NLRP3 is an inflammasome sensor, whose activation leads to a variety of autoinflammatory diseases (NLRP3-AIDs), including three well-documented periodic fever syndromes: familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and chronic infantile neurologic cutaneous and articular syndrome, also known as neonatal-onset multisystem inflammatory disease (CINCA/NOMID) (10–15). Here, NLRP3 is linked to Muckle-Wells syndrome.