Several single amino acid substitutions in NLRP3 are causative for systemicinflammation observed in a spectrum of autoinflammatory diseases known ascryopyrin-associated periodic syndromes (CAPS; cryopyrin being a synonym ofNLRP3): neonatal onset multisystem inflammatory disease (NOMID), Muckle-Wellssyndrome (MWS), and familial cold autoinflammatory syndrome (FCAS) 23. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.