Innate immune activation and cystic fibrosis

Abstract

Since inflammation and infection occur so early in infancy in cystic fibrosis, the function of innate immune defence in cystic fibrosis has been questioned by many investigators. This review aims to summarize the findings relating to the physical, humoral and cellular components of innate immune defence in cystic fibrosis, and highlights the roles of neutrophils, macrophages and epithelial cells in these activities. In addition, recently identified links between antioxidant defences and cystic fibrosis transmembrane conductance regulator (CFTR) function, and how these may impact on innate lung defence, are summarized.