CF lung disease is characterized by a chronic and non-resolving activation
of the innate immune system with excessive release of chemokines/cytokines including
IL-8 and persistent infiltration of immune cells, mainly neutrophils, into the airways.
Chronic infection and impaired immune response eventually lead to pulmonary
damage characterized by bronchiectasis, emphysema, and lung fibrosis. As a complete
knowledge of the pathways responsible for the exaggerated inflammatory response in
CF lung disease is lacking, understanding these pathways could reveal new therapeutic
targets, and lead to novel treatments. Therefore, there is a strong rationale for the
identification of mechanisms and pathways underlying the exaggerated inflammatory
response in CF lung disease. This article reviews the role of inflammation in the
pathogenesis of CF lung disease, with a focus on the dysregulated signaling involved in the overexpression
of chemokine IL-8 and excessive recruitment of neutrophils in CF airways. The findings suggest
that targeting the exaggerated IL-8/IL-8 receptor (mainly CXCR2) signaling pathway in immune cells
(especially neutrophils) may represent a potential therapeutic strategy for CF lung disease.
Keywords: Cystic fibrosis, lung inflammation, CFTR, chemokine signaling, IL-8, CXCR2, NF-κB.
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