Kawasaki Disease

Q: What is Kawasaki disease?


Kawasaki disease is an acute inflammatory disease of the blood vessels that occurs in infants and young children. The inflammation affects many parts of the body, but the most serious effect is on the heart. Kawasaki disease is the leading cause of acquired heart disease among children living in the United States and other developed nations.

Q: Are there any known causes of Kawasaki disease?


We don't know the cause of Kawasaki disease. Many experts suspect an infectious and/or genetic cause.

Q: What are the common symptoms? How is Kawasaki disease diagnosed?


Symptoms include:

  • Fever
  • Changes in extremities, including redness and swelling of the hands and feet
  • Rash
  • Redness of the white part of the eye
  • Red, cracked lips and red tongue (“strawberry tongue”)
  • Lymph node enlargement of the neck

Many illnesses present with some or all of the clinical signs and symptoms listed above. There is no simple diagnostic test for Kawasaki disease. Instead there are criteria based on symptoms and test results that help us make the diagnosis. If fever is present for 5 days or more with at least 4 of the above symptoms, your pediatrician may diagnose Kawasaki disease.

When these criteria are not met, an evaluation including blood work, urinalysis, and possibly an echocardiogram (ultrasound of the heart) can help your pediatrician make the correct diagnosis.

Q: How does Kawasaki disease affect the heart?


Diagnosis and management of Kawasaki disease requires the involvement of a pediatric cardiologist who can perform and evaluate an echocardiogram. Kawasaki disease causes inflammation throughout the body and can affect the heart.

Kawasaki disease can produce inflammation of the coronary arteries (the arteries which supply blood to the heart muscle), resulting in dilation (enlargement) or in the formation of aneurysms. Clots can form inside aneurysms and block the flow of blood to the heart muscle. This can damage the heart muscle, causing a heart attack. Over time, aneurysms can resolve, although sometimes a narrowing can develop where the aneurysm was. A narrowing in the blood vessel may also limit blood flow to the heart muscle.

Q: How is Kawasaki disease treated?


A child diagnosed with Kawasaki disease will be admitted to the hospital and receive IV Immunoglobulins (IVIG) at the time of diagnosis. IVIG helps to “clear the blood” of inflammatory cells circulating in the body. High dose aspirin will also be used to help decrease the inflammation. If the initial echocardiogram does not show any signs of coronary artery involvement, the aspirin dose will be decreased and treatment with a lower dose of aspirin will continue for a total of 6-8 weeks. Most children respond to this treatment regimen. For most children who receive IVIG and aspirin, their fevers subside quickly and other symptoms improve over a short period of time. This treatment decreases the chance that the coronary arteries will develop aneurysms.

If your child continues to have fevers after receiving a dose of IVIG or has a large coronary artery aneurysm, an additional dose of IVIG may be administered or other medications may be prescribed.

After discharge from the hospital, your child will be closely followed by a pediatric cardiologist. If there are coronary aneurysms present, other aspirin or blood thinners might be used to treat your child. Length of time for treatment with blood thinners will depend on how the coronary artery aneurysms behave over time.