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Risk scores for Kawasaki disease, a management tool developed by the KAWA-RACE cohort

Carlos D Grasa1,2,3, Elisa Fernández-Cooke4,5,6, Sara Domínguez-Rodríguez7,8

  • 1Department of Pediatric Infectious Diseases, La Paz Children's Hospital (IdiPaz Foundation), Madrid, Spain.

Clinical Rheumatology
|August 8, 2022
PubMed

Insights

New risk scores for Kawasaki disease (KD) predict coronary artery aneurysms (CAA) and intravenous immunoglobulin (IVIG) unresponsiveness in Spanish children. These scores aim to improve KD management in Western populations.

Area of Science:

  • Pediatrics
  • Cardiology
  • Immunology

Background:

  • Existing risk scores for Kawasaki disease (KD) are not suitable for Western populations.
  • Kawasaki disease requires timely management to prevent complications like coronary artery aneurysms (CAA).
  • Intravenous immunoglobulin (IVIG) is a standard treatment, but some patients remain unresponsive.

Purpose of the Study:

  • To develop and validate two novel risk scores for the Spanish population.
  • To predict the likelihood of developing CAA in pediatric KD patients.
  • To predict unresponsiveness to IVIG treatment in KD patients.

Main Methods:

  • Retrospective analysis of 625 Spanish children with KD (2011-2016).
  • Statistical modeling to identify key variables for predicting CAA and IVIG unresponsiveness.
  • Validation of the developed scores using prospective data from 98 patients.

Main Results:

  • A risk score for CAA was developed with 8 variables; a cutoff of ≥ 8 indicated high risk (sensitivity 22%, specificity 75%).
  • A risk score for IVIG unresponsiveness was developed with 9 variables; a cutoff of ≥ 8 indicated high risk (sensitivity 78%, specificity 50%).
  • Analysis included 439 subjects from the retrospective cohort; 37 developed CAA, 212 were unresponsive to IVIG.

Conclusions:

  • Two validated risk scores for Kawasaki disease have been developed for the Spanish population.
  • These scores aim to predict coronary artery aneurysm development and IVIG unresponsiveness.
  • Further validation in diverse Western cohorts is recommended for clinical implementation.
Abstract

Keywords:
Coronary artery aneurysmKawasaki diseaseScoreUnresponsiveness IVIG

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