Immunoregulation in Acute Kawasaki Disease

Kawasaki disease (KD) is an acute illness of early childhood characterized by prolonged fever, diffuse mucosal inflammation, indurative edema of the hands and feet, polymorphous skin rash and non-suppurative Iymphadenopathy. Histopathological findings in KD indicate a panvasculitis with endothelial necrosis, and the infiltration of mononuclear cells into small and medium-sized blood vessels. Central to the development of coronary artery lesions (CAL) may be the expression of adhesion molecules on vascular endothelial cells, which is induced by tumor necrosis factor α(TNFα) secreted by the increased numbers of activated CD14+ macrophages /monocytes circulating during the acute phase of KD. In addition, activated T-cells are temporarily withdrawn from peripheral circulation during acute KD, and sequestration of activated T-cells may be a feature of this disease. Although the incidence of CAL in KD has declined since the routine application of intravenous infusions of gamma globulin treatment, it still develops in approximately 10% of patients with KD in Japan. Pentoxifylline blocks TNFα production in lipopolysaccharide-treated macrophages and in endotoxin-treated human volunteers, and has been used for treating diseases with the increased TNFα production. We believe that oral administration of high-dose pentoxifylline and intravenous infusions of gamma globulin combination therapy is safe and effective in reducing the incidence of CAL when administered early in the course of KD.

川崎病

冠動脈病変

免疫担当細胞

サイトカイン

サイトカイン抑制療法