The autoinflammatory syndromes associated with gain-of-function mutations of NLRP3 leads to abnormal NLRP3 inflammasome activation causing cryopyrin-associated periodic syndromes (CAPS), a group of rare, inherited, auto-inflammatory diseases, such as Muckle–Wells syndrome, familial cold urticaria, and neonatal onset multisystem inflammatory disease (NO-MID) (12, 13). Here, NLRP3 is linked to CINCA syndrome.