Kawasaki disease: What is the epidemiology telling us about the etiology?

Abstract

Kawasaki disease (KD) is an important and common inflammatory vasculitis of early childhood with a striking predilection for the coronary arteries. It is the predominant cause of paediatric acquired heart disease in developed countries. Despite 40 years of research, the aetiology of KD remains unknown and consequently there is no diagnostic test and treatment is non-specific and sub-optimal. The consensus is that KD is due to one or more widely distributed infectious agent(s), which evoke an abnormal immunological response in genetically susceptible individuals. The epidemiology of KD has been extensively investigated in many populations and provides much of the supporting evidence for the consensus regarding etiology. These epidemiological data are reviewed here, in the context of the etiopathogenesis. It is suggested that these data provide additional clues regarding the cause of KD and may account for some of the continuing controversies in the field.

Modelling seasonal variations in the age and incidence of Kawasaki disease to explore possible infectious aetiologies

“…Epidemiological evidence that supports an infectious aetiology includes seasonality in incidence and a young age distribution indicative of the acquisition of immunity (or resistance to symptomatic disease) following infection [2,3]. The incidence and age distribution of cases may vary seasonally in a manner dependent on important epidemiological features of the infection, including the duration of infectiousness and the nature of transmissionrelevant population mixing [4].…”

“…Kawasaki disease (KD) is a paediatric inflammatory syndrome for which an infectious trigger is strongly suspected, but for which no causative organism(s) have been reliably identified [2,5,6]. KD is an acute systemic vasculitis of young children that is diagnosed by the presence of prolonged fever together with a constellation of clinical signs, including rash, changes to the mucous membranes and peripheries, lymphadenopathy and non-purulent conjunctival injection [7].…”

“…KD specifically and uniquely damages the coronary arteries in a minority of cases. It is the leading cause of acquired heart disease in children in asset-rich countries and may be pro-atherosclerotic [2]. Epidemiological and microbiological studies have attempted to link KD to a variety of infectious and environmental exposures, but no reliable association has been found [5,6].…”

Enfermedad de Kawasaki: Consenso interdisciplinario e intersociedades

“…60 Abciximab es un anticuerpo monoclonal con acción antiagregante y de remodelación vascular. Puede asociarse a tratamiento estándar (clase de recomendación II, nivel de evidencia C).…”

Kawasaki Disease in a 2-year-old Child with Dengue Fever: Correspondence

“…The concurrent occurrence of the two disorders is usually problematic and difficult to diagnose [3,4]. As noted by Burgner and Harnden, "KD is due to one or more widely distributed infectious agent(s), which evoke an abnormal immunological response in genetically susceptible individuals [5]." Hence, to clarify the exact relationship between dengue and KD needs advanced immunological study.…”