In this section
KD is the second most common vasculitis in childhood after Henoch Schonlein purpura, and is the most commoncause of acquired heart disease in children in developed countries causing coronary artery aneurysms (CAA).
It has a worldwide distribution, although is more common in Asian children.
Approximately 85% of cases occur under 5 years of age, peak age 18-24 months
The disease often presents without all diagnostic criteria (see below) and can present a significant diagnostic challenge to the clinician.
Fever persisting for at least 5 days, PLUS 4 of the 5 criteria:
NB. KD can be diagnosed with less than four of the following features if coronary artery abnormalities are present.
Polymorphous; without vesicles, bullae or crusts; occurring in the first few days, involves the trunk and extremities.
Variable presentations such as urticarial, morbilliform, maculopapular, or resembling scarlet fever.
Lips and oral mucosa
Hyperaemia and painful oedema of hands and feet that progresses to desquamation in the convalescent stage.
Perineal desquamation frequently associated.
Irritability is an important sign and one that is very frequently present, although not included as a diagnostic criterion.
Diagnostic features may present sequentially. KD should be considered in any child with fever, rash and evidence of systemic inflammation.
Common findings outside the diagnostic criteria include arthritis, aseptic meningitis, sterile pyuria and dysuria.
It is important to recognise the potential for missed diagnosis in infants:
Although nonspecific, laboratory tests provide support for diagnosis, assessment of severity and monitoring of disease and treatment.
Common abnormalities include elevation of ESR, CRP and WCC. Thrombocytosis is common in the second week of illness.
Incomplete Kawasaki Disease:
Prednisolone 2mg/kg (max 60mg) orally daily for a minimum of 5 days and until CRP normalises. (Evidence for optimal dose/duration is limited)
A steroid course of >10 days will require weaning and consideration of concurrent proton pump inhibitor or H2 receptor blocker.
ALL children with diagnosed or suspected
Kawasaki Disease should be discussed with the local paediatric unit and
For emergency advice and paediatric or neonatal ICU transfers, call the Paediatric Infant Perinatal Emergency Retrieval (PIPER) Service: 1300 137 650.
Early referral to Cardiology for consideration of echocardiography is advised.
Last updated August, 2017