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

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Abstract

Introduction/objectives

Asian scores developed to predict unresponsiveness to intravenous immunoglobulin (IVIG) or development of coronary artery aneurysms (CAA) in patients with Kawasaki disease (KD) are not appropriate in Western populations. The purpose of this study is to develop 2 scores, to predict unresponsiveness to IVIG and development of CAA, appropriate for Spanish population.

Method

Data of 625 Spanish children with KD collected retrospectively (2011–2016) were used to identify variables to develop the 2 scores of interest: unresponsiveness to IVIG and development of CAA. A statistical model selected best variables to create the scores, and scores were validated with data from 98 patients collected prospectively.

Results

From 625 patients of the retrospective cohort, final analysis was performed in 439 subjects: 37 developed CAA, and 212 were unresponsive to IVIG. For the score to predict CAA, a cutoff ≥ 8 was considered for high risk, considering a score system with a different weight for each of the eight variables. External validation showed a sensitivity of 22% and a specificity of 75%. The score to predict unresponsiveness to IVIG established a cutoff ≥ 8 for high risk, considering a score system with a different weight for each of the nine variables. External validation showed a sensitivity of 78% and a specificity of 50%.

Conclusions

Two risk scores for KD were developed from Spanish population, to predict development of CAA and unresponsiveness to IVIG; validation in other cohorts could help to implement these tools in the management of KD in other Western populations.

Key Points

• Two risk scores for KD were developed from Spanish population, to predict development of CAA and unresponsiveness to IVIG.

• External validation of the score to predict development of CAA showed a sensitivity of 22% and a specificity of 75%.

• External validation of the score to predict unresponsiveness to IVIG showed a sensitivity of 78% and a specificity of 50%.

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Acknowledgements

The KAWA-RACE study group is composed by the following:

Study coordinator: Elisa Fernández-Cooke (Hospital Doce de Octubre, Spain).

Scientific committee: Elisa Fernandez-Cooke (Hospital 12 de Octubre, Madrid), Cristina Calvo (Hospital Universitario La Paz, Madrid), Ana Barrios Tascón (Hospital Universitario Infanta Sofia, Madrid), Judith Sánchez-Manubens (Hospital Sant Joan de Deu, Barcelona), Jordi Antón (Hospital Sant Joan de Deu, Barcelona), Javier Aracil Santos (Hospital Universitario La Paz, Madrid), Esmeralda Nuñez Cuadros (Hospital Regional Universitario de Malaga), Maria Luisa Navarro Gómez (Hospital Universitario Gregorio Marañon, Madrid), David Moreno Pérez (Hospital Regional Universitario de Málaga).

