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Kawasaki Disease.
Curr Treat Options Cardiovasc Med. 2007 Apr;9(2):148-158.
Newburger JW, Fulton DR.
Department
of Cardiology, Children's Hospital Boston, 300 Longwood Avenue, Boston,
MA 02115, USA.
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.
Kawasaki
disease is an acute vasculitis of childhood, complicated by coronary
artery aneurysms in up to 25% of affected children. High-dose
intravenous immunoglobulin (IVIG) administered in the acute phase of
Kawasaki disease leads to a fivefold reduction in the prevalence of
coronary aneurysms. When fever persists, a second IVIG infusion should
be administered.
Rescue therapies for IVIG-resistant Kawasaki disease
include pulsed-dose methylprednisolone or other steroid regimens, as
well as infliximab, a tumor necrosis factor-alpha antagonist. Aspirin
is used initially in an anti-inflammatory dosage, then at a low dosage
until approximately 6 weeks after illness onset. Patients with coronary
aneurysms require chronic antithrombotic therapy. For small aneurysms,
aspirin alone is sufficient. For larger aneurysms, agents added to
aspirin include clopidogrel and, for giant aneurysms, warfarin or low
molecular weight heparin. Long-term management is tailored to the
degree of coronary artery involvement. Evidence-based data to guide
indications for transcatheter and surgical intervention are limited.
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