Estudi d’incidència, prevalença i característiques clíniques de la malaltia de Kawasaki a Catalunya

[cat] ANTECEDENTS: La malaltia de Kawasaki (MK) es una vasculitis sistemica autolimitada que afecta predominantment infants menors de 5 anys. L'etiologia de la MK es desconeguda i, malgrat que es mes prevalent en paisos asiatics, especialment al Japo, amb una incidencia anual que ha augmentat fins a 264.8 per 100,000 nens menors de 5 anys al 2012, te una distribucio universal i es pot manifestar en qualsevol etnia. La immunoglobulina intravenosa (IGIV) es un tractament efectiu i redueix la incidencia de complicacions cardiaques, tot i que existeixen pacients que no responen a aquest tractament. En poblacio japonesa, s'han establert diferents escales per tal d'identificar aquests pacients amb risc de no resposta al tractament, una de les mes utilitzades es l'escala d'Egami, tot i que la seva eficacia no s'ha provat en poblacio no japonesa. HIPOTESI I OBJECTIUS: Aquest estudi ha estat dissenyat per a coneixer la incidencia i les caracteristiques cliniques de la MK a Catalunya. Aquestes s'assumeix que son similars a la d'altres paisos europeus i de la conca mediterrania per motius genetics i geografics. Tambe es preten identificar variables cliniques i/o biologiques pronostiques que permetin classificar els pacients segons la gravetat, coneixer les caracteristiques cliniques i epidemiologiques dels pacients diagnosticats de MK a Catalunya, identificar variables geografiques i d'estacionalitat, identificar variables de resistencia al tractament convencional de la MK i aplicar de l'escala d'Egami a la nostra poblacio per tal de determinar si es efectiva en la practica clinica diaria. RESULTATS: Analisi de dades de 399 pacients entre 2004 i 2014. D'aquests, 233 (58.4%) presentaven MK complerta, 159 (39.8%) incomplerta i 7 (1.7%) van ser considerats pacients atipics. La incidencia anual va ser de 8/100,000 en infants 8 anys al diagnostic, la necessitat de mes de 5 dies d’ingres hospitalari, l’elevacio de transaminases i la presencia de piuria esteril, broncospasme, meningitis aseptica, dolor abdominal i uveitis al diagnostic van establir-se com a predictors de major risc d’aneurismes coronaris (p 0.5 pero no ho seria per a la deteccio dels pacients de risc. . CONCLUSIONS: Aquest es el primer estudi poblacional sobre epidemiologia de la MK a Catalunya i l’area del Mediterrani Occidental. En general, la incidencia, les caracteristiques cliniques i els plans de tractament a la nostra cohort son similars a les descrites en altres paisos europeus. L’escala d’Egami presenta una baixa sensibilitat pero una especificitat elevada en la poblacio catalana i podria constituir-se, per tant, com una eina per a descartar aquells pacients amb baix risc de resistencia al tractament. [eng] Background: Kawasaki disease (KD) is a self-limiting systemic vasculitis that predominantly affects children under 5 years. The etiology is unknown and, although it is more prevalent in Asian countries, especially Japan, it has a worldwide distribution. Intravenous immunoglobulin (IVIG) is an effective treatment and reduces the incidence of cardiac complications although there are patients who do not respond to this treatment. In Japanese population, different scores have been described to identify patients at risk of no response to conventional treatment; one of the most used is the Egami score, although its effectiveness has not been reproduced in non-Japanese population. Objectives: To determine the incidence and clinical features of KD in Catalonia. It is assumed that these are similar to that of other European countries and the Mediterranean basin due to genetic and geographical reasons. It is also intended to identify clinical variables or biological predictors to classify patients according to the severity, identifying geographic and seasonal patterns, identifying resistance variables to conventional treatment and applying Egami score to determine if it is effective in daily clinical practice in our population. Results: Data from 399 patients over the 10-year study period was analyzed (2004-2014). Of those, 233 (58.4%) had complete KD, 159 (39.8) incomplete KD and 7 (1.7%) were considered atypical KD. Mean annual incidence was 3.5/105 children 10th day of illness, ages 8yo and the presence of sterile piuria, broncospasm, aseptic meningitis, abdominal pain and uveitis at diagnosis were found to have higher risk of coronary aneurisms (CAA) (p 0.5. CONCLUSIONS: This is the first population-based study on the epidemiology of KD in the Western Mediterranean area. Incidence, clinical features and treatment plans in our cohort are similar to those described in other European studies. Egami score has low sensitivity but high specificity in Catalan population and could be a tool to rule out patients at low risk of resistance to treatment.