Kawasaki disease is a pediatric illness which causes inflammation in blood vessels and can affect the heart. Below, you’ll find answers to frequently asked questions regarding this disease and successful treatment options at Children’s Hospital of Philadelphia’s Cardiology Kawasaki Disease Program.

What is Kawasaki disease? 

Kawasaki disease is a childhood illness that causes inflammation in some blood vessels in the body. It 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 affects children of all races, it occurs more often in children of Asian descent. 

Kawasaki disease requires prompt treatment to prevent damage and aneurysms of the coronary arteries, the blood vessels that provide oxygen-rich blood to the heart.

What causes Kawasaki disease? 

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

How is Kawasaki disease diagnosed? 

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. 

Symptoms of Kawasaki disease include: 

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

Children with Kawasaki disease may have only a few of these symptoms. They may also have other symptoms. 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. The clinician may also recommend tests, such as an echocardiogram, to check for enlarged coronary arteries (coronary aneurysms) or damage to the heart. 

Usually, primary care providers make the Kawasaki disease diagnosis, but sometimes a team of specialists — from departments such as rheumatology, infectious disease and cardiology — are involved. 

What should I do if I am concerned about my child having Kawasaki disease? 

If you have concerns about possible Kawasaki disease in your child, please discuss with your child's primary care provider. They can perform an evaluation and determine the next steps. 

One of those steps may include a visit to the Emergency Department, since children with Kawasaki disease require admission to the hospital in order to receive treatment.

What is the treatment for Kawasaki disease? 

To prevent complications, children with Kawasaki disease need to be treated in the hospital with medications which reduce inflammation. Treatment should begin as soon as possible, ideally within the first 10 days of illness or diagnosis. 

The main treatments for Kawasaki disease include intravenous immunoglobulin (IVIG) and aspirin. These medications help to address inflammation and 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 from the hospital once their fever has been resolved and they are feeling better. 

Will my child require future cardiology appointments? 

Children diagnosed with Kawasaki disease will need regular checkups with a cardiologist after leaving the hospital. These visits are important to make sure the heart and coronary arteries are healthy.

Usually, these checkups will include an echocardiogram. If your child was treated with additional medications, like steroids, they may also need to see a rheumatologist. Most children will continue to take a low dose of aspirin for at least six weeks, until the cardiologist confirms that the coronary arteries are not damaged. 

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

I am a clinician caring for a patient with Kawasaki disease and coronary artery aneurysms. How do I contact the Cardiology Kawasaki Disease Program?

At CHOP, our Cardiac Center Referral Office is exclusively dedicated to helping referring providers connect with the Cardiac Center, including the Cardiology Kawasaki Disease Program. Nurse Navigators assist with transferring records, second opinion consultations, and coordinating care and testing. 

Please call 267-426-9600 to reach the team.

We are here to help, and we look forward to speaking with you.