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

Lynn A Sleeper, L Luann Minich, Brian M McCrindle, Jennifer S Li, Wilbert Mason, Steven D Colan, Andrew M Atz, Beth F Printz, Annette Baker, Victoria L Vetter, Jane W Newburger, Pediatric Heart Network Investigators, David R Fulton, Robert P Sundel, Amy L Woodward, Fatma Dedeoglu, Bryce Beinstadt, Susan Kim, Ellen McGrath, Masato Takahashi, Vanessa Guerrero, Alexa Hogarty, Steven Paridon, Jack Rychik, Meg Harkins, Lisa Imundo, Deborah Levy, Daphne Hsu, Seema Mital, Page Anderson, Ann Marie Nawrocki, Timothy Bradley, Rae Yeung, Elizabeth Radojewski, Girish Shirali, Mark Scheurer, Amy Jones, Richard V Williams, Deborah U Frank, Lloyd Y Tani, Linda M Lambert, Abigail L Smart, Gloria Klein, Patti Nash, Dianne Gallagher, Steven D Douglas, Donald E Campbell, Joseph M McMann, Patricia Wuthnow, Nichole Gilper, David Cabral, Edward Marcus, Gail D Pearson, Victoria Pemberton, Mario Stylianou, Judith Massicot-Fisher, Tracey Hoke, Lynn Mahony, Lynn A Sleeper, L Luann Minich, Brian M McCrindle, Jennifer S Li, Wilbert Mason, Steven D Colan, Andrew M Atz, Beth F Printz, Annette Baker, Victoria L Vetter, Jane W Newburger, Pediatric Heart Network Investigators, David R Fulton, Robert P Sundel, Amy L Woodward, Fatma Dedeoglu, Bryce Beinstadt, Susan Kim, Ellen McGrath, Masato Takahashi, Vanessa Guerrero, Alexa Hogarty, Steven Paridon, Jack Rychik, Meg Harkins, Lisa Imundo, Deborah Levy, Daphne Hsu, Seema Mital, Page Anderson, Ann Marie Nawrocki, Timothy Bradley, Rae Yeung, Elizabeth Radojewski, Girish Shirali, Mark Scheurer, Amy Jones, Richard V Williams, Deborah U Frank, Lloyd Y Tani, Linda M Lambert, Abigail L Smart, Gloria Klein, Patti Nash, Dianne Gallagher, Steven D Douglas, Donald E Campbell, Joseph M McMann, Patricia Wuthnow, Nichole Gilper, David Cabral, Edward Marcus, Gail D Pearson, Victoria Pemberton, Mario Stylianou, Judith Massicot-Fisher, Tracey Hoke, Lynn Mahony

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.

Conflict of interest statement

The authors declare no conflicts of interest..

Copyright © 2011 Mosby, Inc. All rights reserved.

Figures

Figure 1
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

Source: PubMed

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