Abstract
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.
Highlights
Kawasaki disease (KD) is a vasculitis syndrome that frequently develops coronary artery lesions (CALs) [1]
We summarize the pathophysiology of KD and plasma exchange, which is a therapeutic method that can calm the hyper-cytokine state of this disease
Joh was the first to mention the possibility of plasma exchange (PE) for Kawasaki disease [12], and Takagi et al reported that a 4-year-old girl who was refractory to intravenous immuno-globulin (IVIG) was treated with PE for 3 days and that the inflammation was successfully suppressed with no coronary complications [13]
Summary
Kawasaki disease (KD) is a vasculitis syndrome that frequently develops coronary artery lesions (CALs) [1]. In this disease, it is necessary to suppress inflammation within 10 days of onset in order to suppress CALs caused by a large amount of persistent inflammatory cytokines. The results of the 25th nationwide survey showed an extremely high incidence rate of 359.1 per 100,000 population aged 0–4 years in 2018, and the annual number of patients reached a record high of 17,364 [2].
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