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The etiologies of Kawasaki disease
Jane C. Burns
Jane C. Burns
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Review

The etiologies of Kawasaki disease

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Abstract

Kawasaki disease (KD) is a systemic vasculitis that affects young children and can result in coronary artery aneurysms. The etiology is currently unknown, but new clues from the epidemiology of KD in Japan, the country of highest incidence, are beginning to shed light on what may trigger this acute inflammatory condition. Additional clues from the global changes in KD incidence during the COVID-19 pandemic, coupled with a new birth cohort study from Japan, point to the potential role of person-to-person transmission of an infectious agent. However, the rising incidence of KD in Japan, with coherent waves across the entire country, points to an increasing intensity of exposure that cannot be explained by person-to-person spread. This Review discusses new and historical observations that guide us toward a better understanding of KD etiology and explores hypotheses and interpretations that can provide direction for future investigations. Once the etiology of KD is determined, accurate diagnostic tests will become available, and new, less expensive, and more effective targeted therapies will likely be possible. Clearly, solving the mystery of the etiologies of KD remains a priority for pediatric research.

Authors

Jane C. Burns

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Figure 1

Coronary artery damage from KD.

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Coronary artery damage from KD.
Illustrations represent giant aneurysms ...
Illustrations represent giant aneurysms of the right and left anterior descending coronary arteries. IVIG therapy can reduce the prevalence of coronary artery aneurysm following acute KD from 25% to 5%, highlighting the need for accurate diagnostic tests and timely treatment.

Copyright © 2026 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

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