Kawasaki disease

What is Kawasaki disease?

Kawasaki disease is an acute illness characterized by fever. The clinical condition affects previously healthy children between the ages of 6 months and 5 years. Currently, the disease is the most common cause of heart disease in children. The diagnosis is based on the presence of a fever lasting at least 5 days and a number of additional signs and symptoms, which often occur sequentially rather than all at once.

The diagnosis of the disease is made in any child with a prolonged high fever, regardless of other symptoms. It should be noted that the clinical condition is associated with a risk of critical dilation of the coronary arteries – formation of coronary artery aneurysms and very likely subsequent heart attacks in untreated children.

The disease was first reported in Japan in 1960. The incidence of the disease continues to be highest in Japan, followed by Taiwan and then Korea, although cases in Europe and North America continue to increase.

What are the symptoms?

The disease is an acute vasculitis that affects otherwise healthy children. The diagnosis of the disease is based on several criteria as follows:

• Fever lasting at least 5 days, exclusion of other causes of high fever, and at least 4 of the following 5 clinical features:

1. Bilateral nonpurulent conjunctivitis – redness of the eyes;
2. Changes in the shape of the lips and oral cavity – bright red lips, strawberry-colored tongue;
3. A rash that is without petechiae and without bubbles;
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5. Uterine lymphadenopathy – enlarged lymph nodes in the neck
6. Extremity changes – swelling of the hands or feet, redness of the hands or feet, peeling of the skin on the hands or feet.
7. A few of these symptoms may be available to make the diagnosis of the disease, provided that coronary aneurysms or dilation of the coronary arteries are found on echocardiography.

Usually children affected by Kawasaki disease suddenly develop a high fever for no apparent reason. The fever lasts more than 5 days, and the child is irritable and seems lethargic. Along with the high temperature, the symptoms described above can also develop, and the sequence of their appearance is irrelevant. The time of their occurrence is also irrelevant. The diagnosis is made when the above criteria are present and there is no other explanation for the symptoms such as strep throat or an acute drug reaction.

The disease proceeds in several stages:

• Acute /early/ phase – the fever and other symptoms that last from 5 to 10 days
• Subacute phase – development of aneurysms in the coronary arteries – 11 to 30 days;
• Recovery phase – resolution of acute symptoms;

25% of untreated patients develop coronary artery aneurysms, which can lead to a heart attack /acute myocardial infarction/ from several months to several years after diagnosis.

Treatment of Kawasaki disease

Once the diagnosis is made, it is imperative that the treatment be started within 10 days of the high fever. This is due to the fact that the damage to the coronary arteries begins after the 10th day of the disease during the subacute phase. Current therapy consists of hospitalizing the child and administering intravenous immunoglobulin and high-dose aspirin until the child’s fever subsides. Low-dose aspirin is then given for 6 to 8 weeks until a normal ultrasound is obtained. If a child has an established coronary artery anomaly, regular check-ups with a pediatric cardiologist are required.