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

Date Published: May 20, 2019

Publisher: Public Library of Science

Author(s): Elisa Fernandez-Cooke, Ana Barrios Tascón, Judith Sánchez-Manubens, Jordi Antón, Carlos Daniel Grasa Lozano, Javier Aracil Santos, Enrique Villalobos Pinto, Daniel Clemente Garulo, Beatriz Mercader Rodríguez, Matilde Bustillo Alonso, Esmeralda Nuñez Cuadros, Maria Luisa Navarro Gómez, Sara Domínguez-Rodríguez, Cristina Calvo, Iuri Corsini.


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.

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.

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).

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/mm3, maximum temperature < 39.5°C, total duration of fever > 10 days and fever before treatment ≥ 8 days as independent risk factors for developing coronary aneurysms.

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.

Partial Text

Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology presenting predominantly in children less than 5 years of age. Diagnosis is based on clinical criteria that include fever, exanthema, conjunctivitis, changes in the extremities, erythema of oral mucosa and lips and cervical lymphadenopathy. The prognosis depends mainly on the extent of cardiac involvement that can be minimized if treatment with intravenous immunoglobulin (IVIG) is administered before the 10th day of disease[1,2].

Network setup: During 2015 a call for pediatricians who work with KD patients was sent out through the national societies of infectious diseases, rheumatology and cardiology to set up a KD study group that was named KAWA-RACE. A total of 187 pediatricians from 84 Spanish hospitals joined the network. The ethics committee at Instituto de Investigación Hospital 12 de Octubre approved this study (CEIC 15/316). All patient data were fully anonymized before we accessed them.

This is the first national multicenter epidemiological and clinical study on KD in Spain. From a total of 625 children with KD, coronary aneurysms were detected in 60 cases (9.6%), and we identified as primary risk factors associated with their development, the presence of anemia; child’s height bellow than 103 cm, maximum platelets greater than 900,000 / mm3, the total duration of fever greater than 10 days and the duration of fever > 8 days before treatment. In our series, resistance to IVIG treatment was found in more than 15% of the cases, with the development of aneurysms being more frequent in those cases. The known predictive scores of treatment failure are not applicable in our population as it has been described in other non-Japanese populations[23,24].




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