Summary of corticosteroid cohorts (n=92)
| Primary n=70 | Secondary n=22 | |
| Age at fever onset (years) | 2.1 (0.9, 4.3) | 3.4 (1.5, 6.0) |
| Age <6 months | 12 (18%) | 2 (9%) |
| Male sex | 50 (71%) | 17 (77%) |
| Asian ethnicity | 15 (21%) | 5 (23%) |
| Complete KD criteria | 52 (74%) | 18 (82%) |
| Days of fever prior to 1st IVIG | 6 (5, 8) | 5 (5, 8) |
| Initial IVIG >10 days | 5 (7%) | 1 (5%) |
| Days of fever at RAISE | 6 (5, 8) | 12 (9, 14) |
| Days between IVIG and RAISE | 0 (0, 1) | 6 (3, 11) |
| Reason for RAISE | ||
| Baseline CAA | 50 (71%) | 9 (41%) |
| IVIG resistance | 0 (0%) | 2 (9%) |
| Kawasaki shock syndrome | 8 (11%) | 0 (0%) |
| Macrophage activation syndrome | 2 (3%) | 0 (0%) |
| CAA+treatment resistance | 0 (0%) | 8 (36%) |
| CAA+Kawasaki shock syndrome | 2 (3%) | 3 (14%) |
| Other high-risk features | 8 (11%) | 0 (0%) |
| Total days of steroids | 20 (15, 25) | 18 (15, 21) |
| 2nd IVIG | 41 (59%) | 22 (100%) |
| 3rd IVIG | 2 (3%) | 2 (9%) |
| Additional anti-inflammatory | 10 (14%) | 7 (32%) |
| Baseline coronary status | ||
| Aneurysm at diagnosis (CA z-score >2.5) | 56 (80%) | 11 (50%) |
| Aneurysm at any time | 56 (80%) | 20 (91%) |
| Bilateral CAA | 33 (47%) | 7 (32%) |
| z-Score of largest CAA at diagnosis | 3.3 (2.5, 4.9) | 2.1 (1.2, 3.6) |
| z-Score of largest CAA at diagnosis | ||
| No CAA | 14 (20%) | 11 (50%) |
| z-Score <5 | 39 (56%) | 7 (32%) |
| z-Score ≥5, <10 | 12 (17%) | 0 (0%) |
| z-Score ≥10 | 5 (7%) | 4 (18%) |
| Outcomes | ||
| Initial treatment resistance | 3/64 (5%) | 11 (50%) |
| CAA progression (2-unit increase) | 6 (9%) | 12 (55%) |
| CAA zMax over follow-up | 3.4 (2.5–5.2) | 3.6 (3.2–11.2) |
| CAA resolution (of those with baseline CAA) | 44/54 (81%) | 14/22 (63%) |
Values are number (%) or median (IQR).
CAA, coronary artery aneurysm; IVIG, intravenous immunoglobulin; KD, Kawasaki disease.