Abstract 123: Long term Prognosis After Methylprednisolon pulse Combination Therapy For Refractory Kawasaki Disease
Background: We previously investigated the molecular mechanism and the clinical utility of intravenous methylprednisolone-pulse(IVMP) plus intravenous immunoglobulin (IVIG) combination therapy (IVMP+IVIG) for patients with Kawasaki disease (KD) in the acute phase (Pediatr Res 2009, Pediatrics 2012). However, the long-term prognosis after IVMP+IVIG still remains unclear.
Objective: To examine the long-term prognosis after IVIG+IVMP.
Methods: Forty-six patients (Age: 5-70 months, male: 27) who were enrolled in this study were treated with IVMP+IVIG during the period from 2007 to 2014 at Kitasato University Hospital who were all predicted to show resistance to initial IVIG according to their Egami score (J Pediatr 2006). The IVMP therapy (30mg/kg, 1dose) was administered for 2 hours before starting the IVIG therapy. Heparin (10U/kg/h, continuous infusion) was used concomitantly from 2 hours before the start of IVMP therapy and it was thereafter continued for 24 hours. The long-term prognosis of coronary artery lesions (CALs) and adverse events between the time of diagnosis and 5 years after IVMP +IVIG were determined. The “Z-score2.5-5” and “Z–score>5” were defined as dilatation and an aneurysm, respectively, according to the AHA guidelines.
Results: Dilatation cases based on the Z-score2.5-5, were identified in 15 of 46 patients (32.6%) at diagnosis, in 15 of 46 patients (32.6%) after 1 month, and in 3 of 36 patients (8.3%) after 1 year. All patients in whom coronary artery abnormalities were observed at 1 year later demonstrated regression.No patients showed a Z-score≧5 in all phases. Thirty-six patients (78.2%) showed prompt defervescence owing to the combination therapy. The patients had adverse event at acute phase, 16 of 46 patients (34.7%) for hypothermia, 5 patients (10.8%) for hypertension, 2 patients (4.3%) for bradycardia, and 4 patients (8.6%) for recurrent fever. No patients experienced any serious adverse events (short stature, hyperlipidemia, abnormal glucose tolerance) after IVMP +IVIG during the long-term follow-up.
Conclusions: This study showed IVMP+IVIG combination therapy to therefore effectively prevent CALs in KD patients that are predicted to be resistant to intial IVIG according to their Egami score.
Author Disclosures: T. Ebato: None. Y. Ogihara: None. M. Takanashi: None. T. Honda: None. S. Kimura: None. S. Ogata: None. M. Ishii: None.
- © 2015 by American Heart Association, Inc.