Evaluation of a Kawasaki Disease Risk Model for Predicting Coronary Artery Aneurysms in a Japanese Population: An Analysis of Post RAISE.

Abstract

OBJECTIVES\nTo test the performance of the Son risk score which was created to predict coronary artery abnormalities from baseline variables in North American patients with Kawasaki disease.\n\n\nSTUDY DESIGN\nThe dataset from Post RAISE, the largest prospective cohort study of Japanese patients with Kawasaki disease to date, was used for the present study. With high risk defined as ≥3 points, sensitivity, specificity, positive predictive value, and negative predictive value for CAA development were calculated. To evaluate the effect of each risk factor in the Son score, the odds ratios (OR) and 95% confidence intervals (CI) were calculated using logistic regression analysis with the presence of CAA at one month after disease onset.\n\n\nRESULTS\nPost RAISE enrolled 2628 consecutive patients with Kawasaki disease and 304 patients had a high risk score, of whom 15.1% showed CAA. At the cutoff ≥ 3 points, the sensitivity was 37.7%, and the specificity was 87.2%. The maximum Z score at baseline ≥ 2.0 (OR: 3.5, 95%CI: 2.3-5.2) and age < 6 months at disease onset (OR: 3.2, 95%CI: 1.9-5.4), were significantly associated with CAA development. However, a high concentration of C-reactive protein (CRP) was not associated with CAA. The area under the ROC curve for the Son score was 0.65 (95%CI: 0.59-0.71).\n\n\nCONCLUSION\nThe Son score had insufficient sensitivity and good specificity in a Japanese cohort of patients with Kawasaki disease. Among the variables comprising the Son score, a large baseline Z score and young age at disease onset were significant, independent predictors of CAA development.