Gain-of-function mutations in the NLRP3 gene are well known to cause cryopyrin-associated periodic syndromes(CAPSs) including familial cold-induced autoinflammatory syndrome (FCAS, OMIM #120100), Muckle Wells syndrome (MWS, OMIM #191900) and neonatal-onset multisystem inflammatory disease (NOMID, OMIM #607115) (3, 8). This evidence concerns the gene NLRP3 and CINCA syndrome.