Kawasaki Disease

Kawasaki Disease is an autoimmune disease in which the medium sized blood vessels of the body become inflamed. Kawasaki disease is relatively rare, but a common type of vasculitis found among children. Almost 100% of all cases occur among children under the age of five. It occurs more often at the end of winter and beginning of spring. It is common in Japan, but there are cases reported all over the world. A few dozen children are diagnosed in Israel every year.

It is not known what causes the disease. Researchers suspect the cause is a type of infectious agent and that the disease is an overreaction of the immune system. It is not a hereditary disease, but doctor believe there is a genetic connection. It is not contagious and there are no preventative measures. The disease usually only occurs once; recurrences are extremely rare.

Signs and Symptoms of Kawasaki disease

  • High fever that lasts longer than five days
  • Redness in the eye (no mucus)
  • Red skin rash
  • Very red lips/tongue
  • Swelling of the palms and legs
  • Enlarged lymph nodes on one side of the neck
  • Joint pain
  • Stomach pain
  • Diarrhea

Cardiac involvement is the most significant manifestation of Kawasaki disease, since it can indicate long-term side effects. Cardiac involvement generally includes the main arteries, but sometimes heart valves are involved and there may be heart echo problems, pericarditis or rhythmic problems. The disease varies from child to child.

Diagnosis of Kawasaki disease
A clinical diagnosis is done if the child has an inexplicable fever which lasts longer than five days and four of these five symptoms: eye infections, enlarge lymph nodes, aneurysm, redness on lips or tongue, changes in the limbs. Additionally, there should be no other signs of another infection which could explain the symptoms. Rashes on arteries near the heart confirm the diagnosis.

Signs of an infection are an elevated erythrocyte sedimentation rate, leukocytosis, anemia. The level of platelets in the blood is usually normal, but your child may experience an elevated level of platelets in the second week. Children have their blood tested until levels are back to normal. EKGs should be done early on. The EKG helps determine and identify the presence of an aneurysm in the aorta.

Most children with Kawasaki will overcome the disease, but a small percentage will develop heart complications.

Treatments for Kawasaki disease
Any child who may have Kawasaki disease is hospitalized, evaluated and tested. In order to lower the risk of complications, treatment should be started immediately after diagnosis. Treatment includes aspirin and immunoglobin IVs. The two treatments will lower the infection and the symptoms will disappear. Most children are given a one-time large dose of immunoglobins. If they do not respond, they will be given a second dose. A high dose of aspirin is administered to treat the fever. Once the fever breaks, the child is given a smaller dose of aspirin. A child with cardiac involvement will continue to take aspirin for a few weeks.

The blood count needs to be monitored until it returns to normal. Periodic heart echoes are also conducted. Their frequency depends on the severity of the aneurysm. Most aneurysms go away on their own.

Kawaski disease has three main stages: the severe stage (first two weeks), the mild stage (until the fourth week), the recovery stage (until the third month).

Long-Term Prognosis
In most cases, the prognosis is good, and the child will develop normally with no long-term side effects. The prognosis for children who’ve experience aneurysms on their arteries depends on how much the aneurysm developed and how narrow the artery became.

We don't recommend any type of innoculation for the first 3-6 months, as immunoglobins can affect the immune system. Children who haven’t had any cardiac involvement can be active, while children who developed aneurysms should consult a cardiologist regarding sports and other physical exercise.