Kawasaki disease: diagnosis, management and cardiac sequelae

Kawasaki disease (KD) is an acute self-limiting systemic vasculitis of unknown etiology and the most common cause of acquired coronary disease in children aged 6 months to 5 years. The inflammatory process results in coronary arteritis, aneurysmal lesions, arterial thrombotic occlusion or even sudden death. The diagnostic tests are unknown but treatment with immunoglobulin and aspirin is effective at reducing cardiac complications from 25 to 4.7% in the UK. Myocardial, endocardial or pericardial inflammation may occur acutely or many years later and abnormalities of myocardial blood flow may require ongoing medication, interventional catheterization or even cardiac surgery. There are several new drugs that may have important roles to play in managing KD in children and young adults.