Current Approaches to the Management of Infection in Cystic Fibrosis

Abstract

Bacterial infection is the primary initiator and driver of inflammation and lung injury in the airways of people with cystic fibrosis. Infection begins early in life and over time becomes chronic in many patients. Pseudomonas aeruginosa is the dominant pathogen in chronic infection and is associated with an accelerated decline in lung function, more frequent exacerbations, and reduced survival compared to people with no Gram-negative infection. Antibiotic eradication therapy is effective in delaying chronic infection with P. aeruginosa and can be employed repeatedly to new infections. When chronic infection develops, long-term inhaled antibiotics and azithromycin therapies reduce exacerbations, improve QoL, and may impact survival. Pulmonary exacerbations driven by infection and inflammation are associated with decline in lung function and reduced survival. Optimal therapy for these requires multiple antibiotics and close assessment of response. A number of other bacteria such as Staphylococcus aureus, Stenotrophomonas maltophilia, Achromobacter spp. and nontuberculous mycobacteria are an increasing problem in CF infection and bring new challenges to therapy.