Evaluation of Kawasaki disease risk-scoring systems for intravenous immunoglobulin resistance

Abstract

Objectives: To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment.

Study design: We used data from a randomized trial of pulsed steroids for primary treatment of Kawasaki disease to assess operating characteristics of the 3 risk scores, and we examined whether steroid therapy lowers the risk of coronary artery abnormalities in patients prospectively classified as IVIG resistant.

Results: For comparability with published cohorts, we analyzed the data of 99 patients who were not treated with steroids (16% IVIG-retreated) and identified male sex, lower albumin level, and higher aspartate aminotransferase level as independent risk factors for IVIG resistance. The Kobayashi score was similar in IVIG-resistant and -responsive patients, yielding a sensitivity of 33% and specificity of 87%. There was no interaction of high-risk versus low-risk status by treatment received (steroid versus placebo) with any of the 3 risk score algorithms.

Conclusion: Risk-scoring systems from Japan have good specificity but low sensitivity for predicting IVIG resistance in a North American cohort. Primary steroid therapy did not improve coronary outcomes in patients prospectively classified as being at high-risk for IVIG resistance.

Trial registration: ClinicalTrials.gov NCT00132080.

Figure 1

Baseline-adjusted Treatment Effect Estimates and 95% Confidence Interval by Actual IVIG Retreatment Subgroup and by Estimated Kobayashi High vs. Low Risk Subgroup for Maximum Coronary Artery Z-score at One and Five Weeks Post-Randomization