Table 3.
Q703K+ (n: 6) | Q703K- (n: 20) | P-value | |
---|---|---|---|
Median age at onset (years.months) | 0.7 | 0.1 | |
Median age at diagnosis | 6.9 | 4.1 | |
Sex ratio (M/F) | 2/1 | 2/1 | 1 |
Positive familial history* | 1/4 | 9/16 | 0.5820 |
Recurrent Fever | 3 | 4 | 0.2929 |
Pharyngitis | 2 | 0 | 0.0462 |
Abdominal pain | 1 | 2 | 1 |
Neurologic symptoms | 2 | 7 | 1 |
Adenopathy | 1 | 4 | 1 |
Urticaria | 4 | 17 | 0.5581 |
Headache | 3 | 2 | 0.0624 |
Myalgia | 1 | 2 | 1 |
Arthralgia | 4 | 12 | 1 |
Rash | 1 | 1 | 0.4154 |
Diarrhea | 1 | 0 | 0.2308 |
Conjunctivitis | 2 | 5 | 1 |
Oral aphthosis | 1 | 0 | 0.2308 |
Hearing loss | 2 | 5 | 1 |
Disease Complications** | 1 | 6 | 1 |
FCAS | 3 | 4 | 0.2929 |
MWS | 3 | 12 | 1 |
CINCA/NOMID | 0 | 4 | 0.5425 |
familial History is available in only 4 Q703K-positive and 16 negative CAPS patients.
disease complications include ocular complications, renal insufficiency, skeletal and joint deformities, growth and neurodevelopmental delay.