What is Kawasaki disease?

Kawasaki disease is a childhood illness that causes inflammation in some of the blood vessels in the body.  Kawasaki disease primarily affects young children, with most cases occurring in children under 5 years old. Kawasaki disease is slightly more common in boys, and although it can affect children of all races, it is seen more often in children of Asian descent.

Kawasaki disease requires prompt treatment to prevent damage to the coronary arteries, which are the blood vessels that provide oxygen-rich blood to the heart. With treatment though, most children recover completely fortunately.

Causes of Kawasaki disease

The exact cause of Kawasaki disease is unknown, but many researchers are working hard to determine the triggers for this condition. Current studies suggest that it may be linked to a combination of genetic factors and the way a child’s immune system responds to various viruses. 

Diagnosis and testing for Kawasaki disease

There is no single test that can diagnose Kawasaki disease. Clinicians make the diagnosis by examining the child, checking for certain symptoms, and ordering tests that support the diagnosis. 

Symptoms of Kawasaki disease include:

• High fever lasting at least four days
• Redness in both eyes, without pus or discharge
• Rash
• Red, chapped lips and/or red, swollen tongue
• Redness and/or swelling of hands or feet, and/or peeling of skin on hands or feet
• Swollen lymph nodes in the neck

Children with Kawasaki disease may have only a few of these symptoms or may have other symptoms as well. It is important to have a high suspicion for Kawasaki disease to make this diagnosis.

If Kawasaki disease is suspected, the clinician may order blood tests to look for signs of inflammation. They may also recommend heart tests, such as an echocardiogram, to check if the coronary arteries are enlarged or if there is any damage to the heart. 

Most of the time, primary care providers make this diagnosis, but sometimes a team of specialists — such as rheumatology, infectious disease, and cardiology — may be involved.

Treatment for Kawasaki disease 

To prevent complications, children with Kawasaki disease need to be treated in the hospital with medications that reduce inflammation. Treatment should begin as soon as possible, ideally within the first 10 days of illness. The main treatments include intravenous immunoglobulin (IVIG) and aspirin. These medications help to address the inflammation and to prevent coronary artery damage. Most children respond well to IVIG and recover fully, but some may need additional IVIG or other treatments such as steroids or infliximab, especially if they are very young or if their echocardiogram is abnormal. Children are usually able to go home once their fever has resolved and they are feeling better.

Follow-up for Kawasaki disease

Children diagnosed with Kawasaki disease will need regular check-ups with a cardiologist after they leave the hospital. These visits are important to make sure the heart and specifically the coronary arteries are healthy, and during these check-ups, the cardiologist will repeat the echocardiogram. If your child was treated with additional medications, like steroids, they may also need to see a rheumatologist. Most children will continue taking a low-dose of aspirin for at least six weeks, until the cardiologist confirms that the coronary arteries are not damaged.

Some children though will develop coronary artery complications, specifically coronary artery aneurysms, from Kawasaki disease. These children will require ongoing close cardiology follow-up, and our experienced Cardiology Kawasaki Disease Program works with these children, their families, and their other providers to address their cardiac issues long-term.

Additional resources

To learn more about Kawasaki disease, explore these resources:

• Kawasaki Disease Foundation

Contact information

Division of Cardiology – 215-590-4040

Division of Rheumatology – 215-590-2547