Kawasaki disease is an acute
vasculitis of unknown cause that occurs predominantly in infants and young children and produces coronary artery
aneurysms in approximately 15% to 25% of those affected. In the United States,
Kawasaki disease is more commonly the cause of noncongenital
heart disease in children than is
acute rheumatic fever.
Therapy for
Kawasaki disease in the acute phase is aimed at reducing
inflammation of the coronary artery wall and preventing
coronary thrombosis; treatment with high-dose intravenous
gamma globulin and
aspirin has become the standard of care and reduces the risk of development of coronary artery
aneurysms by three- to fivefold. Even when treated with high-dose
intravenous immune globulin G (
IVIG) regimens within the first 10 days of illness, however, approximately 5% of children with
Kawasaki disease develop at least transient coronary artery dilation, and 1% develop giant
aneurysms. For those with persistent or recrudescent
fever despite initial
IVIG infusion, multiple courses of
gamma globulin and treatment with cortico-
steroids may be indicated. Early experience suggests that
therapies aimed at reducing the amount of
tumor necrosis factor alpha have a role. For those who develop coronary artery
aneurysms, chronic antithrombotic regimens are instituted. When
aneurysms are small or moderate in size,
aspirin alone may be sufficient, but for patients with giant
aneurysms, most experts choose to treat
Kawasaki disease with
aspirin plus
warfarin. Acute
coronary thromboses are treated with platelet IIb/IIIa antagonists and
thrombolytic therapy. For children with
coronary artery stenoses and consequent
ischemic heart disease, the therapeutic armamentarium is similar to that used in adults with atherosclerotic
coronary artery disease and includes
coronary artery bypass grafting and transcatheter interventions.