Abstract

The pathobiology of CF lung disease has yet to be fully elucidated and several theories have been proposed in recent years relating the basic biochemical defect caused by CFTR dysfunction to the CF phenotype. Within the last decade accumulating evidence has suggested that the pulmonary inflammation observed in CF patients may be intrinsically related to the CFTR mutation, resulting in an augmented inflammatory response. This review discusses our changing understanding of the link between CFTR dysfunction and CF lung disease, focusing especially on the role of hyperinflammatory processes. A better understanding of these mechanisms will lead to better treatments for CF and other suppurative lung diseases.

Keywords: Cystic fibrosis, inflammation, CFTR, Pseudomonas aeruginosa