Immunomodulation by intravenous immune globulin in Kawasaki disease

Kawasaki disease is an acute febrile illness of infancy and early childhood. Fifteen percent to 25% of children afflicted with Kawasaki disease develop coronary artery aneurysms. The acute phase of Kawasaki disease is characterized by a deficiency of suppressor T cells and the marked activation of T cells, B cells, and monocytes associated with increased secretion of cytokines by these immune effector cells. Evidence that this immune activation contributes to vascular endothelial cell damage in Kawasaki disease is supported by the observation that patients in the acute phase of Kawasaki disease have circulating antibodies lytic for vascular endothelial cells activated with gamma-interferon, IL-1, or tumor necrosis factor. In contrast, sera from these patients do not lyse unstimulated endothelial cells. High-dose intravenous immune globulin treatment is effective in preventing the occurrence of coronary artery abnormalities in Kawasaki disease. Patients treated with intravenous immune globulin have a significant increase in T suppressor cells, a decrease in circulating T helper cells, and a decrease in spontaneous IgG and IgM synthesis. These observations suggest that intravenous immune globulin reduces the vasculitis in Kawasaki disease by suppressing the marked immune activation associated with this disease.