University of Newcastle’s Professor Darryl Knight and fellow (UON) researchers, as part of the nation-wide Pulmonary Fibrosis consortium have been funded by the National Health and Medical Research Council to identify the pathways that lead to the development of Idiopathic Pulmonary Fibrosis (IPF), a silent killer of unknown cause and dismal survival rate that affects five million people worldwide.
IPF is a lung disease characterised by relentless scarring of the lung interstitium. This is the tissue layer between the air sacs and the blood vessels where oxygen transfer occurs. The air sacs of the lung become replaced by fibrotic tissue (akin to a scar), produced by cells called fibroblasts. This causes the lungs to lose their ability to transfer oxygen into the bloodstream. Once diagnosed, the median survival rate is two to five years – this prognosis is worse than many cancers.
Patients with IPF are typically diagnosed in their 50s and 60s but there are currently no accurate figures for the number of Australians affected by the disease. Current estimates from the Australian Bureau of Statistics in 2012 suggest an incidence of five cases every 100,000 people per year, although that number will grow with an aging population. The first two drugs to treat IPF were approved in the USA in 2015 and work to slow the rate of disease progression. However, the treatments are not approved for use in Australia yet.
The study will use whole lungs transplanted from patients with IPF and state-of-the-art protein detection and imaging to model progressive changes in disease severity within an individual lung. The project is a collaboration between investigators from Newcastle, Sydney, Melbourne and Perth, along with international collaborators from Canada and Belgium.
The development of effective treatments for IPF has been hampered by the complexity and diversity of the disease and this has been compounded by the lack of relevant lung tissue from patients with IPF. This study hopes by removing this barrier, researchers will be able to comprehensively analyse connective tissue proteins in areas of normal, damaged and fibrotic lung, allowing researchers to identify the proteins responsible for the disease and create a way IPF can be therapeutically targeted.