Pulmonary Inflammation in Cystic Fibrosis

Many of the clinical studies into the mechanisms of pulmonary inflammation in cystic fibrosis have centered on determining the presence (or absence) of candidate mediators that may contribute to the pathogenesis of lung inflammation. Studies of both sputum specimens and BAL fluid obtained from adolescents and adults with cystic fibrosis have established that a plethora of pro-inflammatory mediators are present in airway surface liquid in patients with the disease. 

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Studies of BAL and sputum have provided important insights into the early development of pulmonary inflammation in cystic fibrosis and, in particular, the role of the CFTR in promoting inflammation. However, the data have to be interpreted with caution. On the one hand, the findings could be interpreted as evidence that the airway environment in cystic fibrosis is able to support or induce a neutrophil-dominated inflammatory response in the absence of airway infection. Alternatively, the data could be interpreted as evidence that early pulmonary inflammation is a consequence of an abnormal response to exogenous stimuli within the airways resulting in a potentiation of responses that, by themselves, would not result in inflammation in normal subjects. Obviously, this could include augmented inflammatory responses to airway bacteria that might not induce an overt inflammatory response in the airways of a normal individual, but could include other noninfectious injurious events. 

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D. Neutrophil Involvement in Pulmonary Inflammation in Cystic Fibrosis... 

Kerby GS, Cottin V, Accurso FJ, Hoffmann F, Chan ED, Fadok V, Riches DWH. Hyperosmolarity down-regulates p42 and Akt activation and augments apoptosis in macrophages. Implications for early pulmonary inflammation in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 2001 283 L188. 

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