Purpose: Review of the literature on adult Kawasaki disease.
Current knowledge and key points: Kawasaki disease is an acute multisystemic vasculitis affecting predominantly young children. Several studies have suggested that Kawasaki disease is mediated by bacterial superantigens. The diagnosis is established on clinical criteria since no specific laboratory test yet exists for this disorder. The severity of Kawasaki disease relates to the possible occurrence of coronary aneurysms in 20% of childhood cases. Treatment with intravenous immunoglobulins before day 10 is recommended to prevent aneurysm formation. The occurrence of Kawasaki disease is unusual in adults and 52 cases only have been reported in adult patients. Seventy-one per cent of cases occur between 18 and 30 years. The incidence of specific clinical features is quite similar between adults and children. However meningitis and thrombocytosis are more common in children than in adults, while conversely both arthralgias and liver function abnormalities are more common among adults. Coronary aneurysms are less common in the adults with Kawasaki disease. Other diseases with similar clinical presentation such as drug hypersensitivity reaction and the toxic shock syndrome must be ruled out. Kawasaki disease is often diagnosed after the acute phase at the step of desquamation, when it is too late to expect any beneficial effect from immunoglobulins.
Future prospects and projects: Diagnostic criteria of Kawasaki disease have not been validated in an adult population. Criteria of exclusion are necessary to eliminate toxic shock syndrome and drug hypersensitivity syndrome. An international retrospective study to collect data on epidemiologic, clinical, laboratory, and cardiovascular features of adult Kawasaki disease is necessary to validate specific diagnostic criteria and to improve the knowledge on this disease.