Aims. Kawasaki disease is an acute systemic vasculitis and is the most common cause of acquired heart disease in children in the developed world. This review aims to synthesise recent insights into the disease and provide an update for clinicians on diagnostic and treatment practices. Methods. We conducted a review of the literature exploring epidemiology, aetiology, diagnosis, and management of Kawasaki disease. We searched MEDLINE, Medline In-Process, Embase, Google Scholar, and reference lists of relevant articles. Conclusions. Kawasaki disease is a febrile vasculitis which progresses to coronary artery abnormalities in 25% of untreated patients. The disease is believed to result from a genetically susceptible individual’s exposure to an environmental trigger. Incidence is rising worldwide, and varies widely across countries and within different ethnic groups. Diagnosis is based on the presence of fever in addition to four out of five other clinical criteria, but it is complicated by the quarter of the Kawasaki disease patients with “incomplete” presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Given its severe morbidity and potential mortality, Kawasaki disease should be considered as a potential diagnosis in cases of prolonged paediatric fever. 1. Introduction Kawasaki disease (KD) is an acute systemic vasculitis which progresses to cause coronary artery abnormalities in 25% of untreated patients [1]. It has surpassed rheumatic heart disease as the leading cause of acquired cardiovascular disease in children in the developed world [2]. Though over 40 years have passed since its initial identification in patients by Kawasaki [3], its aetiology remains largely unknown and a specific diagnostic test eludes researchers and clinicians. Diagnosis is based primarily on clinical criteria; however, the presentation is incomplete in approximately 25% of patients, and this patient subset appears to be at a comparatively higher risk of serious complications [4]. Coronary artery dilation and aneurysms can be prevented by timely administration of intravenous immunoglobulin, a limited and costly resource, placing an onus on practitioners to diagnose the disease promptly and accurately. 2. Epidemiology Kawasaki disease was shown to account for 23% of all paediatric vasculitides in a United States rheumatology clinic population study and is the second most common multisystem vasculitis of infancy and childhood behind
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