Leung D Y
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA.
Int Rev Immunol. 1989;5(2):197-202. doi: 10.3109/08830188909061987.
Kawasaki disease (KD) is an acute febrile illness of early childhood that is associated with the development of coronary artery aneurysms in 15-25% of the cases. The acute phase of KD is characterized by a deficiency of suppressor T cells, marked activation of the immune system and increased secretion of cytokines by immune effector cells. Evidence that this immune activation contributes to the vascular endothelial cell damage in KD is suggested by the observation that patients in the acute phase of KD 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 gammaglobulin (IVGG) treatment is effective in preventing the occurrence of coronary artery abnormalities in KD. Patients treated with IVGG have a significant increase in T suppressor cells, a decrease in circulating activated T helper cells, and a decrease in spontaneous IgG and IgM synthesis. These observations suggest that IVGG reduces the vasculitis in KD by suppressing the marked immune activation associated with this disease.
川崎病（KD）是一种幼儿期急性发热性疾病，15%至25%的病例会并发冠状动脉瘤。KD急性期的特征是抑制性T细胞缺乏、免疫系统显著激活以及免疫效应细胞分泌细胞因子增加。KD急性期患者循环中存在可裂解经γ干扰素、白细胞介素-1或肿瘤坏死因子激活的血管内皮细胞的抗体，这一观察结果提示这种免疫激活与KD的血管内皮细胞损伤有关。相比之下，这些患者的血清不能裂解未受刺激的内皮细胞。大剂量静脉注射丙种球蛋白（IVGG）治疗可有效预防KD患者冠状动脉异常的发生。接受IVGG治疗的患者中，抑制性T细胞显著增加，循环中活化的辅助性T细胞减少，自发性IgG和IgM合成减少。这些观察结果表明，IVGG通过抑制与该疾病相关的显著免疫激活来减轻KD中的血管炎。
