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

J Pediatr. 2011 May;158(5):831-835.e3. doi: 10.1016/j.jpeds.2010.10.031. Epub 2010 Dec 18.


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.

Publication types

  • Comparative Study
  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, N.I.H., Extramural

MeSH terms

  • Child, Preschool
  • Double-Blind Method
  • Drug Resistance*
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage*
  • Immunologic Factors / administration & dosage*
  • Infant
  • Infusions, Intravenous
  • Male
  • Mucocutaneous Lymph Node Syndrome / diagnosis*
  • Mucocutaneous Lymph Node Syndrome / drug therapy
  • Prognosis
  • ROC Curve
  • Retrospective Studies
  • Treatment Outcome


  • Immunoglobulins, Intravenous
  • Immunologic Factors

Associated data

  • ClinicalTrials.gov/NCT00132080