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Kawasaki disease (KD) is the most common form of acquired heart disease in children in
industrialized nations.
KD is a clinical diagnosis in a child with fever to 102°F for at least 5 days who has four of five
additional symptoms: oral mucosal changes; rash; nonpurulent conjunctivitis; extremity changes; and
cervical lymphadenopathy.
Incomplete KD, where children do not meet all the diagnostic criteria, is more common in young
infants, and is a risk factor for the development of coronary artery aneurysms (CAAs).
Current Evidence
KD is a vasculitic condition of unknown etiology that can cause CAAs or ectasia in up to 25% of untreated
children. It is the most common form of acquired heart disease in American children. While first described in
Japanese children, it occurs in children of all races and ethnicities and is most common in infants and preschoolaged children. Older children are also at risk for CAA, as diagnosis is often delayed in this group.
Goals of Treatment
The goal of treatment is to recognize and initiate treatment in children with KD before the 10th day of symptoms,
as delayed treatment increases the risk of CAA development.
Clinical Considerations
Clinical recognition: The symptoms of KD are summarized in Table 94.15 . Incomplete KD, in which a child does
not meet all diagnostic criteria, is more common in infants and rates of CAA are higher in children with
incomplete KD. It is important that clinicians ask all caregivers of children with fever of at least 5 days duration
about KD symptoms. Not all symptoms may be present at the time of ED presentation. The differential diagnosis
of KD is extensive and includes viral infections (especially adenovirus, but can also include EBV, cytomegalovirus
[CMV]), scarlet fever, staphylococcal-scalded skin syndrome, TSS, rickettsial diseases (e.g., RMSF), leptospirosis,
drug hypersensitivity reactions, and some rheumatologic conditions.
Triage considerations: Children with KD can be intravascularly depleted from insensible losses from several
days of high fever. Fluid resuscitation may be needed in the ED. If there is concern for cardiac function based upon
examination (e.g., murmur, hepatomegaly), fluid resuscitation should proceed cautiously and early cardiac imaging
(or a baseline electrocardiogram) should be obtained.