospital, The Prince Charles Hospital). The trial compares high intensity interval training to moderate intensity continuous training (standard care).

The team have published the trial protocol (Dowman et al., 2021a) and reported attenuation of exertional desaturation and preference for interval exercise compared to continuous exercise in people with interstitial lung disease (Dowman et al., 2021b). While the trial is underway, Cochrane reviews of pulmonary rehabilitation for ILD (Dowman et al., 2021c) and telerehabilitation for chronic respiratory disease (Cox et al., 2021d) have been published.

This trial has been impacted by COVID-19, with closure of centre-based pulmonary rehabilitation at all sites for variable periods since early 2020. Despite COVID-related interruptions, more than two thirds of the planned participants have been recruited and recruitment is anticipated to be complete by end 2022 or early 2023.