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Recurrent Kawasaki disease and cardiac complications: nationwide surveys in Japan
  1. Daisuke Sudo,
  2. Nobuko Makino,
  3. Yosikazu Nakamura
  1. Department of Public Health, Jichi Medical University, Shimotsuke, Japan
  1. Correspondence to Dr Daisuke Sudo, Public Health, Jichi Medical University, Shimotsuke 329-0498, Japan; m05049ds{at}


Introduction Based on data obtained before high-dose (2 g/kg) intravenous immunoglobulin (IVIG) therapy prevailed in Japan, children with a history of Kawasaki disease (KD) were highly susceptible to disease recurrence and more likely to develop cardiac sequelae. We aimed to examine the epidemiological features of cardiac complications among patients with recurrent KD following the widespread use of high-dose IVIG therapy.

Design Two cohorts of patients with recurrent KD retrieved from Japanese nationwide surveys (previous cohort: 1989–1994; recent cohort: 2003–2012) were compared.

Results Of 1842 patients with recurrent KD in the recent cohort, 3.5% and 5.2% developed cardiac sequelae at the initial and second episodes, respectively, which were markedly decreased compared with those (>10%, respectively) in the previous cohort. Multivariate analyses showed that the risk factors for cardiac sequelae at the second episode were similar between the cohorts. Patients with recurrent KD in both cohorts were more likely to have coronary aneurysms at the second episode than at the initial episode. However, when patients with coronary aneurysms at the initial episode were excluded from analyses, the difference in the proportions of coronary aneurysms between KD episodes disappeared in the recent cohort. Residual rates of previously formed coronary aneurysms were similar between the cohorts (approximately 50%).

Conclusion This study suggests that KD recurrence is no longer a risk factor for developing cardiac complications, unless cardiac sequelae appear at the initial episode. However, residual rates of previously formed coronary aneurysms remain high. Therefore, the importance of carefully managing coronary aneurysms associated with KD remains unchanged.

  • Kawasaki disease
  • epidemiology
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  • Contributors DS designed the study, conducted the analyses, drafted the initial manuscript and finalised the manuscript. NM enriched the discussions. YN conceptualised the study and supervised the analyses. NM and YN critically reviewed the manuscript. All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.

  • Funding This study was partly financially supported by the non-profit organisation Japan Kawasaki Disease Research Center.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information. Nationwide epidemiological surveys of Kawasaki disease have been conducted every 2 years since 1970. All data sets are managed by the Department of Public Health at Jichi Medical University.

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