What's new in CF airway inflammation: an update.
Neutrophilic airway inflammation is a characteristic feature of cystic fibrosis (CF) lung disease and present in most patients with pulmonary manifestations of the disease. Here we discuss the ongoing controversy whether the CFTR mutation itself causes a pro-inflammatory milieu in the airways or whether inflammation is always secondary to infection. Since the presence of inflammation has been shown to be a risk factor for subsequent lung function decline, noninvasive tests to monitor airway inflammation are urgently needed. Induced sputum is currently being assessed as a clinical and research tool, but unfortunately is only feasible in cooperative children. While nonspecific treatment approaches that decrease infection or improve clearance of airway secretions were found to positively affect airway inflammation, specific anti-inflammatory treatment strategies have been less successful. Since any intervention that decreases inflammation may potentially have a detrimental effect by promoting airway infection, a better understanding of the factors regulating inflammation in the CF lung will form the basis for more targeted treatment strategies in the future.
Ratjen
Felix
F
Hospital for Sick Children, Toronto, Ontario, Canada. felix.ratjen@sickkids.ca
D000818:Animals; D018893:Bronchoalveolar Lavage; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007249:Inflammation / Q000378:metabolism*; D009154:Mutation; D009569:Nitric Oxide / Q000096:biosynthesis*; D011379:Prognosis
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D009569:Nitric Oxide