What Parents Need to Know About Kawasaki Disease

Commentary23/03/26 Jay Mehta, MD, Perelman School of Medicine at The University of Pennsylvania

Kawasaki disease is an autoimmune condition that causes inflammation of blood vessels throughout the body. It most often occurs in children who are under 5 years old. It is rare, but without prompt treatment it can lead to serious complications. Parents should know the signs and symptoms of Kawasaki disease and when to contact a doctor. Here are answers to some of the most common questions pediatricians get about Kawasaki disease.

Who is at risk for Kawasaki disease?

Most children with Kawasaki disease are under 5 years of age. Rarely, infants under 4 months of age, adolescents, and adults can develop the disease. Boys are affected slightly more often than girls.

The cause of Kawasaki disease is not known. Probably, there are a number of triggers, including viral infections, that lead the body to create a very intense short-term inflammatory state. Like other autoimmune conditions, genetics are likely a component, but the exact role they play is unknown.

What are the signs of Kawasaki disease?

Kawasaki disease has several specific symptoms. In some cases, these symptoms can be confused with other more common conditions, such as measles or scarlet fever. For a typical case in a child between the ages of 2 and 6 years, most will have a fever greater than 100.4° F (38° C) for five or more days, and at least four of the following five symptoms:

  • Red eyes with no discharge
  • Reddened, dry, cracked lips and a red strawberry tongue
  • Swelling, redness or dark purple color, and, eventually peeling of the hands and feet
  • Red, purplish, or reddish-brown, patchy rash over the trunk
  • Swollen, tender lymph nodes in the neck

Extreme irritability is often considered a sixth symptom doctors will watch for. When children are very difficult to console, even when given support like a bottle, being held, or a distraction, it can be a sign of Kawasaki disease, if in the presence of the above symptoms.

In children under 6 months of age, the only symptom may be prolonged fever, or infants may have just one or two of the most common signs. At the same time, it’s important to note that there are many more common causes of fever in children than Kawasaki disease, so not all fevers mean that a child has Kawasaki disease.

Why is prompt treatment so important?

The inflammation that defines Kawasaki disease can be especially dangerous if it affects blood vessels around the heart. In fact, Kawasaki disease is the most common cause of acquired coronary artery disease in children. Prompt treatment substantially reduces the risk of coronary artery disease.

If children are not treated, heart problems may develop, usually beginning 1 to 4 weeks after the illness starts. A few children develop the most serious heart problem, a bulge in the wall of a coronary artery (coronary artery aneurysm). These aneurysms can rupture or cause a blood clot, leading to a heart attack and sudden death. Treatment greatly reduces the risk of heart complications.

The standard treatment for Kawasaki disease is pooled immune globulin given intravenously. Often, patients feel better and the fever reduces after a single infusion. Doctors will also focus on treating the fever and other symptoms and preventing coronary artery aneurysms.

When should I call a doctor?

Anytime a child has a fever, it’s important to be vigilant. If the fever has lasted for 5 days, calling the child’s pediatrician is a good next step. At this point, a viral infection is still the more likely cause (remember Kawasaki disease is rare and typically has other more distinguishing symptoms). But after 5 days of fever, in most cases, the doctor will recommend having the child seen by a healthcare professional. In general, pediatricians are very familiar with Kawasaki disease. They’ll know what symptoms to look for and be able to recommend the right next steps.

To learn more about Kawasaki disease, visit the Manuals page.