Epidemiological and clinical features of Kawasaki disease in Spain over 5 years and risk factors for aneurysm development. (2011-2016): KAWA-RACE study group

Fecha de publicación: 20/05/2019

Autores de IIS La Fe

María Remedios Marques Miñana

Autor
Monica Montero Hernandez

Autor
Maria Dolores Santos Agull

Autor
Inmaculada Calvo Penadés

Autor
María Isabel González Fernández

Autor

Participantes ajenos a IIS La Fe

Fernandez-Cooke, E
Tascon, AB
Sanchez-Manubens, J
Anton, J
Lozano, CDG
Santos, JA
Pinto, EV
Garulo, DC
Rodriguez, BM
Alonso, MB
Cuadros, EN
Gomez, MLN
Dominguez-Rodriguez, S
Calvo, C
Perez, DM
Cantero, MM
Gallego, BC
Campos, LIM
Garcia, FS
Lovillo, MC
Neth, O
Silveira, LF
Forte, MS
Montes, AO
Mancheno, BB
Camacho, M
Claros, AFM
Salido, C
Cruz, RC
Rico, MT
Hernandez, MAT
Guzman, EG
Marin, MJA
Aguirre, JR
Cruz, MJL
Carrasco, KM
Gonzalez, MR
Jover, EB
Lopez, AR
Fernandez, JU
Alcalde, MMI
Collado, P
Laleona, CG
Montanes, LJ
Casas, AA
Zapico, BM
Mendez, CP
Aracama, JF
Rodriguez, L
Fernandez, MAI
Campo, SN
Bori, SE
Perello, MCM
Sanchez, MAD
Garcia, PA
Briales, C
Crespi, JC
Gil, MEC
Cabrera, ND
Naranjo, AB
Paez, JP
Yanez, MG
Garcia, MG
Bezanilla, EP
Montero, BJ
Garcia, OD
Pinedo, BL
Martin, GI
Gomez, LME
Cepillo, A
Lillo, M
Buedo, MI
del Rey, L
Rodriguez, EU
Rodriguez, SR
Cantero, T
Izquierdo, BP
Alvarez, LGC
Erroz, IO
Santaolalla, EP
Martin, CA
Malfaz, FC
Gutierrez, EP
Casso, MJ
Prada, F
Bou, R
Iglesias, E
Calzada, J
Garsaball, OC
Rue, MT
Garcia, GG
Ruiz, SY
Lobato, Z
Gordillo, NR
Torres, MP
Garcia, L
Villar, SF
Domingo, LM
Ballester, A
Miralles, A
Soler, BP
Vidal, AF
Castellvi, PS
Aguiar, AS
Rodriguez, JMS
Iranzo, AS
Perez, RA
Cajas, PR
Jornet, PGI
Tejada, AG
Zarallo, C
Martinon-Torres, F
Calle, IR
Grande, AJ
Saez, BR
Sousa, ML
Vilas, AS
Abe, BL
Esteban, ED
Mendez, BR
Blazquez, D
Conejo, PR
Tome, MG
Lozano, CG
Toral, B
De la Torre, LA
de Inocencio, J
de la Pena, RDD
Ramos, PC
Tagarro, A
Raga, T
Latorre, L
Guillen, S
Caballero, IC
Tato, LMP
Monagas, MFG
Lopez, IJ
Villagra, S
Arreo, V
Perez, RP
de la Parte, M
Tamariz-Martes, A
Romano, ML
Ruperez, BH
Martin, SR
Sombrero, HR
Cerro, EG
Cano, IM
Alonso, MV
Marco, MPO
Veron, JJ
Mesa, MDR
Esteban, SR
Amador, JTR
Menchen, CG
Jimenez, AB
Galan, P
Campos, DP
Bueno, MM
Marcos, DC
Barasoain, EOD
Giraldo, WAS
Gamir, ML
Ortega, MJC
Lopez, A
Vaquerizo, CS
Carrasco, AIU
Gomez, EM
Colom, JC
del Castillo, OC
Menendez, BD
Navarro, KB
Baquero, F
Bronte, LD
Fraga, PF
Dominguez, N
Garcia, FC
Romero, EV
Chamorro, AH
Rodriguez, PA
Garcia, MS
Nicolas, MCR
Nicolas, AL
Oloron, PM
Cilveti, BR
Berridi, A
Vazquez, LSD
Fernandez, O
Tamarit, AP
Lopez, B
Otero, MC
Oltra, M
Archiles, MD
Sanchez, P
Gavilan, C
Fos, II
Robles, MIS
Sanchez, YH
KAWA-RACE Study Grp

Grupos

Reumatología Pediátrica

Investigador establecido

Inmaculada Calvo Penadés

Abstract

Background Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology affecting mainly children less than 5 years of age. Risk factors for cardiac involvement and resistance to treatment are insufficiently studied in non-Japanese children. Objective This study aimed to investigate the epidemiology, clinical features and risk factors for resistance to treatment and coronary artery lesions (CAL) in KD in Spain. Methods Retrospective study (May 2011-June 2016) of all patients less than 16 years of age diagnosed with KD included in KAWA-RACE network (84 Spanish hospitals). Results A total of 625 cases were analyzed, 63% were males, 79% under 5 year-olds and 16.8% younger than 12 months. On echocardiographic examination CAL were the most frequent findings (23%) being ectasia the most common (12%). Coronary aneurysms were diagnosed in 9.6%, reaching 20% in infants under 12 months (p<0.001). A total of 97% of the patients received intravenous immunoglobulin (IVIG) with a median number of days from fever onset to IVIG administration of 7.2. A second dose was given to 15.7% and steroids to 14.5% patients. Only 1.4% patients received infliximab. No deaths were reported. A multivariate analysis identified anemia, hypoalbuminemia, hyponatremia, higher creatinine and procalcitonin as independent risk factors for treatment failure and length under 103 cm, hemoglobin < 10.2 mg/dL, platelets > 900,000 cells/mm(3), maximum temperature < 39.5 degrees C, total duration of fever > 10 days and fever before treatment >= 8 days as independent risk factors for developing coronary aneurysms. Conclusions In our population, children under 12 months develop coronary aneurysms more frequently and children with KD with anemia and leukocytosis have high risk of cardiac involvement. Adding steroids early should be considered in those patients, especially if the treatment is not started before 8 days of fever. A score applicable to non-Japanese children able to predict the risk of aneurysm development and IVIG resistance is necessary.