Idiopathic pulmonary fibrosis is a lung disease that occurs when damaged and scarred tissue builds up and stops the lungs from performing as they should.

A highly destructive and rapid disease with no cure, IPF once diagnosed, can see patients die within two to three years.

However, a recent study published in the European Respiratory Journal led by Launceston based University of Tasmania School of Health Sciences head of the Respiratory Translational Research Group Dr Sukhwinder Singh Sohal has unearthed new data that will improve the treatment of IPF.

"What we have discovered is a mechanism called EndMT, or endothelial to mesenchymal transition, in which endothelial cells or the blood cells that line the blood vessels and arteries of the lungs in response to outside insult contribute to the scarring caused in IPF - and destroy the lung in general," he said.

The study by the Respiratory Translational Research Group is the first time the correlation has been identified, with Dr Sohal explaining the discovery will provide new insights into treating the disease.

"EndMT seems to be a very novel and new therapeutic target for this insidious disease, and this is the first time this kind of work has come out," he said.

"If you know this is what the vasculature looks like, this is what the arteries look like, and this is what the pathology looks like, then the clinical practice can treat these patients better.

"There are immediate benefits here to clinical practice, but it also provides avenues for new therapeutic targets, for a potential cure treatment and better treatments down the track."

IN OTHER NEWS:

Rates of IPF in Tasmania are on a continuous increase, as Tasmania recording the second-highest percentage of smokers in Australia and industries like agriculture and mining playing a role in the development of the disease, but Dr Sohal said under-diagnosis is also a factor.

"My understanding from respiratory physicians is that they do see a lot of patients in their clinical practice later with this nasty disease, but also, the feedback I have received was that a lot of this illness is under-diagnosed as well," he said.

"I think there are high numbers, but I would say it is more under-diagnosed, there could be a mix up with chronic obstructive pulmonary disease or other smoking-related diseases."

With the findings of the study having the potential to significantly impact the treatment of IPF, the work of the RTRG has been acknowledged by the American Thoracic Society awarding Dr Sohal's PhD student Archana Vijay Gaikwad with the ATS international scholarship award for her work on the study.

Dr Sohal said, it was very gratifying that the groups work was recognised with the prestigious international award.

"Archana was the only PhD student in Australia to get this award in IPF research from the ATS," he said.

The study which began in 2018 was conducted in collaboration with physicians from Launceston General Hospital, researchers from The Alfred Hospital, University of British Columbia, Canada, Centre of Research Excellence in Pulmonary Fibrosis.

Funding for the study was provided by the Lung Foundation Australia with $45,000, and the Clifford Craig Foundation who provided $213,000 as part of three funding grants.