Identifying Kawasaki Disease With a Low Coronary Artery Aneurysm Risk.

Introduction: Kawasaki disease (KD) patients with a high risk of coronary artery aneurysm (CAA) development are well characterized and targeted for intensified primary intravenous immunoglobulin (IVIG) treatment. However, the characteristics of KD patients with a low CAA risk are less well-known.
Methods: The present study was a secondary analysis of Prospective Observational study on STRAtified treatment with Immunoglobulin plus Steroid Efficacy for Kawasaki disease (Post RAISE), a multicenter, prospective cohort study of KD patients in Japan. The target of the analysis was patients with a Kobayashi score <5 who were predicted to respond to IVIG. The incidence of CAA during the acute phase, the primary outcome, was assessed based on all echocardiographic evaluations performed between week 1 (days 5-9) and month 1 (days 20-50) after the start of primary treatment. Multivariable logistic regression was used to identify the independent risk factors of CAA during the acute phase, based on which a decision tree was created to identify a subpopulation of patients with KD with a low CAA risk.
Results: Multivariate analysis found that a baseline maximum Z score >2.5, age <12 months at fever onset, nonresponsiveness to IVIG, low neutrophils, high platelets and high C-reactive protein were independent predictors of CAA during the acute phase. The decision tree created by using these risk factors identified 679 KD patients who had a low incidence of CAA during the acute phase (4.1%) and no medium or large CAA.
Conclusions: The present study identified a KD subpopulation with a low CAA risk comprising around a quarter of the entire Post RAISE cohort.
Competing Interests: M.M. received an honorarium from the Japan Blood Products Organization, Teijin Pharma Ltd., and Nihon Pharmaceutical Co, Ltd. outside the submitted work. Y.M. received a Health Science and Labor Research Grant and grants from the Tokyo Metropolitan Government Hospitals, the Japan Agency for Medical Research and Development, the Center for Clinical Trials of the Japan Medical Association, the Japan Science and Technology Agency, the New Energy and Industrial Technology Development Organization, and the Japan Society for the Promotion of Science outside the submitted work. None of these entities were involved in this study in any manner. The other authors have no conflicts of interest to disclose.
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