CAPS is associated with a gain-of-function missense mutation of the NLRP3 gene and include three overlapping clinical phenotypes: familial cold autoinflammatory syndrome (FCAS, mild CAPS phenotype), Muckle-Wells syndrome (MWS, moderate) and neonatal-onset multisystem inflammatory disease (NOMID, severe). Here, NLRP3 is linked to CINCA syndrome.