A promising breakthrough in the treatment of idiopathic pulmonary fibrosis (IPF) - a fatal and currently incurable lung condition - has emerged from a study at Tulane University. The disease, which affects around 70,000 people in the UK annually, leads to lung scarring and progressive breathing difficulties. Patients diagnosed with IPF typically succumb to the disease within three years.
The latest research suggests that an FDA-approved cancer drug, ipilimumab, may hold the key to addressing the root cause of IPF. The drug has shown potential in helping the immune system clear out damaged cells that contribute to lung scarring, thereby potentially restoring lung function in patients.
In healthy lungs, specialized cells called fibroblasts aid in repairing lung tissue. However, in individuals with IPF, these cells and nearby epithelial cells malfunction, leading to the accumulation of senescent cells that do not undergo normal cellular processes. The study revealed that aged cells tend to accumulate when the immune system is unable to effectively clear them out due to a protein called CTLA4, which acts as an inhibitor of the immune system.
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In mouse models, ipilimumab was used to block CTLA4, allowing the immune system to remove aging fibroblast cells, resulting in improved lung healing and reduced scarring.
The lead author of the study, Santu Yadav, emphasized the potential of reactivating the immune system to clear out senescent cells rather than resorting to drugs to eliminate these cells. While further research is necessary to determine the efficacy and safety of drugs targeting CTLA4 and similar checkpoint proteins, the findings offer new avenues for treating not only IPF but also other aging-related diseases.
The study’s senior author, Dr. Victor Thannickal, highlighted the possibility of this approach being effective in addressing conditions such as Alzheimer’s and cardiovascular diseases, where the accumulation of senescent cells is known to occur. The challenge lies in identifying a safe dosage that allows the immune system to clear out aging cells without triggering excessive inflammation.