Table 1.

Cryopyrin associated periodic syndrome (CAPS): main clinical features

Familial cold autoinflammatory syndrome (FCAS)	Muckle–Wells syndrome (MWS)	NOMID/CINCA
• Autosomal dominant	• Autosomal dominant	• Autosomal dominant • Sporadic
• Cold-induced: – Fever – Rash – Conjunctivitis – Arthralgia	• Fever • Urticarial rash • Conjunctivitis • Arthralgia • Sensorineural deafness • AA amyloidosis (in 25% of patients) leading to renal failure	• Fever • Urticarial rash • Conjunctivitis • Visual and intellectual damage • Sensorineural deafness • Progressive chronic meningitis • Destructive arthritis • AA amyloidosis leading to renal failure