Marianna Fabi1, Laura Andreozzi2, Elena Corinaldesi3, Tetyana Bodnar2, Francesca Lami4, Cristina Cicero5, Bertrand Tchana6, Chiara Landini7, Monica Sprocati8, Barbara Bigucci9, Claudia Balsamo10, Paola Sogno Valin11, Giorgia Di Fazzio12, Lorenzo Iughetti4, Enrico Valletta13, Federico Marchetti14, Andrea Donti15, Marcello Lanari2. 1. Department of Pediatrics, S.Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 9, 40138, Bologna, Italy. marianna.fabi@aosp.bo.it. 2. Department of Pediatrics, S.Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 9, 40138, Bologna, Italy. 3. Department of Pediatrics, Ramazzini Hospital, Carpi, Italy. 4. Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, Modena, Italy. 5. Department of Pediatrics, AUSL, Guglielmo da Saliceto Hospital, Piacenza, Italy. 6. Department of Pediatrics, Ospedale dei Bambini Barilla, University of Parma, Parma, Italy. 7. Department of Pediatrics, Maggiore Hospital, Bologna, Italy. 8. Department of Pediatrics, Arcispedale Sant'Anna, Ferrara, Italy. 9. Department of Pediatrics, Infermi Hospital, Rimini, Italy. 10. Department of Pediatrics, Bufalini Hospital, Cesena, Italy. 11. Department of Pediatrics, Santa Maria della Scaletta Hospital, Imola, Italy. 12. Department of Pediatrics, IRCCS, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy. 13. Department of Pediatrics, G.B.Morgagni - L. Pierantoni Hospital, AUSL della Romagna, Forlì, Italy. 14. Department of Pediatrics, Santa Maria delle Croci Hospital, AUSL della Romagna, Ravenna, Italy. 15. Pediatric Cardiology and Adult Congenital Unit, S.Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
Abstract
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. Conclusion: Kobayashi, Egami, and Formosa Scores are ineffective in predicting IVIG resistance and coronary involvement in a predominantly Caucasian cohort. A specific score system for mostly Caucasian children with KD is needed enable the early identification of those at risk for CALs who could benefit from intensified treatment. 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.
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. Conclusion: Kobayashi, Egami, and Formosa Scores are ineffective in predicting IVIG resistance and coronary involvement in a predominantly Caucasian cohort. A specific score system for mostly Caucasian children with KD is needed enable the early identification of those at risk for CALs who could benefit from intensified treatment. 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.