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What Is Kawasaki Disease?

Kawasaki disease (KD) is a rare condition that causes inflammation of blood vessels (vasculitis) and several other manifestations noted below. KD usually occurs in children younger than 5, more often in boys than girls. In the United States, about 4000 new cases are diagnosed each year. It’s the leading cause of acquired heart disease in children.

What Causes KD?

The cause is unknown, but an infectious agent may trigger the inflammation in people with a genetic predisposition to it. KD isn’t contagious, however. It is not a hereditary disorder.

What Are the Symptoms of KD?

Symptoms include fever that lasts for more than 5 days, and is associated with red swollen eyes (conjunctivitis); cracking lips; strawberry-colored tongue; red, swollen, sore throat; swollen lymph glands in the neck; and rash on the trunk and limbs. Complications that may mean heart involvement include shortness of breath; chest pain; and symptoms of congestive heart failure, such as leg swelling, being unable to lie flat in bed, suddenly waking up short of breath, and shortness of breath with exertion.

How Is KD Diagnosed?

The health care provider makes a diagnosis from physical examination and symptoms: fever lasting more than 5 days plus four of five other features. These features include inflammation of the whites of the eyes (conjunctivitis) and swollen eyes; mucous membrane changes (red tongue and dry, cracked lips); swollen hands and feet; swollen neck lymph glands; and rash on the trunk of the body.

The health care provider may order blood tests and x-rays, but no specific blood test can diagnose KD.

For suspected heart involvement, echocardiography (a sonogram of the heart) and cardiac catheterization can be done. In catheterization, the doctor puts a tube into the leg artery and moves it up into heart arteries. Dye is then injected to show blockages in coronary (heart) arteries.

How Is KD Treated?

Aspirin is used for fever, rash, and pain and to prevent blood clots in coronary arteries. Intravenous immunoglobulin can significantly reduce coronary artery disease and improve the heart’s pumping if given early. For coronary artery and heart involvement, the health care provider may suggest percutaneous transluminal coronary angioplasty. In this procedure, a balloon-tipped tube is put into the coronary artery. The balloon is inflated to open the diseased artery. A child with many diseased vessels may have coronary artery bypass surgery. Heart transplant may sometimes be considered.

DOs and DON’Ts in Managing KD:

  • DO remember that nearly 20% of children may have heart damage.
  • DO understand that risk factors for coronary artery problems are fever of more than 10 days, age younger than 1 year, and male sex.
  • DO call your child’s health care provider if your child has a fever, rash, or shortness of breath.
  • DO call your child’s health care provider if you see swollen lymph glands in the neck.
  • DO call your child’s health care provider if you need a second opinion.
  • DON’T forget that KD is a rare disease that requires care from a team of doctors who have experience with this condition.
  • DON’T be afraid to ask for a second opinion.
  • DON’T ignore symptoms.

Contact the following sources:

  • Kawasaki Disease Foundation
    Tel: (978) 356-2070
  • American Heart Association
    Tel: (800) 242-8721
  • American College of Cardiology
    Tel: (800) 253-4636

Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.

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