Background Kawasaki Disease (KD) is one of the most common systemic vasculitidies in children today. IVIG is the mainstay of treatment, however, about 1/5 of patients do not respond (Teraguchi) resulting in an increased risk of Coronary Artery Abnormalities (CAA). Based on the current literature and from experience it is our preference to use Intravenous Methylprednisolone (IVMP) in the treatment of IVIG resistant Kawasaki Disease (rKD) patients.
Objectives To examine the differences in CAA seen in patients who were treated with IVMP versus retreatment with IVIG
Methods Demographic, clinical manifestations and echocardiogram data were collected and analyzed. Descriptive statistics were used to describe the data. Chi square/Fisher Exact Test were used to test association, student t test or Mann Whitney U were used to compare the data where appropriate. Kaplan Maier analysis was used to assess the time to CAA resolution. Significance was set at 0.05.
Results Of the 78 rKD patients included (type of KD: complete 55, incomplete 23), 33 received 2nd IVIG and 45 received IVMP. 13 rKD patients who failed the 2nd IVIG treatment were excluded from the analysis (2nd IVIG failure 27.7%). Median age at onset was 1.5 years (IQR 0.76, 2.58) and 45 patients were male. There was no significant difference in regards to gender (p=0.63), age at onset (p=0.14), type of KD (p=0.89) or KD clinical manifestations between treatment groups (p=0.25–0.56). 88.9% of IVMP and 57.6% of 2nd IVIG patients had CAA prior to therapy (p=003). The median duration to completing CAA resolution was 1.19 years; 2nd IVIG group (IQR 0.62, 2.43) and IVMP (IQR 0.84, 3.27). Time to achieve CAA resolution was not different between treatment groups (p=0.11, Figure 1).
Conclusions Between 2nd IVIG and IVMP as rKD rescue therapy, there was no difference in duration of achieving CAA resolution. Our group has described higher number of rKD with CAA prior to IVMP treatment. A larger, prospective randomized controlled trial is needed to confirm our preliminary observations for which this is underway in our region.
Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long term management of Kawasaki disease. Circulation 2004;110:2747–71
Teraguchi M, Ogino H, Yoshimura K, et al. Steroid pulse therapy for children with intravenous immunoglobulin therapy-resistant Kawasaki disease: a prospective study. Pediatr Cardiol. 2013 Apr;34(4):959–63.
Disclosure of Interest None declared