Kawasaki disease is an acute vasculitis of infancy and early childhood. Left untreated, up to 25percnt; of children with Kawasaki disease develop coronary artery abnormalities. Recent studies indicate that patients with Kawasaki disease have elevated cytokine production and increased expression of cytokine-inducible activation antigens on their vascular endothelium. Treatment with intravenous gammaglobulin significantly reduces the incidence of coronary artery abnormalities in this disease. Furthermore, patients who respond clinically to intravenous gammaglobulin demonstrate a marked reduction in immune activation. These recent insights into the immunology and treatment of Kawasaki disease may eventually provide new directions for research into the etiology of this fascinating disease.
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