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Despite being the focus of intensive research and the most common form of acquired heart disease in US and British children, the causes and optimal treatment for Kawasaki disease remain elusive. Herpes viruses might have a part to play, and there seems to be some seasonal variation, with the disease peaking in winter and spring. In Japan, it is more common among siblings, 8 to 9 per cent of whom under the age of 2 are affected.
Recent evidence suggests that high dose aspirin is more effective than low doses for reducing inflammation and the subsequent risk of coronary artery aneurysm and arterial thrombosis. But 30 mg/kg/day will minimise gastrointestinal and other side effects. There is no evidence that additional dipyridamole is any more effective.
Up to 40 per cent of children treated with aspirin alone will develop cardiac abnormalities, and 2g/kg of intravenous immunoglobulin infused over 10 hours reduces these risks by 9 per cent at 30 days. Best given early, it should not be ruled out after 10 days; some patients might benefit from a second dose. Steroids are more controversial, but should be considered when intravenous immunoglobulin is unsuitable.
Aneurysms will develop in up to 40 per cent of patients. Most regress within two years, but giant aneurysms of more than 8 mm carry a risk of death of almost 4 per cent. The latest thinking is that platelet glycoprotein IIb/IIIa receptor blockade treatment may be effective. But yearly lifelong monitoring of all children who have had Kawasaki disease is recommended.