Etiology and pathogenesis of Kawasaki disease

Clinical and epidemiologic features of Kawasaki disease strongly suggest an infectious etiology, but conventional as well as highly sophisticated studies have failed to date to identify one or more causative agents. As with other vasculitides, the pathogenesis of Kawasaki disease is not clearly defined although immunopathologic mechanisms clearly are involved. Marked alterations in T-cells, B-cells, and monocytes/macrophages occur in acute Kawasaki disease and indicate generalized immune activation. In addition, coronary arterial endothelial cells are activated in the acute stage of Kawasaki disease. Future efforts will be directed towards improved understanding of the interrelationships of these observed immune aberrations and to the identification of one or more infectious triggers of the disease process.