vessel vasculitis, one of two primary
predominantly affect children
purpura/IgA vasculitis).
years of age with an incidence in the US of
diabetes.
a low rate of mortality (approximately 0.08%
most common cause of acquired cardiac
and 5% of treated cases developing coronary
of a coronary artery aneurysm is 50% even in
immunoglobulin (IVIG) within the first 10 days of
guidance regarding diagnostic strategies,
role of echocardiography for patients
Definitions and Medications
Table 1. Definitions for Selected Terms Used in the
Recommendations for KD
Term Definition
A. Disease states
KD Fever lasting at least five days without any other explanation with
at least four of the five following principal clinical findings:
• Bilateral bulbar conjunctival injection without exudate
• Erythema and cracking of lips, strawberry tongue, and/or
erythema of oral and pharyngeal mucosa
• Erythema and edema of the hands or feet (acute phase),
and/or periungual desquamation (subacute phase)
• Maculopapular, diffuse erythroderma, or erythema
multiforme-like rash
• Cervical lymphadenopathy (at least one lymph node >1.5 cm
in diameter), usually unilateral
e diagnosis may be made with only 4 days of fever if ≥4
principal clinical findings are present.
Incomplete KD
Prolonged unexplained fever in an infant or child with <4 of the
principal clinical findings of KD (see above), and compatible
laboratory studies (elevated erythrocyte sedimentation rate
[ESR]/ C-reactive protein [CRP], elevated transaminases,
urinalysis [UA] with leukocyte esterase negative white blood
cell [WBC]) or echocardiographic findings (coronary artery
dilatation).
a
Acute KD Initial febrile phase of KD.
B. Medications
IVIG Single dose of 2 g/kg (no defined maximum dose, however
reasonable maximum ranges are 100–140 g ).
Glucocorticoids (GC) Prednisone 2 mg/kg/day (maximum dose 60 mg/day) for 15
days, or equivalent.
Non-GC
immunomodulatory
therapy
Anakinra, cyclosporine, or infliximab.
Aspirin High dose: 80-100 mg/kg/day.
Moderate dose: 30–50 mg/kg/day.
Low dose: 3–5 mg/kg/day.
a
Defined according to algorithm in Burns, J.C. Pediatrics, 2010. 125(2): p. E234–E241 and McCrindle,
B.W., et al. Circulation, 2017. 135(17): p. e927–e999.
