Original Article
Evaluation of Kawasaki Disease Risk-Scoring Systems for Intravenous Immunoglobulin Resistance

https://doi.org/10.1016/j.jpeds.2010.10.031Get rights and content

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.

Section snippets

Methods

We enrolled patients in a randomized, double-blind, placebo-controlled trial of pulsed corticosteroid therapy for primary treatment of KD from December 2002 to December 2004 at 8 clinical centers in North America. The entry criteria, methods, and results of the trial have been published.8 Enrolled patients met modified American Heart Association criteria for KD and were between days 4 and 10 of illness.9 The study was conducted in accordance with institutional review board approval at each

Results

Median time from fever onset to enrollment was 6 days (interquartile range, 6 to 8 days), and the median age of the subjects was 2.9 years (IQR, 1.5 to 4.7 years). Fourteen percent of the subjects were Asian. IVIG re-treatment was administered to 27 of the 198 subjects (14%). Subjects re-treated with IVIG, compared with subjects who were not re-treated, were similar in age but were more likely to be male (82% versus 60%, P = .03) and, in univariate analysis of baseline laboratory variables (

Discussion

With the dataset of the Pediatric Heart Network’s randomized, placebo-blind trial of pulsed corticosteroids for primary therapy for KD, we evaluated the performance of 3 published risk scoring systems for prediction of IVIG resistance, derived from the data of Japanese populations.1, 2, 3 The 3 scoring systems were developed with data from patients who did not receive primary steroid therapy. We therefore performed our analyses both in the entire trial cohort and within the group that received

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Supported by U01 grants from the National Heart, Lung, and Blood Institute (HL068269, HL068270, HL068279, HL068281, HL068285, HL068292, HL068290, and HL068288). The authors declare no conflicts of interest.

Registered with clinicaltrials.gov: NCT00132080.

List of members of the Pediatric Heart Network Investigators is available at www.jpeds.com (Appendix).

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