Table 1
FCAS (n=10) | MWS (n=37) | NOMID (n=6) | Other (n-5) | |
|---|---|---|---|---|
Age <4 years | 0 | 2 | 1 | 1 |
Age 4- <18 years | 2 | 6 | 3 | 1 |
Age ≥18 years | 8 | 29 | 3 | 3 |
Male, n (%) | 3 (30.0) | 20 (54.1) | 3 (50.0) | 2 (40.0) |
NLRP3 mutation, n (%) | 10 (100.0) | 35 (94.6) | 5 (83.3) | 2 (86.7) |
Disease duration (months, mean) | 550 | 370 | 172 | 64 |
Rash/arthralgia/headache/conjunctivitis (%) | 100/100/50/80 | 97.3/97.3/78.4/75.7 | 100/100/93.4/66.6 | 80/100/20/40 |
History of anaemia (%) | 10 | 8.1 | 50 | 0 |
Prior SAA value (mean, mg/L) | 37 | 26 | 47 | 3 |
Prior IL-1 inhibitor treatment, n (%) | 0 | 14 (37.8) | 1 (16.1) | 2 (40) |