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

Background: Primary adjunctive therapy with corticosteroids has been shown to reduce coronary artery (CA) abnormalities in high-risk Kawasaki disease (KD) patients in Japan (the randomized control trial to assess immunoglobulin plus steroid efficacy [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 and 2023. From 2017 to 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 to 2016, when mRAISE therapy was not administered.

Results: A total of 221 high-risk KD patients were treated at our institution between 2010 and 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 CA 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 < .001).

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