title: Role of the Egami score to predict immunoglobulin resistance in Kawasaki disease among a Western Mediterranean population.
abstract: Kawasaki disease is an acute self-limited systemic vasculitis common in childhood. Intravenous immunoglobulin (IVIG) is an effective treatment, and it reduces the incidence of cardiac complications. Egami score has been validated to identify IVIG non-responder patients in Japanese population, and it has shown high sensitivity and specificity to identify these non-responder patients. Although its effectiveness in Japan, Egami score has shown to be ineffective in non-Japanese populations. The aim of this study was to apply the Egami score in a Western Mediterranean population in Catalonia (Spain). Observational population-based study that includes patients from all Pediatric Units in 33 Catalan hospitals, both public and private management, between January 2004 and March 2014. Sensitivity and specificity for the Egami score was calculated, and a logistic regression analysis of predictors of overall response to IVIG was also developed. Predicting IVIG resistance with a cutoff for Egami score ≥3 obtained 26 % sensitivity and 82 % specificity. Negative predictive value was 85 % and positive predictive value 22 %. This low sensitivity implies that three out of four non-responders will not be identified by the Egami score. Besides, logistic regression models did not found significance for the use of the Egami score to predict IVIG resistance in Catalan population although having an area under the ROC curve of 0.618 (IC 95 % 0.538-0.698, p < 0.001). Although regression models found an area under the ROC curve >0.5 to predict IVIG resistance, the low sensitivity excludes the Egami score as a useful tool to predict IVIG resistance in Catalan population.
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Parc Taulí, C/Parc Taulí, 2, 08208, Sabadell, Barcelona, Spain. jsanchez@tauli.cat.|Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain. jsanchez@tauli.cat.
authors.forename: Judith
authors.initials: J
authors.lastname: Sánchez-Manubens
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
authors.forename: Jordi
authors.initials: J
authors.lastname: Antón
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
authors.forename: Rosa
authors.initials: R
authors.lastname: Bou
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
authors.forename: Estíbaliz
authors.initials: E
authors.lastname: Iglesias
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
authors.forename: Joan
authors.initials: J
authors.lastname: Calzada-Hernandez
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
authors.forename: Sergi
authors.initials: S
authors.lastname: Borlan
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
authors.forename: Clara
authors.initials: C
authors.lastname: Gimenez-Roca
authors.affiliation: Unitat de Reumatologia Pediàtrica, Servei de Pediatria, Hospital Parc Taulí, C/Parc Taulí, 2, 08208, Sabadell, Barcelona, Spain.
authors.forename: Josefa
authors.initials: J
authors.lastname: Rivera
mesh_terms: D000293:Adolescent; D019540:Area Under Curve; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D003661:Decision Support Techniques; D004351:Drug Resistance; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage*; D016015:Logistic Models; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology; D016017:Odds Ratio; D011237:Predictive Value of Tests; D012372:ROC Curve; D012189:Retrospective Studies; D012307:Risk Factors; D013030:Spain / Q000453:epidemiology; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
keywords: Coronary aneurism; Egami score; Immunoglobulin resistance; Kawasaki disease