Inability of Asian risk scoring systems to predict intravenous immunoglobulin resistance and coronary lesions in Kawasaki disease in an Italian cohort
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Since resistance to intravenous immunoglobulin (IVIG) is associated with coronary lesions (CALs) in Kawasaki disease (KD), it is crucial to identify patients at risk to protect them from coronary involvement. The available risk scores to predict IVIG resistance were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. The aim of this study is to evaluate the ability of the Kobayashi, Egami, and Formosa risk scores to predict IVIG resistance and CALs in Italian patients with KD. A multicenter retrospective analysis involving children with KD diagnosed between 2000 and 2015 was carried out: 257 patients were enrolled (57.9% boys, 89.9% Caucasian); 43 patients were IVIG resistant (16.7%). The scores have low sensitivity and specificity in predicting IVIG resistance: respectively, KS 64% and 62.5%, ES 41.4% and 77.4%, and FS 70.8% and 44.9%. The predictive value of the 3 scores for predicting CALs was also poor.
What is Known:
• There are several risk scores developed in the Asian population to early identify patients with KD at risk for immunoglobulin-resistance and thus for coronary lesions.
• Data are scarce on their effectiveness in non-Asian children.
What is New:
• We present a comprehensive analysis of the ability of 3 Asian risk scores in a cohort of mostly Caucasian children to predict immunoglobulin resistance and coronary involvement.
• Low sensitivity and specificity of the Asian scores for immunoglobulin-resistance and coronary lesions suggest the need for criteria specific for different ethnicities.
KeywordsKawasaki disease Caucasian Risk-scoring systems IVIG resistance Coronary artery lesions
American Heart Association
Coronary artery lesions
Erythrocyte sedimentation rate
Red blood cells
White blood cells
Marianna Fabi conceptualized and designed the study and the data collection instruments, drafted the initial manuscript, and supervised the reviewed literature.
Laura Andreozzi carried out the statistical analysis and contributed to the collection of data, and to write the initial draft.
Elena Corinaldesi contributed to the collection of data and supervised the reviewed literature.
Tetyana Bodnar coordinated and supervised data collection, contributed to the critical revision of literature, and critically reviewed the manuscript.
Francesca Lami coordinated and supervised data collection, contributed to the critical revision of literature, and critically reviewed the manuscript.
Andrea Donti coordinated and supervised data collection, contributed to the critical revision of literature, and critically reviewed the manuscript.
Chiara Landini contributed to data acquisition and critically revised the manuscript.
Cristina Cicero contributed to data acquisition and critically revised the manuscript.
Monica Sprocati contributed to data acquisition and critically revised the manuscript.
Barbara Bigucci contributed to data acquisition and critically revised the manuscript.
Claudia Balsamo contributed to data acquisition and critically revised the manuscript.
Giorgia Di Fazzio contributed to data acquisition and critically revised the manuscript.
Federico Marchetti helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.
Bertrand Tchana helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.
Paola Sogno Valin helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.
Enrico Valletta helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.
Lorenzo Iughetti helped to draft the manuscript, contributed to the critical revision of literature, and reviewed the manuscript.
Marcello Lanari supervised the reviewed literature, contributed to the interpretation of data, and critically reviewed the manuscript for important content.
All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
The study was approved by the Central Review Board.
Informed consent was obtained from all individual participants included in the study.
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