News release

From: University of Technology Sydney (UTS)

Pulmonary fibrosis is a deadly disease where scar tissue grows in the lungs, making breathing more difficult. Approximately 2,170 Australians are diagnosed annually with idiopathic pulmonary fibrosis (IPF), a form of the disease with no known cause and very few treatments.

“In pulmonary fibrosis, the normal wound healing process in the body goes wrong. Instead of repairing damaged tissue, it starts to produce scar tissue in the lungs,” said Associate Professor Gang Liu from the University of Technology Sydney (UTS) School of Life Sciences.

“People with idiopathic pulmonary fibrosis have a very short survival time, usually only two to five years from diagnosis. Only two drugs are approved to treat it, and neither of them can reverse the scarring and cure the disease.”

Associate Professor Liu is the co-first author on new study published in Science Advances that has identified a protein in the lungs – vitronectin – that can switch on the scarring that causes pulmonary fibrosis.

“There’s an important type of immune cell – known as a macrophage – that helps repair tissue after an injury,” he said

“We discovered that these macrophages sometimes get reprogrammed to produce scarring rather than normal wound healing.”

Senior author on the paper, Associate Professor Katrina Binger from the Department of Biochemistry and Molecular Biology at Monash University, created a 3D tissue culture system that mimics the fibrotic environment and identified vitronectin as being critical in switching the macrophages to cause scarring rather than healing.

“We normally think of vitronectin as a structural protein that maintains the integrity of organs like the lungs. But we found it also can also act as a signal,” she said.

“Vitronectin changes how macrophages produce energy, and this drives them to have a heightened fibrotic state. This is a completely new mechanism to understand how fibrosis happens that was only possible by studying these cells in more natural, 3D environments.”

“Importantly, what we saw in our 3D cultures was perfectly mirrored by animal models run by Associate Professor Liu’s team and in tissue samples from patients with IPF.”

The next step for the team is to identify new treatments that can use the vitronectin-macrophage pathway to treat IPF.

“Understanding this mechanism is critical to identifying new therapeutic agents for fibrosis patients,” Associate Professor Liu said.

“Now we can work to identify new drugs that can most effectively inhibit vitronectin so we can translate this research into clinical practice and help find a new cure for this debilitating disease.”