The pathologic hallmark of CAPS is the presence of activating mutations in the NLRP3 inflammasome, which result in induction of caspase 1 and deregulated release of IL-1β (Agostini et al., 2004; Goldbach-Mansky et al., 2006; Gattorno et al., 2007). This evidence concerns the gene NLRP3 and cryopyrin-associated periodic syndrome.