Table 1 Cryopyrin associated periodic syndrome (CAPS): main clinical features
From: Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome: a review
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 |