Primary Adjunctive Corticosteroids in the Acute Management of High-Risk Kawasaki Disease in a North American Cohort

Background: Primary adjunctive therapy with corticosteroids have been shown to reduce coronary artery abnormalities in high-risk Kawasaki Disease (KD) patients in Japan (RAISE study). We evaluated their effect on outcomes in North American patients with high-risk KD.

Methods: We performed a single-center retrospective review of high-risk KD patients between 2010 - 2023. From 2017-2023, adjunctive corticosteroids in a modified RAISE regimen (mRAISE) were given to high-risk patients as primary adjunctive therapy with intravenous gammaglobulin (IVIG) and aspirin. We compared CA outcomes in these patients and those presenting from 2010-2016, when mRAISE therapy was not administered.

Results: A total of 221 high-risk KD patients were treated at our institution between 2010-2023. Among these, 83 received the mRAISE regimen and 138 did not (no corticosteroid, n=82, corticosteroid in a non-mRAISE regimen, n=56). There were no significant differences in coronary artery outcomes in the mRAISE and non-mRAISE groups. Patients receiving the mRAISE regimen were significantly less likely to receive more than one dose of IVIG when compared to those who did not receive this regimen (11% vs 33%, p<0.001).

Conclusion: Use of adjunctive primary therapy with corticosteroids in a mRAISE regimen in high-risk KD patients resulted in significantly decreased IVIG retreatment.