For the first time, drugs have been found that can slow the progression of a fatal lung disease, idiopathic pulmonary fibrosis (IPF), which affects about 100,000 Americans.
IPF causes scar tissue to build up in the lungs, impairing the ability to breath. Eventually the entire lung fills with scar tissue, and the loss of lung function is relentless.
In clinical trials, the two new drugs—nintedanib and pirfenidone—were each able to slow the loss of lung function in some patients. Results were published May 18 in the New England Journal of Medicine.
“This is an amazing day for idiopathic pulmonary fibrosis,” says pulmonologist David Lederer, MD, associate professor of medicine at P&S, who was on the steering committee of the pirfenidone trial. “Before this week we had no drugs that can treat IPF, and now we have two.”
Before this week we had no drugs that can treat IPF, and now we have two.But Dr. Lederer cautioned that neither drug is a cure for IPF, both have side effects, and they have not been approved by the FDA, which will review the data later this year. (Read Dr. Lederer’s blog post, Pulmonary Fibrosis: Clearing the Air, for more details about the drugs and trials).
“These drugs are promising, but we still need to find out what causes the disease, which is unknown, and develop new treatments that can halt the scarring and maybe even reverse the scar tissue,” Dr. Lederer says.
Recent research by Dr. Lederer suggests that sleep apnea may cause IPF, and he hopes to start a trial to test whether CPAP (continuous positive airway pressure) can slow the rate of lung scarring and loss of lung function in patients.