GP68 More than a fever: a case series of atypical kawasaki disease

Introduction Kawasaki Disease is a vasculitis which tends to occur in children between the ages of 1 and 8 years. It is characterized by prolonged fever (>38.5°C, >5 days), exanthem, non-exudative conjunctivitis, inflammation of mucous membranes and cervical lymphadenopathy. Coronary artery aneurysms may develop and rupture or cause myocardial infarction. Diagnosis is by clinical criteria and treatment includes aspirin and IV immunoglobulin.Diagnostic criteria for Kawasaki disease are fever and at least four of five additional clinical signs. Atypical (Incomplete) Kawasaki disease should be considered in all children with unexplained fever for more than 5 days, associated with 2 or 3 of the main clinical findings of Kawasaki disease. Diagnosis of Atypical Kawasaki disease is based on echocardiographic findings indicating the involvement of the coronary arteries. Case Series From February 2016 to October 2018, four patients presented to Sligo University Hospital with Atypical Kawasaki’s Disease. The patients varied in age from seven months to four years old. The classical signs of Kawasaki disease include persistent high grade pyrexia bilateral non-exudative conjunctivitis, bright red (‘strawberry’) tongue, cervical lymphadenopathy and skin desquamation. Less specific signs include rash and irritability. All four patients developed persistent high-grade pyrexia (>38.5°C, >5 days) and lymphadenopathy. None had all the typical signs of Kawasaki disease. Three were observed to have a widespread maculopapular rash. Two had bilateral exudative conjunctivitis, or irritability. One had a ‘strawberry tongue’ appearance. None had skin desquamation. Only 50% of patients had Kawasaki disease in their initial differential diagnosis. Other differentials included upper respiratory tract infection, tonsillitis, scarlet fever, transient synovitis, cervical lymphadenitis and retropharyngeal abscess. Management All four patients were treated with IVIG and aspirin. The mean duration of pyrexia prior to commencement of treatment was 7.5 days. The mean duration from hospital presentation to commencement of treatment was 3.5 days. Outcome 50% of patients made a complete recovery. Despite treatment, 25% developed a mild fusiform dilatation of the left coronary artery. A further 25% developed a saccular aneurysm of the left circumflex coronary artery. Discussion It is imperative to include Kawasaki and Atypical (Incomplete) Kawasaki disease in the differential diagnosis of a child with persistent high-grade pyrexia. Delays in treatment may lead to a greater risk of coronary aneurysm and mortality.