Abstract 9271: Significance of Primary Therapy With Intravenous Immunoglobulin Plus Prednisolone For Severe Kawasaki Disease: Result From Japanese Multicenter Randomized Clinical Trial.

Abstract

Background Recent re-assessment reports have suggested that primary corticosteroid therapy for severe Kawasaki disease patients might be beneficial. Methods We conducted a multicenter, prospective, randomized, open, blinded-endpoints, and parallel-group study to determine whether the addition of prednisolone to conventional intravenous immunoglobulin therapy for severe Kawasaki disease reduces the risk of coronary artery abnormality. Between September 2008 and December 2010, 248 severe Kawasaki disease patients with risk score ≥5 points were randomly assigned to receive either intravenous immunoglobulin (IVIG group; n = 123) or intravenous immunoglobulin plus prednisolone (IVIG+PSL group; n = 125). The primary endpoint was presence of coronary artery abnormality during study period. Secondary endpoints included incidence of coronary artery abnormality at week 4, Z score of coronary arteries, duration of fever after enrollment, need for additional rescue therapy, serum levels of C-reactive protein (CRP) at weeks 1 and 2 after enrollment, and severe adverse events. Results Incidence of coronary artery abnormality during study periodt was significantly lower in the IVIG+PSL group than in the IVIG group (3% vs 23%, respectively; P<0.001). Similarly, incidence of coronary artery abnormality at week 4 after enrollment was significantly lower in the IVIG+PSL group than in the IVIG group (3% vs 13%, respectively; P=0.014). Furthermore, IVIG+PSL group had shorter duration of fever after enrollment (P<0.001), lower risk of need for additional rescue therapy (P<0.001), and lower CRP level at weeks 1 and 2 (both P<0.001) compared with IVIG group. Incidence of severe adverse events was similar in the two groups. Conclusions Intravenous immunoglobulin plus prednisolone is superior to intravenous immunoglobulin alone to prevent coronary artery abnormality in patients with severe Kawasaki disease. (UMIN-CTR Number, UMIN000000940.)