Table 1.
Demographics
| Diagnosis | % Female | Age (median, min–max years old) | Ethnicity |
|---|---|---|---|
| CANDLEa (n = 11) | 45.5 | 6.1 (1.7–19.5) | 4 white |
| 4 Hispanic | |||
| SAVIb (n = 7) | 42.9 | 16.9 (0.7–24.0) | 7 white |
| NOMIDc (n = 18) | 38.9 | 8.5 (0.8–42.2) | 12 white |
| 3 Hispanic | |||
| HCd (n = 26) | 65.4 | 25.9 (0.6–53.6) | 15 white |
| 8 Hispanic |
Other ethnicities: 2 black, 1 unknown. All with clinically active disease before Janus kinase inhibitor therapy, 7 with elevated C-reactive protein (CRP), 4 with rash.
All clinically active, 6 with elevated CRP, 1 with elevated erythrocyte sedimentation rate.
Other ethnicities: 1 black, 1 Asian, 1 multiple race. All clinically active; 16 before IL-1 blockade with elevated CRP, 2 on early IL-1 blockade with persistent cerebrospinal fluid pleocytosis.
Other ethnicities: 1 black, 1 multiple race, 1 unknown.
CANDLE, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature; HC, healthy control; NOMID, neonatal-onset multisystem inflammatory disease; SAVI, STING-associated vasculopathy with onset in infancy; STING, stimulator of IFN genes.