Collaborating members: María Martín Cantero Pérez (Hospital Regional Universitario de Málaga), Esmeralda Nuñez Cuadros Pérez (Hospital Regional Universitario de Málaga), Begoña Carazo Gallego Pérez (Hospital Regional Universitario de Málaga), Fernando Sánchez García (Hospital La Inmaculada, Almería), Marisol Camacho Lovillo (Hospital Virgen del Rocío, Sevilla), Renata Marqués (Hospital Virgen del Rocío, Sevilla), Olaf Neth (Hospital Virgen del Rocío, Sevilla), Laura, Fernández Silveira (Hospital Virgen del Rocío, Sevilla), Miguel Sánchez Forte (Hospital Torrecardenas, Almería), Ángeles Ortega Montes (Hospital Torrecardenas, Almería), Leticia Isabel Martínez Campos (Hospital Torrecardenas, Almería), Beatriz Bravo Mancheño (Hospital Universitario Virgen de las Nieves, Granada), Margarita Camacho (Hospital Universitario Virgen de las Nieves, Granada), Antonio F Medina Claros (Hospital de la Axarquía, Murcia), Carlos Salido (Hospital de Jerez), María Torres Rico (Hospital Juan Ramón Jiménez de Huelva), Beatriz Ruiz Saez (Hospital Reina Sofia de Córdoba), Elena Fernadez de la Puebla Lechuga (Hospital Reina Sofia de Córdoba), Mª José Lirola Cruz (Instituto Hispalense de pediatría, Sevilla), Kety Maya Carrasco (Instituto Hispalense de pediatría, Sevilla), Moisés Rodríguez González (Hospital Universitario Puerta Del Mar de Cádiz), Enrique Blanca Jover (Complejo Hospitalario Granada), José Uberos Fernández (Complejo Hospitalario Granada), María Mercedes Ibáñez Alcalde (Hospital de Poniente, Almería), Miguel Lafuente Hidalgo (Hospital Universitario Miguel Servet, Zaragoza), Lorenzo Jiménez Montañés (Hospital Universitario Miguel Servet, Zaragoza), Matilde Bustillo Alonso (Hospital Universitario Miguel Servet, Zaragoza), Ariadna Ayerza Casas (Hospital Clínico Universitario Lozano Blesa, Zaragoza), Bárbara Montes Zapico (Hospital San Agustín, Avilés), Carlos Pérez Méndez (Hospital Universitario de Cabueñes, Gijón), Javier Fernández Aracama (Hospital Universitario Basurto, Bilbao), Lucía Rodríguez (Hospital Central de Asturias, Oviedo), María Aleida Ibáñez Fernández (Hospital Central de Asturias, Oviedo), Silvia Escribà Bori (Hospital Universitario Son Espases, Palma de Mallorca), María Concepción Mir Perelló (Hospital Universitario Son Espases, Palma de Mallorca), Mª Ángeles de la Fuente Sánchez (Hospital Universitario Son Espases, Palma de Mallorca), Patricia Aparicio García (Hospital Son Llátzer, Palma de Mallorca), Carlos Briales (Hospital Son Llátzer, Palma de Mallorca), Joaquín Castilla Crespí (Hospital Son Llátzer, Palma de Mallorca), María Elena Colino Gil (Hospital Materno-Infantil de Las Palmas de Gran Canaria), Nerea Delgado Cabrera (Hospital Materno-Infantil de Las Palmas de Gran Canaria), Ana Bello Naranjo (Hospital Materno-Infantil de Las Palmas de Gran Canaria), Jesús Poch Páez (Hospital Materno-Infantil de Las Palmas de Gran Canaria), Moneyba García Yáñez (Hospital Materno-Infantil de Las Palmas de Gran Canaria), Montse González García (Hospital Universitario Nuestra señora de Candelaria de Santa Cruz de Tenerife), Maite Viadero (Hospital Universitario Marqués de Valdecilla, Santander), Beatriz Jiménez Montero (Hospital Universitario Marqués de Valdecilla, Santander), Olga Domínguez García (Hospital Virgen de la Salud de Toledo), Begoña Losada Pinedo (Hospital Virgen de la Salud de Toledo), Gema Iñigo Martín (Hospital Virgen de la Salud de Toledo), Lucía María Escribano Gómez (Hospital General de Albacete), Miguel Lillo Lillo (Hospital General de Albacete), María Isabel Buedo (Hospital General de Albacete), Laura del Rey (Hospital General de Albacete), Elena Urbaneja Rodríguez (Hospital Clínico Universitario de Valladolid), Sara Rellán Rodríguez (Hospital Clínico Universitario de Valladolid), Teresa Cantero (Hospital de Palencia), Beatriz Plata Izquierdo (Complejo Asistencial Universitario de