[Inflammation and cystic fibrosis].
Lung inflammation plays a pivotal role in the pathogenesis of airway disease in cystic fibrosis (CF). Inflammation occurs very early and can be identified in very young infants. Inflammatory response is also more intense in CF than in non-CF airway inflammatory diseases. Among the different cell types involved in the airway inflammation, neutrophils are recognized to play a central role by releasing proinflammatory mediators, such as reactive oxygen species and proteolytic enzymes. Whether inflammation arises independently from infection remains debated. While infection was demonstrated to clearly amplify the inflammation, several studies argue for the possibility of an intrinsic inflammation. Finally, there is also evidence that the severity of pulmonary disease is linked to other genetic factors outside the CFTR gene locus, involved in host defence and inflammation.
Delacourt
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Centre hospitalier intercommunal, 40, avenue de Verdun, 94000 Créteil, France. christophe.delacourt@chicreteil.fr
D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics / Q000276:immunology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D006801:Humans; D007239:Infections / Q000150:complications; D007249:Inflammation; D008168:Lung / Q000276:immunology* / Q000473:pathology*; D012720:Severity of Illness Index
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator