Pulmonary exacerbations are frequent and clinically important events in the natural history of CF. The frequency of pulmonary exacerbations is associated with poor clinical outcomes. Preventing pulmonary exacerbations is a high priority in delivering optimal care for people with CF. A number of therapies have been shown to reduce pulmonary exacerbations in clinical trials, and these should be used in a targeted way in individual patients. When pulmonary exacerbations occur, treatment should be commenced promptly, and for each event, people with CF and their multidisciplinary team should consider the optimal treatment regime with new antibiotics combined with augmentation of regular therapies.

Cite as: Elborn JS. Cystic fibrosis: treatment and prevention of pulmonary exacerbations. In: Burgel P-R, Contoli M, López-Campos JL, eds. Acute Exacerbations of Pulmonary Diseases (ERS Monograph). Sheffield, European Respiratory Society, 2017; pp. 167–180 [https://doi.org/10.1183/2312508X.10016616].