Mori Masaaki, Yamazaki Susumu, Naruto Takuya
Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo 113-8510, Japan.
J Clin Med. 2021 Mar 4;10(5):1062. doi: 10.3390/jcm10051062.
Kawasaki disease (KD) is a vasculitis syndrome that frequently develops coronary artery lesions (CALs). In the treatment of KD, the utility of high-dose intravenous immuno-globulin (IVIG) therapy has already been clarified, and it has been established as the first-line treatment method. However, since approximately 10% of patients are refractory to this IVIG therapy and 2.6% of all patients have coronary sequelae, 500 children with KD still remain every year in Japan. In this disease, it is necessary to calm inflammation within 10 days of onset in order to suppress CALs caused by a large amount of persistent inflammatory cytokines. Indeed, the early suppression of inflammation is an effective means of suppressing the onset of CALs. Here, we describe the pathophysiology of Kawasaki disease and plasma exchange (PE), which is a therapeutic method that can calm the hyper-cytokine state of this disease. The treatment result of PE for IVIG-refractory Kawasaki disease is outstanding, and an extremely large effect can be expected if it can be started before the appearance of CALs. It seems that it should always be considered as one of the powerful additional treatments in the future.
川崎病（KD）是一种常引发冠状动脉病变（CALs）的血管炎综合征。在KD的治疗中，大剂量静脉注射免疫球蛋白（IVIG）疗法的效用已得到明确，并且已被确立为一线治疗方法。然而，由于约10%的患者对这种IVIG疗法无效，且所有患者中有2.6%会出现冠状动脉后遗症，日本每年仍有500名KD患儿。在这种疾病中，有必要在发病10天内控制炎症，以抑制由大量持续存在的炎性细胞因子引起的CALs。事实上，早期控制炎症是抑制CALs发生的有效手段。在此，我们描述川崎病的病理生理学以及血浆置换（PE），这是一种能够缓解该疾病高细胞因子状态的治疗方法。PE对IVIG难治性川崎病的治疗效果显著，如果能在CALs出现之前开始治疗，有望产生极大的效果。在未来，它似乎应始终被视为强有力的辅助治疗方法之一。
