Effect of Plasma Exchange in Kawasaki Disease Refractory to High Dose Intravenous Gammaglobulin Therapy

Abstract

Background: In Kawasaki disease (KD), administration of intravenous gammma-globulin (IVGG) reduced frequency of coronary artery lesion (CAL) from 25% to 8%. However, approximately 500 children still develop CAL every year in Japan. We evaluated the efficacy and safety of plasma exchange (PE) for children with KD intractable to IVGG therapy. Patients and Methods: Among 255 children with KD treated with initial IVGG, 119 children were identified at high risk for CAL according to the increases in fractional changes (FC) of white blood cell count, neutrophils, and CRP between baseline and 1 to 2 days after IVGG treatment. Seventy-five were administered additional IVGG, and finally 50 of them were underwent the PE therapy in 3 consecutive days. All the children were serially monitored for the development of CAL by echocardiography during the course of the disease until days 28-30. The incidence of CAL was compared among the children treated and those untreated with PE. Results: The demographic differences between the treated and untreated children were not significant on admission. The PE therapy was safely performed without any adverse reactions, and has completed in each child. Out of 50 children treated PE, only 10 (20.0%) had CAL, including temporary dilatation in 7, persistent CAL in 1, and giant aneurysms in 2 children. In contrast, 24 children (40.7%) among 69 without PE were affected with CAL, including giant aneurysm in 5 (P < 0.0004). Conclusions: We suggested that PE therapy performed within 10 days after the onset of the disease should be effective and safe for the children with KD intractable to the IVGG therapy. The PE therapy may be applied as soon as possible when the fractional increases in inflammatory markers after the initial IVGG are determined.