Cardiac Center

Kawasaki Disease

What is Kawasaki disease?

Kawasaki disease is a condition that causes high fever and inflammation (redness and swelling) of the mouth, hands, feet and other areas, among other symptoms. Most of the other symptoms do not cause a significant problem. However, the disease also causes some of the body’s arteries to become inflamed. The most important arteries that can be affected by Kawasaki disease are the coronary arteries — the arteries that feed the heart.

Kawasaki disease is most common in young children. If treated quickly, children can make a full recovery. If the disease is not treated quickly, it can cause damage to the heart.

Doctors do not know for sure what causes Kawasaki disease, although it is believed to be an autoimmune response to a possible infectious agent that has not been able to be identified. Kawasaki disease is not felt to be contagious — even though the initial problem is typically an infection, the disease occurs because of the body’s immune response. People of Asian descent are more likely to get the disease. It is named for a doctor in Japan who was the first to identify it.

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What are the symptoms of Kawasaki disease?

The first symptom is a high fever lasting more than five days that won’t come down with medicine such as acetaminophen (TYLENOL®) or ibuprofen (ADVIL®). Other symptoms of Kawasaki disease may include:

There is not a blood test or any other definitive test for the diagnosis of Kawasaki disease. Instead, the decision to begin treatment is based on the patient’s condition, symptoms and medical history, as well as on the results of laboratory tests.

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What is the treatment for Kawasaki disease?

If a doctor suspects Kawasaki disease, your child will be admitted to the hospital and a medicine called intravenous immunoglobulin (IVIG) will be given through an IV line. The medicine is made from proteins extracted from human blood. Immunoglobulin is another word for antibodies, which are blood proteins that fight infections.

Corticosteroids and immune-suppressants are occasionally used in Kawasaki disease when patients fail to show significant improvement, even after receiving two doses of immunoglobulin. The immunoglobulin treatment tends to be ineffective at times in children less than 6 months of age.

Your child will be given the IVIG infusion, which can take up to 12 hours. He will then stay in the hospital for at least another 24 hours so that doctors can make sure his fever is gone and other symptoms are gone or have decreased.

As part of the treatment, your child will also take a high dose of aspirin pills for up to 14 days, and then a lower dose until a cardiologist says it’s okay to stop the aspirin.

During the evaluation for Kawasaki disease, your child will undergo an echocardiogram to evaluate the anatomy and function of the heart as well as to look at the coronary arteries. This will be repeated in the clinic after your child has been released from the hospital to ensure that there has been no lasting damage to the coronary arteries.

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How does Kawasaki disease affect the heart?

Most commonly, Kawasaki disease affects the coronary arteries.

The coronary arteries (there are two) are connected to the aorta, the large artery that carries oxygen-rich blood out of the heart to be delivered to the rest of the body. The coronary arteries carry a little of that blood back to the heart to feed its muscle and tissue with oxygen so it can pump.

Kawasaki disease can cause the coronary arteries to become enlarged. The walls of the arteries sometimes form abnormal bulges (aneurysms). Blood can collect in the aneurysms and begin to clot. The clot can block the artery, causing a heart attack. Children with Kawasaki disease are treated with aspirin because it helps prevent blood clots.

Most children with Kawasaki disease do not develop aneurysms or heart damage. To avoid problems, it’s very important that the disease is treated quickly and a cardiologist performs echocardiograms to check for damage.

Children who do develop aneurysms in the coronary arteries might have to keep taking aspirin or another “blood-thinner.” They will visit a cardiologist for check-ups at least once a year, and may need to undergo a heart catheterization.

If damage to the arteries is significant, the child will have to be monitored by a cardiologist for the rest of his life so that problems, should they develop, can be addressed immediately.

For those children who do not have any damage to the coronary arteries and who are treated successfully with IVIG and aspirin, one possible concern is that they might eventually develop premature atherosclerosis (coronary artery disease). The long-term risk for these children developing atherosclerosis early is unknown. We do know that, for the short term (into their early 30s), these children grow up and do not have complications. Along with the American Heart Association, we recommend that these children be re-evaluated at least every five years to ensure that there have been no problems with their hearts.

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Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.

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Reviewed by: Anirban Banerjee, MD
Date: September 2013

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Kawasaki Survival Story

Learn about Kendall's experience with Kawasaki disease. Read Kendall's Story.