Table 1

Summary of corticosteroid cohorts (n=92)

Primary n=70Secondary n=22
Age at fever onset (years)2.1 (0.9, 4.3)3.4 (1.5, 6.0)
Age <6 months12 (18%)2 (9%)
Male sex50 (71%)17 (77%)
Asian ethnicity15 (21%)5 (23%)
Complete KD criteria52 (74%)18 (82%)
Days of fever prior to 1st IVIG6 (5, 8)5 (5, 8)
Initial IVIG >10 days5 (7%)1 (5%)
Days of fever at RAISE6 (5, 8)12 (9, 14)
Days between IVIG and RAISE0 (0, 1)6 (3, 11)
Reason for RAISE
 Baseline CAA50 (71%)9 (41%)
 IVIG resistance0 (0%)2 (9%)
 Kawasaki shock syndrome8 (11%)0 (0%)
 Macrophage activation syndrome2 (3%)0 (0%)
 CAA+treatment resistance0 (0%)8 (36%)
 CAA+Kawasaki shock syndrome2 (3%)3 (14%)
 Other high-risk features8 (11%)0 (0%)
Total days of steroids20 (15, 25)18 (15, 21)
2nd IVIG41 (59%)22 (100%)
3rd IVIG2 (3%)2 (9%)
Additional anti-inflammatory10 (14%)7 (32%)
Baseline coronary status
Aneurysm at diagnosis (CA z-score >2.5)56 (80%)11 (50%)
Aneurysm at any time56 (80%)20 (91%)
Bilateral CAA33 (47%)7 (32%)
z-Score of largest CAA at diagnosis3.3 (2.5, 4.9)2.1 (1.2, 3.6)
z-Score of largest CAA at diagnosis
 No CAA14 (20%)11 (50%)
 z-Score <539 (56%)7 (32%)
 z-Score ≥5, <1012 (17%)0 (0%)
 z-Score ≥105 (7%)4 (18%)
Outcomes
Initial treatment resistance3/64 (5%)11 (50%)
CAA progression (2-unit increase)6 (9%)12 (55%)
CAA zMax over follow-up3.4 (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.