Salamanca), Luisa García-Cuenllas Álvarez (Complejo Asistencial Universitario de Salamanca), Ignacio Oulego Erroz (Hospital de León), Elena Pérez Santaolalla (Hospital Universitario de Burgos), Carlos Alcalde Martín (Hospital Universitario Río Hortega de Valladolid), Fernando Centeno Malfaz (Hospital Universitario Río Hortega de Valladolid), Elena Pérez Gutiérrez (Hospital Universitario Río Hortega de Valladolid), Mª Soledad Jiménez Casso (Hospital General de Segovia), Fredy Prada (Hospital Sant Joan de Déu, Barcelona), Rosa Bou (Hospital Sant Joan de Déu, Barcelona), Estibaliz Iglesias (Hospital Sant Joan de Déu, Barcelona), Joan Calzada (Hospital Sant Joan de Déu, Barcelona), Olga Calavia Garsaball (Hospital Joan XXIII de Tarragona), Marc Tobeña Rue (Hospital Vall d’Hebron, Barcelona), Gemma Giralt García (Hospital Vall d’Hebron, Barcelona), Zulema Lobato (Xarxa Assistencial i Universitaria de Manresa), Neus Rius Gordillo (Hospital Sant Joan de Reus), Montserrat Pascual Torres (Hospital Sant Joan de Reus), María Méndez Hernández (Hospital Germans Trias i Pujol de Badalona), Lourdes García (Hospital de Mataró), Sergio Flores Villar (Hospital Universitari Mútua de Terrassa), Silvia Yevenes Ruiz (Hospital Universitari Mútua de Terrassa), Laura Minguell Domingo (Hospital Arnau de Vilanova, Lleida), Anna Ballester (Hospital de Calella), Ana Miralles (Hospital de Palamós), Berta Pujol Soler (Hospital General de Granollers), Anton Foguet Vidal (F. Hospital d’Olot Comarcal de la Garrotxa), Pere Sala Castellví (Hospital de Barcelona), Angelita Serrano Aguiar (Hospital Comarcal Mòra d’Ebre), José Manuel Siurana Rodríguez (Hospital HM Nens. HM Hospitales), Anna Sangorrin Iranzo (Hospital HM Nens. HM Hospitales), Roser Álvarez Pérez (Hospital de la Santa Creu i Sant Pau de Barcelona), Nathalia Joaqui López, Vanessa Laveglia (Hospital General de Catalunya), Ricardo González Amador (Hospital Verge de la Cinta, Tortosa), Ana Grande Tejada (Complejo Hospitalario Universitario Infanta Cristina, Badajoz), Cristina Zarallo (Complejo Hospitalario Universitario Infanta Cristina, Badajoz), Federico Martinón-Torres (Hospital Clínico Universitario de Santiago de Compostela), Irene Rivero Calle (Hospital Clínico Universitario de Santiago de Compostela), Antonio Justicia Grande, (Hospital Clínico Universitario de Santiago de Compostela), María López Sousa (Hospital Clínico Universitario de Santiago de Compostela), Alejandro Souto Vilas (Hospital Clínico Universitario de Santiago de Compostela), Bernardo López Abel (Hospital Clínico Universitario de Santiago de Compostela), Elisa de Miguel Esteban (Hospital San Pedro, Logroño), Bibiana Riaño Méndez (Hospital San Pedro, Logroño), Daniel Blázquez (Hospital Universitario Doce de Octubre, Madrid), Pablo Rojo Conejo (Hospital Universitario Doce de Octubre, Madrid), Carlos Grasa Lozano (Hospital Universitario La Paz, Madrid), Belén Toral (Hospital Universitario Doce de Octubre, Madrid), Leticia Albert De la Torre (Hospital Universitario Doce de Octubre, Madrid), Jaime de Inocencio (Hospital Universitario Doce de Octubre, Madrid), Mar Santos (Hospital Universitario Gregorio Marañon, Madrid), Rafael Díaz-Delgado de la Peña (Hospital Severo Ochoa, Leganés), Paz Collado Ramos (Hospital Severo Ochoa, Leganés), Alfredo Tagarro (Hospital Universitario Infanta Sofia, Madrid), Teresa Raga (Hospital Universitario Infanta Sofia, Madrid), Libertad Latorre (Hospital Universitario Infanta Sofia, Madrid), Sara Guillén (Hospital de Getafe), Ignacio Callejas Caballero (Hospital de Getafe), María Esquivas Asenjo (Hospital de Getafe), Luis Manuel Prieto Tato (Hospital 12 de Octubre), Sandra Villagrá (HM Hospitales Madrid), Viviana Arreo (HM Hospitales Madrid), Roi Piñeiro Pérez (Hospital General de Vilalba), María de la Parte (Hospital General de Villalba), Amalia Tamariz-Martes (Hospital Infantil Universitario