What is Kawasaki disease?

• Kawasaki disease, also sometimes referred to as mucocutaneous lymph node syndrome, is an illness that causes inflammation (swelling) of medium sized blood vessels in the body.
• Kawasaki disease often goes away on its own, but if it is not treated it can cause serious injury to the heart and other organs.
• In some cases, the disease can affect the coronary arteries, which are blood vessels that supply oxygen-rich blood to the heart. This can cause serious heart problems.

What are the signs and symptoms?

• Fever lasting more than five days, often the fever won’t resolve with Tylenol® or Motrin®
• 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)
• Irritability and/or fussiness
• Less common: abdominal pain, joint pain

What is the cause?

• The exact cause of Kawasaki disease is unknown. There are many researchers working to identify a specific trigger.
• Current research suggests that it is likely to be related to a combination of specific genetic factors and how children’s immune system react to a virus or infection.
• Kawasaki disease is not contagious and cannot be prevented.
• To learn more about the research into the genetics of Kawasaki disease, please visit the NIH website.

Who gets Kawasaki disease?

• Kawasaki disease usually affects young children. Most patients are under 5 years old.
• Boys develop the disease one-and-a-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 descent, followed by children of African-American descent.

How do doctors diagnose Kawasaki disease?

• There is no single test that can diagnose Kawasaki disease.
• Doctors make the diagnosis after examining a child, observing symptoms, and obtaining additional testing that are consistent with the diagnosis.
• If Kawasaki disease is suspected, your child’s doctor will order blood tests to look for inflammation and possibly an echocardiogram or electrocardiogram (EKG) to see if the coronary arteries of the heart are enlarged or there is heart damage.
• While most children can be diagnosed by a general pediatrician, making the diagnosis may sometimes involve a team of doctors that include infectious disease, rheumatology, and cardiology specialists.

How is Kawasaki disease treated?

• To prevent complications, children must be treated in the hospital with medicines that decrease inflammation.
• Treatment should begin soon after the diagnosis, especially within the first 10 days of illness.
• The treatment helps stop the fever, improves symptoms, and prevents damage to the heart blood vessels.
• The treatment includes aspirin and intravenous gamma globulin (IVIG).
• Some children may also receive steroids if they are very young or have signs of inflammation on their first echocardiogram.
• Most children with Kawasaki disease respond quickly to the IVIG and fully recover, but some children need more IVIG or other treatments like steroids to help with the symptoms.
• Children are discharged once their fever is gone and they are feeling better.

What are the steps after treatment?

• Children diagnosed with Kawasaki disease will need to be seen regularly by a cardiologist after leaving the hospital to make sure their heart is not affected.
• They may also need to be seen by a rheumatologist if they received additional therapies such as steroids.
• At the cardiology visits, your child will get repeat echocardiograms to check for inflammation of the heart vessels. It is very important to keep these appointments.
• Children usually receive a low dose of aspirin for at least six to eight weeks until the cardiologist makes sure there is no damage to the heart blood vessels.

Want to learn more about Kawasaki disease?

• Contact CHOP's Division of Rheumatology at 215-590-2547.
• Additional Resources:
  • Kawasaki Disease Foundation
  • healthychildren.org