6038 Atypical kawasaki disease – a diagnostic dilemma

Objectives Kawasaki Disease (KD) is the second most common vasculitis in childhood and is the leading cause of acquired heart disease in children in high-income countries.¹ ³ Lack of appropriate treatment may lead to coronary artery aneurysms (CAA) formation and approximately 75% of cases occur under 5 years of age, with children aged <6 months and >5 years more at risk of developing CAA.² KD can present without all diagnostic criteria which is classified as atypical or incomplete KD and can present a significant diagnostic challenge for clinicians.³ Methods Two cases of incomplete KD will be discussed which proved a diagnostic dilemma to the paediatric team Results The first case is a 7-month-old baby who was admitted with two day history of fever and vomiting. Initially treated as suspected sepsis as initial blood culture and CSF gram stain showed gram positive cocci. However, CSF culture did not grow any organisms. Despite being on broad spectrum antibiotics, she continued to be pyrexial for more than 12 days which were associated with a blanching rash, red lips and bilateral conjunctivitis. Subsequently the child was managed as KD and received intravenous immunoglobulins (IVIG) with good effects. The second case is a 9-year girl who presented with fever, lymphadenitis and deranged clotting with normal liver enzymes. Initially treated as lymphadenitis with intravenous antibiotics with poor response. She remained febrile and become hypotensive. Tertiary advice was sought, and she was treated with IVIG and intravenous steroids. Both children developed large CAA and are now on anticoagulant therapy and cardiac surveillance. Conclusion Kawasaki Disease is a rare disease which may prove a diagnostic challenge to clinicians, if incomplete presentation. Long-term sequelae, with CAA being the most common one, remains one of the major risks associated with this disease. We will present the two cases of atypical Kawasaki Disease and share the learning points. References Forsey J, Mertens L. Eur J Pediatr, 2012;171:609–611. Eleftheriou D, et al. Arch Dis Child, 2014;99:74–8. De Graeff N, et al. Rheumatology (Oxford), 2019;58(4):672–682.