Niño Jesús, Madrid), Marta Llorente Romano (Hospital del Sureste, Madrid), Maria Belén Hernández Rupérez (Hospital del Sureste, Madrid), Henar Rojo Sombrero (Hospital Universitario Príncipe de Asturias, Alcalá de Henares), Estefanía García Cerro (Hospital Universitario Príncipe de Asturias, Alcalá de Henares), Irene Maté Cano (Hospital del Henares, Coslada), Marta Villares Alonso (Hospital de Móstoles), Marta Pilar Osuna Marco (Hospital de Móstoles), Julia Jensen Veron (Hospital Infanta Cristina, Madrid), Cristina Zarallo Reales (Hospital Infanta Cristina, Madrid), María Dolores Rodríguez Mesa (Hospital Infanta Cristina, Madrid), Santiago Rueda Esteban (Hospital Clínico San Carlos, Madrid), José Tomás Ramos Amador (Hospital Clínico San Carlos, Madrid), Cristina González Menchén (Hospital Clínico San Carlos, Madrid), Ana Belén Jiménez Jiménez (Hospital Universitario Fundación Jiménez Díaz, Madrid), Pilar Galán (Hospital Universitario de Fuenlabrada), Dolores Pérez Campos (Hospital Universitario de Fuenlabrada), Mª Mercedes Bueno (Fundación Hospital de Alcorcón), David Crespo Marcos (Fundación Hospital de Alcorcón), Enrique Otheo de Tejada Barásoain (Hospital Ramón y Cajal, Madrid), Walter Alberto Sifuentes Giraldo (Hospital Ramón y Cajal, Madrid), María Luz Gámir Gámir (Hospital Ramón y Cajal, Madrid), María José Cilleruelo Ortega (Hospital Puerta de Hierro, Majadahonda), Agustín López López (Hospital Puerta de Hierro, Majadahonda), Cristina Sánchez Vaquerizo (Hospital Puerta de Hierro, Majadahonda), Ana Isabel Usano Carrasco (Hospital Puerta de Hierro, Majadahonda), Ester Moreno Gómez (Hospital San Rafael, Madrid), Olga Carvajal del Castillo (Hospital Rey Juan Carlos, Móstoles), Beatriz Del Pozo Menéndez Hospital de Torrejón), Katie Badillo Navarro (Hospital de Torrejón), Fernando Baquero (Hospital Universitario La Paz, Madrid), Lucía Deiros Bronte (Hospital Universitario La Paz, Madrid), Pablo Fernández Fraga (Hospital Universitario La Paz, Madrid), Nieves Domínguez (Hospital Universitario Infanta Elena, Madrid), Beatriz Mercader Rodríguez (Hospital Clínico Universitario Virgen de la Arrixaca, Murcia), Francisco Castro García (Hospital Clínico Universitario Virgen de la Arrixaca, Murcia), Águeda Herrera Chamorro (Hospital Clínico Universitario Virgen de la Arrixaca, Murcia), Paula Alcañiz Rodríguez (Hospital Clínico Universitario Virgen de la Arrixaca, Murcia), Moisés Sorlí García (Hospital Clínico Universitario Virgen de la Arrixaca, Murcia), María Concepción Rex Nicolás (Hospital Universitario Santa Lucía, Cartagena), Elena Vera Romero (Hospital Universitario Santa Lucía, Cartagena), Patricia Martínez Olorón (Complejo Hospitalario de Navarra), Beatriz Rocandio Cilveti (Hospital de Donostia), Amaia Berridi (Hospital de Donostia), Laura Santos-Díez Vázquez (Hospital de Cruces, Bilbao), Olaia Fernández (Hospital Universitario Basurto, Bilbao), Inmaculada Calvo (Hospital Universitario y Politécnico La Fe, Valencia), Belén Fernández Tudela (Hospital Universitario y Politécnico La Fe, Valencia), Manuel Oltra Benavent (Hospital Universitario y Politécnico La Fe, Valencia), Marta Dapena Archilés (Hospital General de Castellón), Franciasco Sanchez Ferrer (Hospital Universitario de San Juan de Alicante), César Gavilán (Hospital Universitario de San Juan de Alicante), Ignacio Izquierdo Fos (Hospital Universitario de Elche), María Isabel Serrano Robles (Hospital Universitario de Elche), Yolanda Herranz Sánchez (Hospital Marina Baixa de Alicante), Enrique Villalobos Pinto (Hospital Infantil Universitario Niño Jesús, Madrid), Daniel Clemente Garulo (Hospital Infantil Universitario Niño Jesús, Madrid), Stella Pie (Hospital Universitari Doctor Josep Trueta, Girona), Manuel Marrero Calvo (Hospital Nuestra Señora de Sonsoles, Ávila), José María Olmos García (Hospital Mare de Déu dels Lliris, de Alcoy, Alicante).

