Inhaled hypertonic saline for cystic fibrosis: Reviewing the potential evidence for modulation of neutrophil signalling and function

Figure 6 Potential effects of active neutrophil elastase in cystic fibrosis. Excessive NE activity can lead to proteolysis causing protease-antiprotease imbalance by cleaving protease inhibitors. Cleavage of matrix proteins and surface receptors leads to tissue damage and prolonged immune response, respectively. NE can further activate pro-inflammatory molecules (MMPs and ADAM-17) and receptors. These cumulative effects exacerbate tissue destruction and hyper-inflammation. NE: Neutrophil elastase; CF: Cystic fibrosis; MMPs: Matrix metalloproteinases.