Key Points
` Kawasaki disease (KD) is a medium vessel vasculitis, one of two primary
common vasculitis syndromes that predominantly affect children
(the other being Henoch-Schönlein purpura/IgA vasculitis).
• KD typically presents in children under 5 years of age with an incidence in the US of
25–50/100,000, similar to that of type 1 diabetes.
• KD treated appropriately is associated with a low rate of mortality (approximately 0.08%
case fatality rate in the largest series).
• However, in the developed world, KD is the most common cause of acquired cardiac
disease in childhood, with 25% of untreated and 5% of treated cases developing coronary
artery aneurysms.
• In infants less than 6 months of age, the risk of a coronary artery aneurysm is 50% even in
KD patients treated with intravenous immunoglobulin (IVIG) within the first 10 days of
illness.
` These recommendations provide guidance regarding diagnostic strategies,
use of pharmacologic agents, and role of echocardiography for patients
with suspected or confirmed KD.
Definitions and Medications
Table 1. Definitions for Selected
Recommendations for
Term Definition
A. Disease states
KD Fever lasting at least
at least four of the
• Bilateral bulbar
• Erythema and
erythema of oral
• Erythema and
and/or periungual
• Maculopapular,
multiforme-like
• Cervical lymphadenopathy
in diameter), usually
e diagnosis may
principal clinical
Incomplete KD
Prolonged unexplained
principal clinical
laboratory studies
[ESR]/ C-reactive
urinalysis [UA] with
cell [WBC]) or
dilatation).
a
Acute KD Initial febrile phase
B. Medications
IVIG Single dose of 2
reasonable maximum
Glucocorticoids (GC) Prednisone 2 mg/kg/day
days, or equivalent.
Non-GC
immunomodulatory
therapy
Anakinra, cyclosporine,
Aspirin High dose: 80-100
Moderate dose:
Low dose: 3–5 mg/kg/day.
a
Defined according to algorithm in Burns, J.C. Pediatrics,
B.W., et al. Circulation, 2017. 135(17): p. e927–e999.
