The Present and Future
Review Topic of the Week
Kawasaki Disease

https://doi.org/10.1016/j.jacc.2015.12.073Get rights and content
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Abstract

Kawasaki disease is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and children. If not treated early with high-dose intravenous immunoglobulin, 1 in 5 children develop coronary artery aneurysms; this risk is reduced 5-fold if intravenous immunoglobulin is administered within 10 days of fever onset. Coronary artery aneurysms evolve dynamically over time, usually reaching a peak dimension by 6 weeks after illness onset. Almost all the morbidity and mortality occur in patients with giant aneurysms. Risk of myocardial infarction from coronary artery thrombosis is greatest in the first 2 years after illness onset. However, stenosis and occlusion progress over years. Indeed, Kawasaki disease is no longer a rare cause of acute coronary syndrome presenting in young adults. Both coronary artery bypass surgery and percutaneous intervention have been used to treat Kawasaki disease patients who develop myocardial ischemia as a consequence of coronary artery aneurysms and stenosis.

Key Words

aneurysm
coronary aneurysm
coronary thrombosis
immunoglobulin
intravenous
mucocutaneous lymph node syndrome
myocardial infarction

Abbreviations and Acronyms

CAA
coronary artery aneurysm
CABG
coronary artery bypass graft
CMR
cardiac magnetic resonance
CTA
computer tomographic angiography
IL
interleukin
IVIG
intravenous immunoglobulin
KD
Kawasaki disease
LMP
luminal myofibroblastic proliferation
MI
myocardial infarction
PCI
percutaneous coronary intervention
TGFβ
transforming growth factor beta
Th
T helper

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Dr. Newburger was supported by a grant from the McCance Family Fund. Dr. Burns was supported by a grant from the Gordon and Marilyn Macklin Foundation. Dr. Takahashi has reported that he has no relationships relevant to the contents of this paper to disclose.

Listen to this manuscript's audio summary by JACC Editor-in-Chief Dr. Valentin Fuster.