CAPS encompass a spectrum of three overlapping autosomal dominant (AD) autoinflammatory disorders caused by heterozygous gain-of-functions mutations in NLRP3 gene: familial cold autoinflammatory syndrome (FCAS, OMIM 120100), Muckle-Wells syndrome (MWS, OMIM 191900) and chronic infantile neurologic, cutaneous and articular syndrome/neonatal-onset multisystem inflammatory disease (CINCA/NOMID, OMIM 607115) (Table 1). Here, NLRP3 is linked to Muckle-Wells syndrome.