CAPS includes a spectrum of apparently distinct inflammatory disorders of increasing severity: Familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and chronic infantile neurological, cutaneous, articular (CINCA) syndrome/neonatal-onset multisystem inflammatory disease (NOMID), all caused by mutations in NLRP3, the gene encoding cryopyrin, a component of the IL-1 inflammasome that regulates the production of IL-1β [25]. Here, NLRP3 is linked to familial cold autoinflammatory syndrome.