Kawasaki Disease

Kawasaki disease (also called mucocutaneous lymph node syndrome) is a rare childhood disease that typically occurs in children younger than 5 years old. 

In this form of vasculitis, any type of blood vessels can become inflamed. Up to 25 percent of children with Kawasaki disease develop coronary artery problems later in life.

Remarkable Care for Kids

• Experts in pediatric infectious diseases: At Rush, specialists in the Section of Pediatric Infectious Diseases diagnose and treat any and all suspected infectious diseases in children, including Kawasaki disease.
• Comprehensive care for children with heart problems: Rush University Children's Hospital provides comprehensive services for both congenital and acquired heart disease. Pediatric cardiologists and pediatric cardiovascular surgeons offer all of the latest techniques for treating and repairing the heart and its arteries.

What is Kawasaki disease?

If Kawasaki disease (KD) affects the coronary arteries that carry blood to the heart, serious heart problems may develop.

The cause of Kawasaki disease is unknown, but it is not contagious. It is thought that KD might be the body's response to a virus or infection combined with genetic factors. One serious complication is aneurysms, bulges in blood vessels that must be treated to prevent them from rupturing.

Symptoms of Kawasaki disease

Symptoms appear in phases. The first phase often includes the following:

• A fever greater than 102°F lasting at least five days
• Bloodshot eyes
• Cough and runny nose
• Irritability
• Rashes on the trunk and genitals
• Swollen lymph nodes

The second phase often includes the following symptoms:

• Peeling skin in genital area, hands and feet
• Joint pain and swelling
• Diarrhea, vomiting and abdominal pain

In the third phase, signs and symptoms slowly go away unless complications develop.

Care for Kawasaki disease at Rush

Diagnosis

Starting treatment within 10 days of onset of symptoms typically limits the chances of serious complications. With early diagnosis and treatment, most children make a full recovery.

Talk to your child's pediatrician if your child has any of the symptoms above. There is no one test for Kawasaki disease. Your child's doctor will first rule out other diseases with similar symptoms. These include Rocky Mountain spotted fever, scarlet fever, juvenile rheumatoid arthritis and measles.

The following tests may also be performed:

• Blood tests to determine if the blood vessels are inflamed
• Chest X-ray to see if Kawasaki disease has affected the heart
• Electrocardiogram, or EKG, to test if Kawasaki disease has affected the heart’s electrical activity
• Echocardiogram (ultrasound) to check the disease’s effect on your child’s coronary arteries

If KD has affected your child’s coronary arteries, they will be referred to a pediatric cardiologist at Rush. Other specialists may be involved in your child’s care, depending on the type and severity of symptoms.

Treatment

Your child will need to be admitted to the hospital, at least for early treatment of KD. Treatment must be started as soon as possible, preferably while the fever is high.

The goals of treatment include the following:

• Reducing fever and inflammation
• Protecting the coronary arteries

Medication are the main treatments for Kawasaki disease. Most often, children receiving these treatments improve within 24 hours.

Your child might receive one or more of the following:

• Intravenous gamma-globulin therapy to boost the immune system
• High-dose aspirin to reduce inflammation and fever and prevent clotting
• Anticoagulants to prevent clotting
• Steroids to reduce inflammation

Procedures. In severe cases of damage to the coronary arteries, your doctor may recommend a procedure, including the following:

• Cardiac catheterization. A flexible tube is put into a major blood vessel and threaded to the heart to help doctors see if there is damage to the coronary arteries.
• Angioplasty. A catheter with a balloon tip is inflated to open a blocked artery and restores blood flow through the artery.
• Stent placement. During angioplasty, a small mesh tube is placed to support a narrowed or weakened artery.
• Coronary artery bypass. A healthy artery or vein from another part of the body is used to re-route blood flow around a coronary artery blockage.

Ongoing care. Most children recover completely from the acute phase and do not need further treatment. However, Kawasaki disease is a leading cause of acquired heart disease in the U.S. You child should be monitored with regular echocardiograms, EKGs and stress tests.

If your child is treated with gamma-globulin, wait 11 months before having measles and chicken pox vaccines. The treatment can prevent these vaccines from working well.