Approximately 80 pathogenic mutations in the NLRP3 gene have been identified in patients with systemic autoinflammatory disorders known as cryopyrinopathies or cryopyrin-associated periodic syndromes (CAPS), which include neonatal-onset multisystem inflammatory disease (NOMID), Muckle-Wells syndrome (MWS) and familial cold autoinflammatory syndrome (FCAS) [8]. Here, NLRP3 is linked to Muckle-Wells syndrome.