Novel classification criteria of CAPS based on the genotype and/or typical clinical manifestation have been proposed by Eurofever/PRINTO (Paediatric Rheumatology International Trials Organization) (62) FCAS, MWS, and CINCA/NOMID are autosomal dominant diseases caused by a mutation of the NLRP3 gene, which causes the overproduction of IL-1β by cryopyrin inflammasomes (63). This evidence concerns the gene NLRP3 and CINCA syndrome.