Adult cystic fibrosis

Michael P. Boyle, MD

JAMA. 2007;298:1787-1793.

Cystic fibrosis is a multisystem disease characterized primarily by chronic pulmonary infection and bronchiectasis, pancreatic exocrine impairment, and elevated sweat chloride. In the last 4 decades, new treatment strategies and aggressive nutritional management have resulted in a significant increase in expected survival, with median predicted survival in cystic fibrosis now to older than 35 years. This increase in predicted survival has also been aided by a greater appreciation of the potential variability in the presentation and severity of cystic fibrosis, resulting in identification of a growing number of mild cases. As it is estimated that within the next decade more than half of all individuals with cystic fibrosis will be aged 18 years or older, adult medicine caregivers are increasingly likely to encounter patients with cystic fibrosis and be exposed to their unique medical management.