New Evidence That Inflammation For Cystic Fibrosis May Be Present Before Patients Show Respiratory Manifestations.
A new study also provides additional evidence that the persistent and excessive inflammation in the lungs of CF patients involves a failure of the mechanisms that control the inflammatory response.
Cystic Fibrosis (CF) is one of the most frequent lethal chromosomal hereditary disorders in Caucasian populations and occurs in approximately one in every 3,500 births. Caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, a defective cAMP-dependent chloride ion conductance occurs. In patients with CF, lung disease is the major cause of sickness and death with the progressive decline of pulmonary function attributed to a vicious cycle of airway infection and inflammation. There is now evidence that inflammation plays a pivotal role and may be present very early in life, even before the onset of respiratory manifestations.
These results are consistent with a dysregulated cytokine production by lung and blood neutrophils in CF. They provide support to the hypothesis that not only the CF genotype but also the local environment may modify the functional properties of the neutrophils.
This study is the first report comparing airway and blood neutrophils from children with CF in terms of pro- and anti-inflammatory cytokine production and their respective responsiveness to glucocorticoids. Comparison of airway and blood neutrophils from the same CF patients showed distinct profiles of cytokine production spontaneously and in the presence of LPS, as well as differences in the response to dexamethasone, supporting the view that the local environment may modify the functional properties of the cells. In addition, comparisons of cytokine production by circulating neutrophils from children with CF and controls and by airway neutrophils from children with CF or dyskinetic cilia syndrome revealed significant differences, suggesting that genetic components may also participate in the altered neutrophil function in CF.
The findings provide additional evidence that the persistent and excessive inflammation in the lungs of CF patients involves a failure of the mechanisms that control the inflammatory response. An altered regulation of cytokine production by neutrophils is certainly an important factor that promotes continued inflammation and injury. Development of therapeutic interventions with specific cytokine inhibitors, anti-inflammatory cytokines, as well as anti-inflammatory drugs, which could target airway neutrophils, appears essential to control CF inflammation.
Donna Krupa | American Physiological Society
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