AB0928 CORONARY ARTERY LESIONS AT PRESENTATION IN KAWASAKI DISEASE: A MARKER OF IVIG RESISTANCE?A RETROSPECTIVE STUDY FROM NORTH INDIA

ANNALS OF THE RHEUMATIC DISEASES(2019)

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摘要
Background Kawasaki disease has been well described from all around the world.This has been well described from our country. We recently changed our practice with regards to addition of corticosteroids and undertook this study. Objectives To study the clinical profile of children with Kawasaki disease presenting to our unit. To study the response of therapy in children with Kawasaki disease To study the efficacy of addition of upfront steroids in children with coronary artery lesions (z score>2.5) Methods All children with Kawasaki disease seen in the last 5 years were included and data collected on prefilled proforma. IVIG 2gm/kg as a single dose over 18-24 hours was given to all children. Aspirin in dose of 30-80mg/kg/day was given to all children along with IVIG. Response was defined as defervescence within 36 hours of completion of IVIG. Fever beyond 36 hours of completion of IVIG was considered as no response/resistance to 1st dose of IVIG. Results 60 children diagnosed with Kawasaki disease were included. Median age at diagnosis of disease was 3.12 years(0.3-15 years).33 children had complete Kawasaki disease, 26 children had incomplete disease and one child had atypical Kawasaki disease36 children were diagnosed within first 10 days of disease onset and 24 children were diagnosed after day 10 of illness.18 children had history of infections prior to onset of Kawasaki disease.29 children(48%) had coronary artery lesions at the time of presentation.48 children(80%) were given1st dose of IVIG without any corticosteroids.36 children (75%)responded to first dose of IVIG, one child was not given IVIG as he was diagnosed retrospectively and had self limiting disease. 14 children(29%) did not respond to 1st dose of IVIG. 9 children were given intravenous methylprednisolone 2mg/kg/day in four divided doses along with IVIG. All of these responded to the first dose of IVIG. The size of coronary artery lesion also plays an important role. In children with z score >2.5 the response to 1st dose of IVIG was 61.5% whereas it was 94%in those who had z scores Response rate was significantly higher in patients with no coronary artery lesions (p value0.007).In children with coronary artery lesions at presentation, the response rate was significantly higher in children who were given steroids upfront (p value 0.027). Conclusion We see many children with coronary artery lesions at presentation. This seems to be an indicator of resistance to IVIG. It is beneficial to add steroids upfront along with IVIG to children with coronary artery lesions more than 2.5 z score. Reference [1] Dallaire F, Dahdah N. New equations and a critical appraisal of coronary artery Z scores in healthy children. J Am Soc Echocardiogr. 2011Jan;24(1):60-74. Disclosure of Interests None declared
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