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Clinical Services at Rush Kawasaki Disease

Kawasaki disease (KD)

What is the cause of Kawasaki disease?
What are the symptoms of Kawasaki disease?
How is the diagnosis of Kawasaki disease made?
What is the treatment for Kawasaki disease?
What is the prognosis for Kawasaki disease?

Kawasaki disease (mucocutaneous lymph node syndrome; infantile polyarteritis) is a rare condition in children that causes inflammation of the blood vessels, including the coronary arteries, which supply blood to the heart. Kawasaki disease also affects lymph nodes, skin, and the mucous membranes inside the mouth, nose and throat.

Kawasaki disease occurs most frequently in Japan, where the disease was first discovered. In the United States, after congenital heart defects, Kawasaki disease is the leading cause of heart disease in children. Most patients are younger than age 5. The disease occurs more often in boys than in girls.

What is the cause of Kawasaki disease?

The cause of Kawasaki disease is unknown, but it may be an autoimmune disorder. There may be a genetic component or a viral cause.

What are the symptoms of Kawasaki disease?

The symptoms of KD appear in phases. Treating the disease within 10 days of its onset may greatly reduce the chances of lasting damage.

First phase

Kawasaki disease often begins with a high, persistent fever greater than 102°F, often as high as 104°F. Such a fever lasting at least five days is considered a classic sign. The fever may last for up to two weeks and does not usually go away with normal doses of acetaminophen (Tylenol) or ibuprofen (Advil).

Other symptoms often include:

  • Extremely bloodshot or red eyes (without a thick discharge)
  • Bright red, chapped or cracked lips
  • Red mucous membranes in the mouth
  • Strawberry tongue, white coating on tongue or prominent red bumps on back of tongue
  • Red palms of hands and soles of feet
  • Swollen hands and feet
  • Cough and runny nose
  • Irritability
  • Skin rashes on trunk and in genital area
  • Swollen lymph nodes, particularly in neck area

Second phase

  • Peeling skin in genital area, hands and feet (especially around nails, palms and soles)
  • Swollen lymph nodes (frequently only one lymph node is swollen), particularly in neck area
  • Joint pain and swelling, frequently on both sides of body
  • Diarrhea, vomiting and abdominal pain

Third phase

In the third phase of the disease, signs and symptoms slowly go away unless complications develop. It can take up to eight weeks for the child’s energy levels to be restored.

How is the diagnosis of Kawasaki disease made?

No tests specifically diagnose Kawasaki disease. The diagnosis is usually made based on the patient having most of the classic symptoms.

However, some children may have a fever lasting more than five days, but not all of the classic symptoms of the disease. These children may be diagnosed with atypical Kawasaki disease. Therefore, all children with fever lasting more than five days should be evaluated, with Kawasaki disease considered as a possibility. Early treatment is essential for those who do have the disease.

In addition to a physical exam, the following tests may be performed:

  • Complete blood count (CBC)
  • C-reactive protein (CRP)
  • Erythrocyte sedimentation rate (ESR)
  • Serum albumin
  • Serum transaminase
  • Urinalysis
  • Chest X-ray
  • Echocardiogram (ultrasound)
  • Electrocardiogram (ECG)

Procedures such as ECG and echocardiography may reveal signs of myocarditis, pericarditis, arthritis, aseptic meningitis and inflammation of the coronary arteries.

Diagnosis largely is a process of ruling out diseases that cause similar symptoms, including scarlet fever, juvenile rheumatoid arthritis, Stevens-Johnson syndrome (a disorder of the mucous membranes), toxic shock syndrome, measles and tick-borne illnesses such as Rocky Mountain spotted fever.

What is the treatment for Kawasaki disease?

Children with Kawasaki disease are admitted to the hospital. Treatment must be started as soon as possible after diagnosis, preferably while the child still has a fever, to prevent damage to the coronary arteries and heart.

Intravenous IV gamma globulin given by intravenous infusion in high doses is the standard treatment. The child's condition usually improves notably within 24 hours of treatment.

High-dose aspirin is often given to help reduce inflammation and fever. Anticoagulants can help to prevent clots.

Some studies have suggested that adding steroids to the usual treatment routine may improve a child's outcome, but more research is needed.

In severe cases, angioplasty or surgery may be needed to repair or bypass affected arteries.

What is the prognosis for Kawasaki disease?

With early recognition and treatment, full recovery can usually be expected. Even when they're treated with aspirin and IV gamma globulin, however, up to 25 percent of children may still develop problems in their coronary arteries. Patients who have had Kawasaki disease should have an echocardiogram every one to two years to screen for heart problems.

Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
Contact E-mail

Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612

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