Abstract 131: Retrospective Study of The Timing Of Additional Therapy For Severe Kawasaki Disease Before And After The Induction Of Raise Study Protocol

Abstract

Background: The RAISE study showed that combination therapy with intravenous immunoglobulin (IVIg) and prednisolone (PSL) improved coronary artery outcomes in patients with severe Kawasaki disease (KD). We encountered KD cases in which fever and findings such as WBC were masked by the steroid administration in combination therapy. We hypothesized that combination therapy might delay second- and third-line therapy for severe KD. Purpose: The goal of the study was to investigate whether combination therapy with IVIg and PSL might delay second- and third-line therapy for severe KD patients. Methods: We retrospectively investigated clinical data from acute KD patients admitted to our hospital from January 2011 to September 2014. We examined the following: 1) prediction score for refractory KD 2) first-line therapy for KD (IVIg group, n=10; IVIg/PSL group, n=11); 3) additional rescue therapy such as additional IVIg, steroid pulse therapy, plasma exchange, and so on 4) period from KD onset to end of main therapy for KD; 5) day of increased albumin from previous examination; and 6) day of CRP reaching <1.0 mg/l. Results: Seventy-four acute KD patients were admitted to our hospital during the study period. Only 1 KD patient had coronary artery lesions (CAL) at 1 month after onset. Detailed data were as follows: 1) Twenty-one patients were at high risk for refractory KD. 2) number of patients with additional IVIg (IVIg, 6/10; IVIg/PSL, 6/11); 3) number of patients with steroid pulse therapy (IVIg, 4/10; IVIg/PSL, 4/11); 4) period from KD onset to end of main therapy (IVIg, 8.3 days; IVIg/PSL, 8.7 days); 5) day of increased albumin (IVIg, 12.0 days; IVIg/PSL, 11.8 days); 6) day of CRP reaching <1.0 mg/l (IVIg, 13.7 days; IVIg/PSL, 11.4 days). No differences between IVIg and IVIg/PSL groups were seen for any factor. Discussion and conclusion: In this study, overall frequency of CAL was 1.3%, lower than that reported from nationwide surveillance in Japan. This study found no differences between groups in regard to frequency or timing of additional therapy and response. We concluded that combination therapy with IVIg and PSL did not delay the timing of additional therapy for acute KD. The cohort for this retrospective study was small, and further investigation is thus needed.