Epidemiology

Kawasaki disease (KD) almost exclusively affects young children, with peak incidence between the ages of 13-24 months.[2] It is rare in the first 6 months of life, and 80% of all cases occur before the age of 5 years.[3] It affects approximately 5000 US children annually with an incidence in the US of 24.7 per 100,000 children ages <5 years in 2010, based on hospitalization data.[4] The incidence in the UK is 4.5 per 100,000 children ages <5 years.[5]

Although KD has been described in all ethnicities throughout the world.[6] The incidence is highest in patients from Northeast Asia, especially Japan and Korea, suggesting a significant role for host genetics in pathogenesis. In the US, children of Asian/Pacific origin have the highest KD rate (50.4 per 100,000), followed by children of black (29.8) and white (22.5) origins.[4] An earlier report from 1997-2000 showed an annual incidence of 16.9, 11.1, and 9.1 in 100,000 in the US for black, Hispanic, and white children ages <5 years, respectively.[7] However, the annual incidence of KD among American Indians and Alaskan native children ages <5 years in 1999 was found to be only 4.3 in 100,000, despite the Asian ancestral origin of these children.[8] The figures for children of Asian-American and Pacific Island origin are reported to be higher, at 32.5 in 100,000.[7]

The annual incidence in Japan was estimated in 1999 as 75-125 cases in 100,000 in children ages <5 years.[9] However, a more recent study showed that children of Japanese ancestry have a much higher incidence of 243.1 to 264.8 per 100,000.[4][10] The recurrence rate in Japanese children is 3%.[4]

Japanese KD surveillance studies have shown the incidence of KD is increasing relative to the decreasing birth rate.[11]

There is increasing awareness of the long-term morbidity and mortality associated with coronary artery aneurysm development; with a lifelong risk of coronary artery thrombosis, rupture, and myocardial infarction.[12]

Untreated, the incidence of coronary artery aneurysm is thought to be between 20% to 25%. The British Paediatric Surveillance Unit survey reports 19% of treated patients in the UK with KD still developed coronary artery aneurysms increasing to 39% in those ages under 1 year despite intravenous immune globulin.[5]

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