Background: Amelioration of systemic inflammatory changes during the acute phase of Kawasaki Disease (KD) is critical for preventing coronary artery lesions (CALs). Plasma exchange (PE) therapy has been applied to patients with severe KD in several medical facilities in Japan and been covered by health insurance since 2012.
Methods: We reviewed medical records of five KD patients, aged 3 to 44 months, who were treated with PE in Nagasaki University Hospital from December 2012 to August 2015.
Results: All patient were treated with two courses of intravenous immunoglobulin (IVIG). After IVIG that had offered little or no improvement, two of the five patients used infliximab and another used cyclosporine A before PE. PE was performed for the consecutive 3 or 5 days until defervescence and clinical improvement were obtained. Acute inflammatory changes were successfully ameliorated upon PE therapy in all five patients, although two patients who had already developed CALs before PE on days 20 and 21, respectively, residual CALs were confirmed one year after the treatment. No CALs had developed in the remaining three patients who started PE therapy within nine days after onset (days 8, 8 and 9, respectively). PE therapy was carefully performed in the intensive care unit, and no severe adverse events occurred.
Discussion: PE therapy is able to ameliorate acute inflammation in most cases of severe KD refractory to standard IVIG therapy; however, it is critical to start PE therapy earlier than day-10 in order to prevent CALs. Thus, pediatricians have to make prompt decision on referral of their KD patient who did not respond IVIG to the institutions where PE is applicable in Japan.