Idiopathic pulmonary fibrosis is a common and devastating lung disease often found in those aged 50 and over.
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There is no cure, with an average survival rate of just three years.
However, new research underway hopes to unlock the early pathology of IPF, to enable health professionals to diagnose IPF earlier and more accurately, paving the way for better medical treatments.
The Tasmanian study is being led by Launceston based University of Tasmania School of Health Sciences head of the Respiratory Translational Research Group Dr Sukhwinder Singh Sohal.
The study aims to determine how the process of the endothelial-to-mesenchymal transition contributes to pulmonary hypertension and fibrogenesis in IPF.
A previous study by Dr Sohal, published in the European Respiratory Journal found patients with IPF experienced significant scarring of the lungs and arteries leading to pulmonary hypertension.
Dr Sohal said the new study would interrogate the early stages of the disease and the mechanisms that contribute to the scarring.
"Obviously, there is something active there which is leading to this, so what is that mechanism, what is that pathway," he said.
"What we are now keen to discover going forward with this project is what is the mechanism which is contributing to this scarring."
Having received $80,000 from medical research organisation, the Clifford Craig Foundation as part of its 2022 medical research grants program, Dr Sohal said the study had the potential to create new diagnostic tools, therapeutic treatments and improvements in clinical practice.
"With this grant, we are going to look at - in detail - this mechanism, and then try to find the therapeutic targets on how we can inhibit this pathway and also, it will lead to new diagnostic tools for early identification of IPF," he said.
"So there is a therapeutic value here, there is a diagnostic value here and there is also great help to clinical practice because clinicians would know, this is what the tissue looks like, this is what the disease looks like and that will help them in making a better-informed decision for the patient."
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A highly insidious disease, Dr Sohal said the value of the study was in the positive outcomes patients stood to gain if diagnosed and treated early.
"If we are trying to intervene late in the disease, then changing the disease trajectory is difficult and what you're doing at that time is you're just treating symptoms, you're not changing the trajectory of the disease," he said.
"Once diagnosed it's two to three years and the patient is unfortunately gone, so it's a really aggressive disease.
"We have to intervene early on to change it, halfway through is not going to help us and that's what we're trying to do.
"Let's capture it early on, and then fix it there and help the patients from there."
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