Paediatric Vasculatides- Kawasaki

Question 1

What is Kawasaki disease

Answer 1

acute, necrotizing vasculitis of unknown etiology.

condition primarily affects children under the age of five and is more common among those of Asian descent

Sex: ♂ > ♀
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% D/2 MI/stroke

Question 2

Clinical

Feature

Answer 2

fever for at least 5 days that’s u responsive to abiotic and:
≥ 4 other specific symptoms, or
< 4 specific symptoms if the coronary arteries are involved

Specific symptoms

Erythema and edema of hands, feet,palms and soles (the first week)

desquamation of fingertips and toes (after 2–3 weeks)

Polymorphous rash, originating on the trunk

Painless bilateral “injected” conjunctivitis without exudate

Erythema and swelling of the tongue (strawberry tongue)

Cracked and red lips

Cervical lymphadenopathy (mostly unilateral)

Question 3

Dg of Kawasaki

Answer 3

Mainly based on clinical findings

Laboratory findings:
↑ ESR and CRP
Leukocytosis
Thrombocytosis 
Anti-endothelial cell antibodies (AECA)

Echocardiography: for evaluating coronary artery aneurysms

Question 4

Rx of Kawasaki

Answer 4

1)IV immunoglobulin (IVIG)
high single-dose
reduce the risk of coronary artery aneurysms
most effective within the first 10 days following onset

High-dose oral aspirin (To avoid the risk of Reye syndrome, children should not be treated with aspirin, especially if a viral infection is suspected. Kawasaki disease is an exception to this rule!)

2)IV glucocorticoids: may be considered in addition to standard treatment in high-risk patients
lower the risk of coronary involvement;
in cases of treatment-refractory disease

Question 5

Complications of Kawasaki

Answer 5

Coronary artery aneurysm: highest during the 2nd–3rd week after symptom onset

Myocardial infarction

Myocarditis

Arrhythmias