- Clinical science
Kawasaki disease is an acute, necrotizing vasculitis of unknown etiology. The condition primarily affects children under the age of five and is more common among those of Asian descent. The disease is characterized by a high fever, desquamative rash, conjunctivitis, mucositis (e.g., “strawberry tongue”), cervical lymphadenopathy, as well as erythema and edema of the distal extremities. However, coronary artery aneurysms are the most concerning possible manifestation as they can lead to myocardial infarction or arrhythmias. Kawasaki disease is a clinical diagnosis, further supported by findings such as elevated ESR or evidence of cardiac involvement on echocardiography. Treatment with IV immunoglobulins and high-dose aspirin is essential and should be initiated immediately after diagnosis.
- Sex: ♂ > ♀ (1.5:1)
- Age: primarily children < 5 years
- Peak incidence: occurs mostly in late winter and spring
- Prevalence: : approx. 20 per 100,000 children; highest rate in children of Asian and Pacific-Islander descent
- Mortality: approx. 1%
Epidemiological data refers to the US, unless otherwise specified.
- The exact cause of Kawasaki disease remains uncertain.
- However, it is associated with infectious and genetic factors: the prevalence is higher in patients of Asian descent and in siblings of affected children.
- Clinical diagnosis requires fever for at least 5 days and:
- ≥ 4 other specific symptoms, or
- < 4 specific symptoms if the coronary arteries are involved
Erythema and edema of hands and feet, including the palms and soles (the first week)
- Possible desquamation of fingertips and toes (after 2–3 weeks)
- Polymorphous rash, originating on the trunk
- Painless bilateral “injected” conjunctivitis without exudate
- Oropharyngeal mucositis
- Cervical lymphadenopathy (mostly unilateral)
- Erythema and edema of hands and feet, including the palms and soles (the first week)
- Nonspecific symptoms may precede the onset of Kawasaki disease (e.g., diarrhea, fatigue, abdominal pain)
Always consider Kawasaki disease in small children with a rash and high fever unresponsive to antibiotics!
Think of “CRASH and BURN” to remember the clinical features of Kawasaki disease: C = Conjunctivitis, R = Rash, A = Adenopathy, S = Strawberry tongue, H = Hands and feet, BURN = fever ≥ 5 days.
Kawasaki disease is a clinical diagnosis (see “Clinical features” above) which is supported by the following diagnostic findings:
- Laboratory findings
Echocardiography: for evaluating coronary artery aneurysms
- Minimal evaluation: should be performed at diagnosis, at 2 weeks, and at 6–8 weeks after onset
- IV immunoglobulin (IVIG)
- High-dose oral aspirin
- IV glucocorticoids: may be considered in addition to standard treatment in high-risk patients, as they lower the risk of coronary involvement; also in cases of treatment-refractory disease
To avoid the risk of aspirin, especially if a viral infection is suspected. Kawasaki disease is an exception to this rule!, children should not be treated with References: