CAPS is caused by gain-of-function mutations in the NLRP3 gene, which encodes cryopyrin, resulting in increased IL-1 secretion; clinical manifestations of CAPS include cold-induced episodes, urticaria-like rash, sensorineural hearing loss, chronic meningitis, myalgia and arthritis (15). This evidence concerns the gene NLRP3 and cryopyrin-associated periodic syndrome.