Kawasaki Syndrome

Kawasaki’s disease is a disease of unknown etiology. The manifestations relate to super-antigen formation and it is a multi-system inflammatory disease. And the main thing is fever of five days or more and then four of the remaining five manifestations. The definition is a surveillance definition and I think one of the take-home messages is that you should be prepared, in young children, to treat patients even though they don’t have four or five of the manifestations. There are certain things that just seeing it alone, and that is changes in the extremities, edema, induration of the hands and feet, is enough to strongly suspect Kawasaki’s disease if you don’t have another etiology.

Other findings are conjunctival injection, mouth lesions, fissuring, crusting of the lips, strawberry tongue. The induration of the hands and feet in a child under a year is diagnostic in itself. Rash of quite variability and the least common manifestation is enlarged lymph nodes, even thought the original description included lymph nodes in the title. Associated with this is evidence of multi-system pyuria, various other findings. Important is aseptic meningitis and this should help you with the diagnosis, not turn you away from the diagnosis such as I have seen in one case.

This is a picture and the rash can be multiforme, it can be just erythematous maculopapular, more important thing here is the swelling of the hands. The scleral conjunctivitis. This is late in the illness, the desquamation in the tips of the fingers and this is desquamation in the feet. This is the red palm, one of the original papers from Japan. Strawberry tongue in a Kawasaki’s patient. Now the crucial thing here, or the main problem with Kawasaki’s disease, are giant aneurysms of multiple different arteries, and the important ones are coronary arteries leading to infarcts. So patients need to be treated and you need to make a decision. With fever for a few days you have a lot of differentials of which Kawasaki’s is sort of at the bottom. Once you get beyond five days there are less differential possibilities and you want to treat by ten days.

Treatment is IVIG, and the most usual treatment today is 2 gm/kg over 12 hours. Also for inflammation, aspirin in high dose, four doses a day at 100 mg/kg. Then later put the patient on long-term aspirin for the anticoagulant effect. There are differences in this but certainly you need to wait until evidence of acute inflammation is over. There is no evidence that aspirin has any benefit as far as the heart is concerned, but there is evidence that IVIG treatment is beneficial. The other thing is that one dose is frequently not enough, so roughly 25% of patients need one or more doses, particularly in the children under a year.

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