Immunological abnormalities and endothelial cell injury in Kawasaki disease.

Kawasaki disease is an acute febrile disease causing vasculitis, which may lead to as severe a complication as myocardial infarction. This disease is characterized by marked activation of the immune system, including elevation of various cytokines, polyclonal B cell activation, and decrease of CD8+ cells. The high levels of cytokines induce adhesion molecules and a new antigen on endothelial cells. As a result, the immune cells bind to the endothelium and the activated immune cells produce cytotoxic antibodies against the new antigen on the endothelial cells. Thus, the activated immune system is involved in the endothelial cell injury. IVIG (intravenous immunoglobulin) therapy suppresses the activated immune system and reduces the endothelial cell damage.