42 - PRIMARY ADJUNCTIVE CORTICOSTEROIDS IN THE ACUTE MANAGEMENT OF HIGH-RISK KAWASAKI DISEASE

BACKGROUND. Corticosteroids added to initial standard therapy have been shown to reduce CA abnormalities in high-risk KD patients in Japan (RAISE study). We evaluated the effect of primary adjunctive corticosteroid therapy on outcomes in patients with high-risk KD at our children’s hospital.

METHODS. We performed a single-center retrospective review of high-risk KD patients between 2010 – 2023. From 2012-2017, patients with high-risk KD (infants and children >8 yrs, baseline CA Z score >2.5, shock, or CRP >15 mg/dL + one other high-risk lab test) at our institution were treated with IVIG and aspirin. Short course steroid therapy was administered to 41% of these patients as initial or salvage therapy. From 2017-2023, adjunctive corticosteroids in a RAISE regimen were given to high-risk patients as primary therapy with IVIG and aspirin. For both groups, we analyzed maximum z-scores (z-Max), need for more than one dose of IVIG, % with increase in CA Z scores by 2 standard deviations (SD), and % with decrease in the most recent z-score by more than 2 SD from z-Max.  

RESULTS. A total of 221 high-risk KD patients were treated at our institution between 2012 – 2023. Among these patients, 83 (38%) received the RAISE protocol and 138 (62%) received standard therapy. Demographic and clinical features were similar in the two groups except that a higher percentage of patients had incomplete KD in 2017-2023. There were no significant differences in baseline RCA [1.0 (0.2-2.3) vs 1.14 (0.3-2.08), NS] or LAD [0.8 (0.02-2.1) vs. 0.9 (-0.02-2.04), NS] z-scores between patients receiving the RAISE protocol and those receiving standard therapy. There were no significant differences in z-Max of the LAD or RCA, increase in z-score by 2 SD from baseline to z-Max, or decrease in the most recent z-score by 2 SD between the two treatment groups. RAISE protocol patients were significantly less likely to receive more than one dose of IVIG when compared to standard therapy (11% vs 33%, P< 0.001).

CONCLUSION. High-risk patients who received a RAISE regimen were significantly less likely to require IVIG retreatment when compared with those who initially received IVIG and aspirin alone. Our study is limited by its retrospective nature. We did not observe significant differences in CA outcomes in the two groups.