Epidemiologic Features of Kawasaki Disease in Japan: Results of the 2009–2010 Nationwide Survey

Abstract

BackgroundAlthough the number of patients and incidence rate of Kawasaki disease (KD) are increasing in Japan, the most recent epidemiologic features of KD are not known.MethodsThe 21st nationwide survey of KD was conducted in 2011 and included patients treated for the disease in 2009 and 2010. Hospitals specializing in pediatrics, and hospitals with a total of 100 or more beds and a pediatric department, were asked to report all patients with KD during the 2 survey years.ResultsA total of 1445 departments and hospitals reported 23 730 KD patients (10 975 in 2009 and 12 755 in 2010): 13 515 boys and 10 215 girls. The annual incidence rates were 206.2 and 239.6 per 100 000 children aged 0 to 4 years in 2009 and 2010, respectively; the 2010 rate was the highest ever reported in Japan. Monthly number of patients peaked during winter to spring months; lower peaks were noted during summer months. However, the seasonal patterns in 2009 and 2010 differed from those of previous years. The age-specific incidence rate had a monomodal distribution, with a peak during the latter half of the year of birth. The prevalences of cardiac lesions during acute KD and cardiac sequelae were higher among infants and older age groups. Despite a decrease in prevalence, the proportion of patients with giant coronary aneurysms—the most severe sequela of KD—did not substantially decrease.ConclusionsThe incidence rate and number of patients with KD continue to increase in Japan.

Tropospheric winds from northeastern China carry the etiologic agent of Kawasaki disease from its source to Japan

“…A core element in addressing these questions is the high-resolution daily KD case record for the 47 Japanese prefectures (8). The Japanese Epidemiologic Surveillance System for KD provided the KD onset dates for all cases during the interval 1970-2010 (Data and Methods; see also the clinical definition of KD cases in ref.…”

“…Apart from the three main KD epidemics in Japan, there are seasonal peaks and troughs in disease occurrence (8). We therefore investigated coincidence in potential source regions for KD during periods out of epidemics, and conducted backward trajectory analyses also for high KD activity after the last big epidemic (in the postepidemic interval, 1987-2010).…”

The Role of Neutrophil Activation in the Pathogenesis of Kawasaki Disease

“…The inhibitory effect of protease inhibitor on neutrophilmediated EC injury in KD vasculitis: Although IVIG is the goldstandard therapy for acute KD, CAL formation cannot be completely prevented by IVIG alone [6,7]. Thus, it is believed that intensive initial therapy combined with primary IVIG and other anti-inflammatory drugs may reduce IVIG resistance and CAL formation in high-risk KD patients.…”

“…This disease is well known to be a type of systemic vasculitis, and the most critical complication is the occurrence of a coronary artery lesion (CAL), which may lead to myocardial infarction [5]. Although high-dose intravenous immunoglobulin (IVIG) is the standard initial therapy for acute KD [6,7], approximately 10-20% of KD patients are resistant to IVIG therapy. IVIG-resistant patients are at higher risk of developing CAL than IVIG-responsive patients [6,7].…”

“…Although high-dose intravenous immunoglobulin (IVIG) is the standard initial therapy for acute KD [6,7], approximately 10-20% of KD patients are resistant to IVIG therapy. IVIG-resistant patients are at higher risk of developing CAL than IVIG-responsive patients [6,7]. Approximately 3% of KD patients are reported to have cardiac sequelae, such as coronary dilatation and aneurysm [8].…”