ELANE and cystic fibrosis: It is characterized by an excessive neutrophilic infiltration of the airways, abundant protease (neutrophil elastase (Le Gars et al., 2013) and bacterial elastase (Saint-Criq et al., 2018)) and elevated levels of IL-1 and IL-8 in the CF airways lumen (Konstan et al., 1994; Balough et al., 1995; Armstrong et al., 1997; Montgomery et al., 2018).