Macrolides and Cystic Fibrosis

Macrolide antibiotics attracted interest as a potential therapy in cystic fibrosis (CF) due to remarkable similarities in pathogenic features with diffuse panbronchiolitis (DPB), as described in the chapter by Azuma and Taniuchi. Azithromycin in particular is widely used for acute pulmonary exacerbations in CF, both for its direct antimicrobial and indirect immunomodulatory properties. A number of randomised clinical trials also support the long-term use of macrolides in CF, predominantly in patients chronically infected with Pseudomonas aeruginosa, which is surprising given that they have little inherent antibacterial activity against this ubiquitous CF pathogen. In this chapter, we recapitulate the pathophysiology of CF, discuss the purported mechanisms of action of macrolides and summarise the key evidence for their use in this multisystem disease.