Author information

Authors and Affiliations

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

    Carlos D. Grasa, Javier Aracil-Santos & Cristina Calvo

  2. IdiPaz, Institute for Health Research from La Paz Hospital, Madrid, Spain

    Carlos D. Grasa & Cristina Calvo

  3. CIBERINFEC, CIBER of Infectious Diseases in Spain (Instituto de Salud Carlos III – ISCIII), Seville, Spain

    Carlos D. Grasa & Cristina Calvo

  4. Pediatric Infectious Diseases Unit, Pediatric Research and Clinical Trial Unit (UPIC), Department of Pediatrics, Hospital Universitario 12 Octubre, Avda. Córdoba s/n., 28041, Madrid, Spain

    Elisa Fernández-Cooke & Sara Domínguez-Rodríguez

  5. imas12, Instituto de Investigación Sanitaria Hospital 12 de Octubre, Madrid, Spain

    Elisa Fernández-Cooke & Sara Domínguez-Rodríguez

  6. RITIP, Spanish Network for the Research in Pediatric Infectious Diseases, Madrid, Spain

    Elisa Fernández-Cooke & Cristina Calvo

  7. Department of Pediatrics, Hospital Universitario Infanta Sofia, San Sebastian de los Reyes, Madrid, Spain

    Ana Barrios Tascon

  8. Department of Pediatric Rheumatology, Hospital Sant Joan de Deu, Universitat de Barcelona, Barcelona, Spain

    Judith Sánchez-Manubens & Jordi Antón

  9. Department of Pediatric Rheumatology, Hospital Parc Tauli, Sabadell, Spain

    Judith Sánchez-Manubens

  10. Department of Pediatrics, Hospital Virgen de la Arrixaca, Murcia, Spain

    Beatriz Mercader

  11. Pediatric Rheumatology Unit, Department of Pediatrics, Hospital Regional Universitario, Malaga, Spain

    Esmeralda Nuñez

  12. Department of Pediatrics, Hospital Niño Jesús, Madrid, Spain

    Enrique Villalobos

  13. Department of Pediatric Infectious Diseases, Hospital Miguel Servet, Zaragoza, Spain

    Matilde Bustillo

  14. Pediatric Infectious Diseases, Rheumatology and Immunology Unit, Department of Pediatrics, Hospital Virgen del Rocio, Sevilla, Spain

    Marisol Camacho

  15. Department of Pediatrics, Hospital Universitario y Politécnico La Fe, Valencia, Spain

    Manuel Oltra Benavent

  16. Department of Pediatric Cardiology, Hospital Vall d’Hebron, Barcelona, Spain

    Gemma Giralt

  17. Department of Pediatrics, Hospital Universitario Materno-Infantil de Las Palmas de Gran Canaria, Canarias, Spain

    Ana Maria Bello Naranjo

  18. Department of Pediatrics, Hospital Universitario Donostia, San Sebastian, Spain

    Beatriz Rocandio

Authors
  1. Carlos D. Grasa
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  2. Elisa Fernández-Cooke
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  3. Sara Domínguez-Rodríguez
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  6. Judith Sánchez-Manubens
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  7. Beatriz Mercader
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  9. Esmeralda Nuñez
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  10. Enrique Villalobos
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on behalf of the Spanish group for the study of Kawasaki Disease (KAWA-RACE)

  • Elisa Fernandez-Cooke
  • , Cristina Calvo
  • , Ana Barrios Tascón
  • , Judith Sánchez-Manubens
  • , Jordi Antón
  • , Javier Aracil Santos
  • , Esmeralda Nuñez Cuadros
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  • , Ignacio Oulego Erroz
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  • , Ana Isabel Usano Carrasco
  • , Ester Moreno Gómez
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  • , Beatriz Del Pozo Menéndez
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  • , Fernando Baquero
  • , Lucía Deiros Bronte
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  • , Nieves Domínguez
  • , Beatriz Mercader Rodríguez
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  • , Paula Alcañiz Rodríguez
  • , Moisés Sorlí García
  • , María Concepción Rex Nicolás
  • , Elena Vera Romero
  • , Patricia Martínez Olorón
  • , Beatriz Rocandio Cilveti
  • , Amaia Berridi
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  • , Olaia Fernández
  • , Inmaculada Calvo
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  • , Enrique Villalobos Pinto
  • , Daniel Clemente Garulo
  • , Stella Pie
  • , Manuel Marrero Calvo
  •  & José María Olmos García

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Correspondence to Elisa Fernández-Cooke.

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Grasa, C.D., Fernández-Cooke, E., Domínguez-Rodríguez, S. et al. Risk scores for Kawasaki disease, a management tool developed by the KAWA-RACE cohort. Clin Rheumatol 41, 3759–3768 (2022). https://doi.org/10.1007/s10067-022-06319-4

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