New drug could offer hope for people with incurable condition
A drug that is currently used for cancer could be the key in treating a disease that has no cure, and a life expectancy of just three to five years
A lesser-known medication could potentially offer some mch needed hope for those suffering from an incurable and fatal condition, which often results in death within three years of diagnosis. Idiopathic Pulmonary Fibrosis (IPF) is a progressive and terminal disease that causes lung scarring and increasing breathing difficulties.
It's a severe and life-limiting illness affecting approximately 70,000 people annually in the UK, according to Action for Pulmonary Fibrosis. Amy Price, mother of Katie Price, was diagnosed with IPF in 2017 and continues to battle the disease.
Sadly, there is no known cure and while existing treatments can provide some relief, they only slow down its progression. Although this is predominantly a disease associated with aging and is seldom seen before the age of 50, experts believe these findings could also offer significant hope for other similar age-related diseases.
A drug currently used for cancer treatment could be the answer. According to recent research by Tulane University, scientists have discovered a potential new method to treat IPF.
A study from Tulane University, published in the Journal of Clinical Investigation, suggests an existing cancer medication might help the immune system clear out cells causing lung scarring, potentially enhancing lung function for those with Idiopathic Pulmonary Fibrosis (IPF).
The research points out that while fibroblasts, specialised cells in healthy lungs, contribute to tissue repair, in IPF patients, some fibroblasts and adjacent epithelial cells become "senescent"—cells that stop dividing but don't die as expected. These malfunctioning cells pile up, leading to the formation of stiff, scarred lung tissue.
The team found that these senescent cells accumulate because the immune system struggles to remove them effectively, a problem linked to a protein known as CTLA4, which suppresses immune response like an emergency brake. To counter this, the researchers administered the cancer drug ipilimumab to mice, inhibiting CTLA4.
This action released the immune system's "brake," allowing T cells, crucial for immunity, to eliminate the senescent fibroblast cells. Consequently, the treated mice exhibited better lung healing and less scarring.
"The CTLA4 protein typically functions to inhibit excessive inflammation by restraining overactive T cells," explained senior author Dr. Victor Thannickal, professor and Harry B. Greenberg Chair of Medicine at Tulane University's John W.
Deming Department of Medicine. "An excess of this 'blocker protein' may lead to the loss of the 'good' inflammation necessary for eliminating senescent cells. Essentially, we're blocking the blocker."
The team zeroed in on CTLA4 as a potential therapeutic target after discovering elevated levels of it on T cells in the most scarred areas of lung tissue from humans and mice with IPF. Mice treated with ipilimumab exhibited improved lung healing and quicker recovery than those not administered the drug.
"This paves the way for an entirely new approach to treating IPF," stated lead author Santu Yadav, PhD, assistant professor of medicine at the Tulane University School of Medicine. "Rather than employing drugs to destroy senescent cells, we are reactivating our own immune system to clear them out."
Further research is required to determine the efficacy of drugs targeting CTLA4 or other "checkpoint proteins" in rejuvenating the immune system. A significant hurdle is identifying a safe dosage that allows the immune system to purge ageing cells without inciting excessive inflammation.
"If it works in IPF, this immune rejuvenating approach to treatment may be effective in other diseases such as Alzheimer's or cardiovascular diseases in which senescent cells are known to accumulate.
"Can the right drug activate T cells in a way that clears senescent cells without causing collateral damage? If so, we may be closer to combating many aging related diseases and perhaps even aging itself."
What are the signs and symptoms of pulmonary fibrosis?
Common symptoms can include:
Unexplained weight loss
Clubbing of fingers and toes (swollen and rounded finger and toe tips)
Shortness of breath (breathlessness)
Dry cough
Tiredness (fatigue)
What are the risk factors of pulmonary fibrosis?
Risk factors for pulmonary fibrosis can include:
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