Every year, about 2,170 Australians receive a diagnosis that sounds like a death sentence: idiopathic pulmonary fibrosis, a disease that fills the lungs with scar tissue until breathing becomes impossible. Most patients survive only two to five years after diagnosis, and until now, doctors had only two drugs to offer—neither of which could heal the damage.
But researchers at two Australian universities may have cleared a path toward an actual cure.
A team from the University of Technology Sydney and Monash University has discovered a protein called vitronectin that appears to trigger the scarring process in the lungs of people with this disease. The findings, published in the journal Science Advances, could open the door to treatments that actually reverse the damage instead of just slowing it down.
"In pulmonary fibrosis, the normal wound-healing process in the body goes wrong," said Associate Professor Gang Liu from UTS's School of Life Sciences. "Instead of repairing damaged tissue, it starts to produce scar tissue in the lungs."
The researchers found that immune cells called macrophages—whose job is normally to help repair injured tissue—can get reprogrammed to produce scarring instead. These macrophages were studied in a special 3D environment that mimics what happens inside a human lung, which is a big deal because scientists have struggled to study this process accurately before.
"We normally think of vitronectin as a structural protein that maintains the integrity of organs like the lungs," said Associate Professor Katrina Binger from Monash University's Department of Biochemistry and Molecular Biology. "But we found it can also act as a signal. Vitronectin changes how macrophages produce energy, and this drives them to a heightened fibrotic state."
The 3D culture results matched what the researchers saw in animal studies and in tissue samples taken directly from IPF patients, which gives them confidence the discovery is real and relevant to human disease.
Now, the team is working to identify drugs that can block vitronectin and stop the scarring before it starts. "Understanding this mechanism is critical to identifying new therapeutic agents for patients with fibrosis," 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."
For the hundreds of thousands of people worldwide living with this disease, the discovery offers something rare: real hope that better treatments—and maybe even a cure—could be coming.