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Kawasaki Disease

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Key Points ` Kawasaki disease (KD) is a medium vessel vasculitis, one of two primary common vasculitis syndromes that predominantly affect children (the other being Henoch-Schönlein purpura/IgA vasculitis). • KD typically presents in children under 5 years of age with an incidence in the US of 25–50/100,000, similar to that of type 1 diabetes. • KD treated appropriately is associated with a low rate of mortality (approximately 0.08% case fatality rate in the largest series). • However, in the developed world, KD is the most common cause of acquired cardiac disease in childhood, with 25% of untreated and 5% of treated cases developing coronary artery aneurysms. • In infants less than 6 months of age, the risk of a coronary artery aneurysm is 50% even in KD patients treated with intravenous immunoglobulin (IVIG) within the first 10 days of illness. ` These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and role of echocardiography for patients with suspected or confirmed KD. Definitions and Medications Table 1. Definitions for Selected Recommendations for Term Definition A. Disease states KD Fever lasting at least at least four of the • Bilateral bulbar • Erythema and erythema of oral • Erythema and and/or periungual • Maculopapular, multiforme-like • Cervical lymphadenopathy in diameter), usually e diagnosis may principal clinical Incomplete KD Prolonged unexplained principal clinical laboratory studies [ESR]/ C-reactive urinalysis [UA] with cell [WBC]) or dilatation). a Acute KD Initial febrile phase B. Medications IVIG Single dose of 2 reasonable maximum Glucocorticoids (GC) Prednisone 2 mg/kg/day days, or equivalent. Non-GC immunomodulatory therapy Anakinra, cyclosporine, Aspirin High dose: 80-100 Moderate dose: Low dose: 3–5 mg/kg/day. a Defined according to algorithm in Burns, J.C. Pediatrics, B.W., et al. Circulation, 2017. 135(17): p. e927–e999.

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