cryopyrin-associated periodic syndrome
Any of a spectrum of hereditary CIAS1* gene-mediated autoinflammatory disorders characterised by IL-1-mediated recurrent systemic inflammation.
*NLRP3 is the symbol preferred by the HUGO; it encodes cryopyrin, a component of the IL-1 inflammasome. NLRP3 mutations lead to unregulated production of interleukin 1β
Clinical findings CAPS include a range of clinical entities including familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease–NOMID.
References Otolaryngol Head Neck Surg. 2011 Aug;145(2):295-302.