Clinical and immunologic aspects of Kawasaki disease.

Kawasaki disease (KD) is an acute febrile disease of infancy and early childhood characterized by diffuse vasculitis. Although the disease is generally self-limited, 15-25% of children with KD may develop coronary artery aneurysms, and sudden death due to cardiovascular complications can occur. The acute phase of KD is characterized by marked activation of the immune system, increased cytokine production by immune effector cells, and the generation of cytotoxic antibodies directed against vascular endothelial cells stimulated with cytokines. High-dose intravenous gammaglobulin (IVGG) treatment is effective in preventing the occurrence of coronary artery disease in KD. Treatment of patients with IVGG results in a significant increase in circulating suppressor T cells, a decrease in circulating activated helper T cells, and a decrease in spontaneous IgG and IgM synthesis. These observations suggest that IVGG reduces the vascular injury in KD by suppressing the marked immune activation associated with this disease.