Table 1.

Clinical characteristics of the affected patient and the cryopyrin associated periodic syndromes

Disease	Age at Disease Onset (years)	Fevers	Flare Duration	Dermatologic	Neurologic	Ocular Features	Musculoskeletal	AA amyloidosis
FCAS	<1	Yes	<24 hours	Cold-induced neutrophilic urticaria-like	No	Conjuctivitis	Arthralgias/myalgia	<10%
MWS	<20	Yes	1–3 days	Neutrophilic urticaria-like	Sensorineural hearing loss	Conjuctivitis	Arthralgias/arthritis	~30%
NOMID/CINCA	<1	Yes	Continuous	Neutrophilic urticaria-like	Aseptic meningitis, sensorineural hearing loss	Papilledema uveitis	Arthralgias/arthritisepi physeal bone formation	Unknown
Patient	Possible symptoms in early childhood, severe symptoms in late 40’s	Stress- induced	Initially <48 hours, increasing to continuous	Stress-induced neutrophilic urticaria-like	No	Conjuctivitis	Arthralgia/Myalgia	No