Cystic Fibrosis displays some similarities with systemic autoinflammatory diseases (SAIDs), with CFTR dysfunction and increased ENaC-mediated sodium influx, driving NLRP3-dependent inflammation and an enhanced proinflammatory signature, as evidenced by increased levels of IL-18, IL-1β, caspase-1 activity and ASC speck release in monocytes and epithelia as well as serum with CF-associated mutations [3, 27, 28]. This evidence concerns the gene IL1B and cystic fibrosis.