Early recognition is vital to prevent cardiac complications

Dr Tomisaku Kawasaki described the disease that bears his name nearly 30 years ago.1 Kawasaki disease is now the commonest cause of acquired heart disease in children in developed countries. Its cause remains unknown, and it presents doctors with many difficulties in diagnosis and management. Kawasaki disease is a systemic febrile vasculitis predominantly affecting children aged under 5 years. The incidence in Britain is 3.4 per 100 000 children aged under 5 years2—about a third of the incidence reported in the United States and a 30th of that in Japan.3 The most important complication, coronary arteritis leading to formation of aneurysms, occurs in 20-30% of untreated patients.3 Thrombosis within an aneurysm, myocardial infarction, and dysrhythmias may occur in the acute phase of the illness. The case fatality rate in Britain in 1990 was 3.7%,2 which compares unfavourably with the United States and Japan, where in some centres it is as low as 0.1%.2 Patients also suffer long term morbidity as a result of scarring of coronary arteries, intimal thickening, and accelerated atherosclerosis.3