Iowa study shows infection links cystic fibrosis, lung disease

University of Iowa researchers are one step closer to understanding how cystic fibrosis causes lung disease.

The findings, published online last week in the journal Science Translational Medicine, could help improve treatments for lung disease, which causes most of the deaths and disability among people with cystic fibrosis.

Researchers at the UI and University of Missouri appear to answer a long-standing “chicken and egg” question about which comes first in cystic fibrosis lungs - infection or inflammation.

“Using our model, we are beginning to answer that question, and it looks like infection does precede inflammation,” said Dr. David Stoltz, UI assistant professor of internal medicine and lead study author. “Knowing that infection is first suggests that if we can prevent or fight infection, then that might delay or prevent the lung disease in people with CF.”

For example, the finding seems to support early and aggressive treatment of lung infections in children, said Stoltz, who also is assistant director of the Adult Cystic Fibrosis Center at UI Hospitals and Clinics.

The new experimental model researchers used were pigs with a CF-causing gene mutation.

Pigs developed lung disease typical of what is seen in humans, including infection in the lungs, inflammation, and accumulation of mucus in the airways, which is a significant problem for patients with cystic fibrosis.

The study was funded in part by grants from the National Institutes of Health and the Cystic Fibrosis Foundation.