Macrolides in Cystic Fibrosis

Cystic fibrosis (CF) results in chronic pulmonary disease in the majority of patients resulting from the production of viscous secretions and impairment of mucociliary clearance due to alterations in airway surface liquid. Chronic infection results, and the combination of tissue damage by pathogenic organisms and a robust host inflammatory response leads to the development of bronchiectasis and progressive lung disease. Macrolide antibiotics have demonstrated efficacy in diffuse panbronchiolitis, a disease that shares many similarities to CF, and this has generated interest in the use of these agents in patients who have CF. The authors review the data that demonstrate clinical benefits of long-term azithromycin administration in patients who have CF and discuss potential host- and pathogen-related explanations for the positive therapeutic effect.