Kawasaki Disease Shock Syndrome (KDSS) – Presentation of 3 Children and Review of the Literature

Background

Kawasaki disease (KD) is an acute, self-limited vasculitis commonly affecting children <5 years. It is the leading cause of acquired heart disease in children in developed countries and characterized by fever, bilateral non-exudative conjunctivitis, erythematous lips and oral mucosa, changes in extremities, rash and cervical lymphadenopathy. Importantly, 15–25% of untreated children will develop coronary artery aneurysms or ectasia and are at risk to suffer from myocardial infarction, sudden death, or ischemic heart disease. Incomplete or atypical KD is more common in young infants who have a higher risk to develop coronary artery aneurysms. Treatment with intravenous immunoglobulin (IVIG) in the first 10 days of illness significantly decreases the prevalence of this complication (Newburger JW et al., Circulation 2004; 110: 2747–2771). Over the last years several cases of KD associated with hemodynamic instability or Kawasaki disease shock syndrome (KDSS) have been described. (Chen PS et al., J Microbiol Immunol Infect 2015; 48: 43–50; Gámez-González LB et al., Eur J Pediatr 2013; 172: 337–342; Lin, MT et al., Pediatr Infect Dis J 2013; 161: 513–515; Dominguez SR et al., Pediatrics 2008; 122; e786–e790; Kanegaye JT et al., Pediatrics 2009; 123; e783–e789; Gatterre P et al., Intensive Care Med 2012; 38: 872–878). Here we report 3 children with KDSS requiring ICU admission and review the current literature.