Kawasaki Disease (KD)

Kawasaki disease (KD) is a childhood illness that causes vasculitis (inflamed blood vessels). KD is more common in children younger than 5 years old. KD occurs more often among boys and is more commonly seen in the winter and spring months. The exact cause of KD is unknown, but it is suspected that it may be triggered by an infection. It may also occur in children who have a genetic predisposition to the disease. KD is not contagious.

What Are the Signs/Symptoms?

The most common symptoms include prolonged fever, rash, bloodshot eyes, red/cracked lips and tongue, and lymph node swelling. Children with KD may also have painful or swollen joints, extreme fussiness, and swelling of the gallbladder that can cause belly pain and vomiting. The symptoms of KD often go away on their own and the child recovers. Without medical evaluation and treatment, however, serious damage to the blood vessels of the heart may develop and not be initially recognized. This can usually be prevented by early treatment. There is no specific test to diagnose KD. Rather, doctors diagnose KD based on symptoms and physical exam. A prolonged fever (i.e., more than five days) is often the first symptom that alerts a doctor to consider KD. Helpful tests may include: (1) blood and urine tests, (2) electrocardiogram, (3) echocardiogram (ultrasound of the heart).

What Are Common Treatments?

There are two main treatments for KD:

• Aspirin therapy. Aspirin is given every 6 hours initially. Then the dose decreases to once a day. A child may have to continue taking aspirin for an extended period.
• Intravenous immunoglobulin (IVIG) is an IV medicine that reduces the swelling in the blood vessels Parents are sometimes concerned about using aspirin in children due to concern over Reye syndrome. Reye syndrome is an inflammatory process that affects the brain and liver and has occurred in patients taking aspirin who are infected with chicken pox or influenza. Most doctors suggest that if your child is taking aspirin for a long period for any reason, they should have a flu shot to avoid influenza infection. If your child is on aspirin and they have been exposed to or have chicken pox, you should contact your doctor immediately for advice.

Living with KD

There is no specific test to diagnose KD. Rheumatologists help to diagnose and treat children with KD. Follow up care is very important because, often, damage to the coronary arteries does not show up until several weeks later. Fortunately, with appropriate treatment, most children do not have any long-term sequelae from KD. Children that do have coronary artery involvement will need regular cardiology evaluations.

Updated February 2023 by Kanika Monga, MD, and reviewed by the American College of Rheumatology Communications and Marketing Committee.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.