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Treatment of pulmonary exacerbations in cystic fibrosis

Daniel J Smith, David W Reid and Scott C Bell

People with the genetic condition cystic fibrosis suffer repeated respiratory tract infections from an early age, which are often superseded by chronic airway infection, with resultant progressive loss of lung function and premature death from respiratory failure. Acute pulmonary exacerbations contribute to diminished quality of life, reduced lung function, morbidity and mortality in cystic fibrosis, and remain a major obstacle to improving long term outcomes. This article discusses current diagnostic and therapeutic strategies employed in the treatment of pulmonary exacerbations, with a focus on critical appraisal of the evidence based on which strategies are founded. Areas of uncertainty are highlighted and current and potential future research directions are discussed.