Cryopyrinopathies (cryopyrin-associated periodic syndrome, CAPS), also referred to as NLRP3 mutation-associated autoinflammatory diseases (NLRP3-AID), have historically been described as three dominantly inherited clinical entities: Familial cold urticaria (FCAS), Muckle-Wells syndrome (MWS), and the Chronic Infantile Neurological Cutaneous and Articular syndrome (CINCA). Patients with MWS have been described with urticaria, chronic inflammation, recurrent fever, sensorineural deafness, ocular inflammation, headache, arthritis and may be complicated by AA amyloidosis [1,2].
