1 | Positive genetic analysis for NLRP3 gene | 554 | 71 | 7.8 |
2 | Urticarial rash§ # | 479 | 66 | 7.3 |
3 | Response to IL-1 β blockade | 469 | 69 | 6.8 |
4 | Recurrent fever§ | 434 | 55 | 7.9 |
5 | Increase of acute-phase reactants and serum amyloid A during fever episodes # | 331 | 52 | 6.4 |
6 | Hearing loss§ # | 314 | 57 | 5.5 |
7 | Episodes triggered by cold exposure# | 291 | 50 | 5.8 |
8 | Age at onset < 1 yr | 185 | 28 | 6.6 |
9 | Chronic urticaria§ | 177 | 29 | 6.1 |
10 | Chronic meningitis# | 171 | 39 | 4.4 |
11 | Increase of acute-phase reactants and serum amyloid A during fever episodes and between episodes | 166 | 28 | 5.9 |
12 | Chronic disease course | 165 | 29 | 5.7 |
13 | Fever | 157 | 22 | 7.1 |
14 | Positive family history | 151 | 34 | 4.4 |
15 | Eye involvement§ | 104 | 19 | 5.5 |
16 | Neurologic involvement# | 102 | 18 | 5.7 |
17 | Positive NLRP12 genetic test | 94 | 14 | 6.7 |
18 | Conjunctivitis§ | 85 | 20 | 4.3 |
19 | Osteoarthropathy# | 74 | 14 | 5.3 |
20 | Arthralgia# | 70 | 24 | 2.9 |
21 | Cartilage overgrowth# | 66 | 14 | 4.7 |
22 | Age at onset < 1 mo | 66 | 11 | 6.0 |