ABSTRACT
Although cystic brosis (CF) aects multiple organ systems, the pulmonary manifestations account for the major morbidity and mortality associated with this condition.1 Pulmonary events include, mucus hypersecretion, impaired mucociliary clearance, and pathogenic colonization by Staphylococcus aureus, Pseudomonas aeruginosa, Haemophilus influenza, and Aspergillus fumigatus, among others. is in turn leads to the clinical picture of recurrent infections, persistent chronic neutrophilic inammation causing bronchiectasis and airway destruction, culminating in respiratory failure and premature death.2 It is this interplay between inammation, infection, and immunity that makes CF such a complex lung condition. In this chapter, we describe the major lung defenses including the lung epithelium, inammatory cells, and antimicrobial peptides that are dysregulated in CF. We then describe the interactions between these defenses and the bacterial, viral, and fungal pathogens specic to CF.
