Researchers Find Drugs That Slow Progression Of Idiopathic Pulmonary Fibrosis

Studies recently published in The New England Journal of Medicine have presented research demonstrating that researchers have found several new treatments for idiopathic pulmonary fibrosis. These new treatments—pirfenidone and nintedanib — did not cure patients or reverse the disease, however these drugs did slow the progression of the fatal lung disease. Researchers hope that by stabilizing lung function, they will eventually be able to prolong patients’ lives.

The drugs, pirfenidone and nintedanib, are produced by InterMune and Boehringer Ingelheim respectively. They were not studied in combination, and each was examined compared to a placebo so it is uncertain which drug would be the most effective treatment for the disease. The research is significant because the lung disease affects more than 80,000 Americans. The disease causes scarring on the lungs, which affects breathing and often leads to death.  Half of all patients die three to five years after the disease begins progressing. The only drugs on the market for the disease today treat symptoms only, such as coughing.

 Dr. Gary M. Hunninghake, the author of the study and a lung specialist at Brigham and Women’s Hospital in Boston, commented on the research. “There’s been nothing,” he said. “That’s about to change. I think for a lot of patients this is going to be pretty exciting.”

Dr. Kevin K. Brown, an author of another one of the studies, said that after the diagnosis, people start to plan for how long they’ll be living. “Patients hope they can get better, but pray they don’t get any worse,” he said.

Though lung transplants are sometimes a viable option for treatment, many of the patients with idiopathic pulmonary fibrosis are too old or sick to have the surgery. The cause of the lung scarring has not yet been discovered.  Although smokers have a higher risk of the disease, non-smokers are also at risk.