Table 1 Clinical characteristics of CAPS (adapted from [10,11])
| Â | FCAS | MWS | CINCA/NOMID |
|---|---|---|---|
Severity | mild | moderate | severe |
Age at onset |  < 6 months-adulthood | Early childhood-adulthood | Perinatal |
Family history | Often positive | Often positive | Often negative |
Triggers | Generalized cold/pneumovax | Stress/excercise/infection/pneumovax | Stress/excercise/infection/pneumovax |
Cold trigger | Yes | Possible | Rare |
Fever | Yes | Yes | Yes |
Fatigue | Rare | Yes | Yes |
Cutaneous manifestations | Urticaria-like rash | Urticaria-like rash | Urticaria-like rash |
Ocular manifestations | Conjunctivitis/keratitis | Conjunctivitis/ketatitis/uveitis | Conjunctivitis/ketatitis/uveitispapilledema |
Muskulosceletal manifestations | Myalgia/arthralgia | Myalgia/arthralgia/arthritis | Myalgia/arthralgia/arthritis/distal femur overgrowth |
Central nervous system manifestations | Headache | Headache/intermittent aseptic meningitis | Headache/sterile meingtitis/elevated intracranial pressure/brain atrophy |
Hearing loss | No | Yes | Yes |
Amyloidosis | Rare | Yes | Yes |