Nintedanib, a drug approved for the treatment of idiopathic pulmonary fibrosis, slows the decline in lung function in patients with a wide range of lung scars.
The findings, published in New England Journal of Medicine, suggest that many more people may benefit from nintedanib than are currently approved for treatment. They also suggest that dozens of different forms of fibrotic interstitial lung disease may share similar scarring mechanisms despite different causes and forms.
We have spent decades dividing interstitial lung disease into more than 200 different categories because we believed that their causes and patterns have important implications for treatment and prognosis.
Our findings suggest that progressive fibrotic interstitial lung disease can share scarring mechanisms and respond to the same treatments. This can have profound implications for ongoing treatment, clinical trials and basic research in progressive, lung scars. "
Dr. Kevin K. Brown, senior author of studies and professor of medicine, National Jewish Health
Nintedanib was approved in 2014 for the treatment of idiopathic pulmonary fibrosis, lethal progressive interstitial lung disease (ILD) for an unknown reason. It is the most common form of ILD, accounting for about 20 percent of cases. Other fibrosis ILDs include, but are not limited to, asbestosis, lung scarring caused by autoimmune diseases, and chronic hypersensitive pneumonitis.
National Jewish Health researchers across the country have recruited patients with various progressive lung diseases other than IPF to determine if nintedanib can slow the progression of their disease. Six hundred and sixty-three patients in the INIBIBLD trial were randomized to 52 weeks of treatment with either naltanib or placebo.
Lung function, measured as forced vital capacity (FVC), decreased by 57 percent less in patients taking nintedanib than in those receiving placebo, a response similar to that previously observed in patients with IPF.
The researchers thought that a subset of patients with the usual pattern of interstitial lung fibrosis pneumonia could show a greater response to the drug. However, all patients, regardless of the pattern of lung fibrosis, showed a similar response.
While nintedanib slowed the decline in lung function, it had no significant effect on quality of life. Sixty-seven percent of patients also suffered from diarrhea while taking "nonsanib", compared to 24 percent taking placebo.
"Because nintedanib had a similar effect on the full spectrum of progressive, lung scars, we believe these diseases have a similar scarring process, which may be subject to similar interventions," Dr Brown said. "These findings give us further insights into pulmonary fibrosis and will help speed up our search for new, more effective therapies in the future."