The term CAPS, or NLRP3-associated AID (NLRP3-AID) [64] describes a continuum of clinical entities of increasing severity, whose pathogenetic mechanism is a gain-of-function mutation of NLRP3 gene, also known as cold-induced autoinflammatory syndrome 1 (CIAS1) gene, on chromosome 1q44, encoding for cryopyrin [65]. This evidence concerns the gene AICDA and cryopyrin-associated periodic syndrome.