Kawasaki Disease

  • What is Kawasaki disease?

    Kawasaki disease is an illness that causes inflammation of the medium sized blood vessels, including the arteries, veins and capillaries.

    In some children, Kawasaki disease can affect the coronary arteries. These arteries carry oxygen-rich blood to the heart. When these arteries are more severely affected, children can develop serious heart problems.

    Kawasaki disease usually affects young children. Most patients are under 5 years old and boys develop the disease one and half times as often as girls. Although it affects children of all races, in the United States it is found more often in children of Asian American ancestry, followed by children of African American descent.

    Kawasaki disease is named after Dr. Tomisaku Kawasaki, the Japanese pediatrician who first described the illness in 1967.

  • Causes

    Doctors and researchers have not determined the cause of Kawasaki disease. It is believed it is related to a combination of specific genetic factors and how children with these factors respond to a virus or infection.

    Kawasaki disease is not contagious and it cannot be prevented.

  • Signs and symptoms

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

    • Redness in both eyes, without pus or discharge
    • Red, chapped lips and red, swollen tongue
    • Redness and/or swelling of the hands or feet
    • Red skin rash (including in the groin area)
    • Swollen lymph nodes (in the neck)
    • Diarrhea, vomiting and/or abdominal pain
  • Tests and procedures

    Doctors usually diagnose Kawasaki disease after carefully examining a child, observing his or her symptoms and eliminating the possibility of other, similar diseases. If Kawasaki disease is suspected, your child’s doctor will order blood tests and possibly a chest x-ray, echocardiogram or electrocardiogram to look for signs of inflammation and to see if the coronary arteries of the heart are enlarged or there is heart damage.

    Most children can be diagnosed by a pediatrician or physicians in the emergency department. Occasionally, infectious disease physicians or rheumatologists are called on to help make the diagnosis or to help with treatment decisions.

    Cardiologists monitor all children with Kawasaki disease for heart involvement. Occasionally other specialists also help evaluate children depending on individual circumstances.

  • Treatment

    To reduce the risk of complications, treatment for Kawasaki disease should begin soon after the diagnosis, preferably while a child still has a fever. The goals of this initial treatment are to reduce fever, rash and inflammation and possible damage to the blood vessels of the heart.
    Children with Kawasaki disease need to be treated in the hospital with aspirin and intravenous gamma globulin to decrease the risk of developing heart disease. In the rare event your child does not respond to intravenous gamma globulin, the dose may need to be repeated. There are other medications that have also been successful in treating Kawasaki disease.

    If the heart is involved, specific treatment of the heart may also be needed.

  • Outlook

    Most children with Kawasaki disease respond quickly to the intravenous gamma globulin and fully recover. Children usually receive a low dose of aspirin for a month or so until we are sure there is no damage to the coronary arteries. This is evaluated by another echocardiogram performed during an outpatient visit by the cardiologist .

  • Follow-up care

    Even if there is no evidence of a heart abnormality when your child recovers from Kawasaki disease, it is important to bring your child in for follow-up visits. This can help your child receive treatment for any issues that do not show up immediately. Your child will need to return to cardiology for follow-up echocardiograms and other tests as recommended by your healthcare team.

Reviewed by Anirban Banerjee, MD, David D. Sherry, MD on January 21, 2016