Kawasaki Disease

What is Kawasaki disease?

Kawasaki disease (also called mucocutaneous lymph node syndrome) is a children’s illness that causes inflammation of the blood vessels in many parts of the body, including the heart. It can also affect your child’s lymph nodes and the mucous membranes inside the mouth, nose and throat.

The immediate effects of Kawasaki disease are rarely serious and most children recover in six to eight weeks. However, early diagnosis and treatment is critical to preventing long-term heart complications, which can be seen as early as two weeks after the condition's onset.

Complications include damage to the coronary arteries that supply the heart with oxygen, and damage to the heart itself.

Kawasaki disease is relatively common and tends to occur among children less than five years old. In the United States, Kawasaki disease is a major cause of heart disease in children.

What causes Kawasaki disease?

We don’t yet fully understand the cause of Kawasaki disease. Some researchers believe that an infection triggers an autoimmune response. Some children may also be genetically predisposed to the disease.

What are the symptoms of Kawasaki disease?

Symptoms of Kawasaki disease generally appear in phases. Initial symptoms include:

  • High fever for more than three days
  • Rash, including in genital area
  • Swelling and redness of the hands and feet
  • Irritation and redness of the whites of the eyes, without discharge
  • Swollen lymph glands in the neck
  • Chapped, cracked lips
  • Swollen, red tongue
  • Irritability

Later, or second phase, symptoms include:

  • Peeling skin on hands and feet
  • Joint pain
  • Diarrhea or vomiting
  • Abdominal pain

How is Kawasaki disease diagnosed?

There is no definitive test for Kawasaki disease. Your doctor will conduct a full medical exam and evaluate your child’s symptoms. If Kawasaki disease is suspected, your doctor will schedule some tests, including:

  • Blood and urine tests, which may indicate inflammation or rule out other diseases.
  • Electrocardiogram (ECG or EKG), which measures the electrical impulses of your child’s heartbeat to look for heart rhythm problems caused by Kawasaki disease.
  • Echocardiogram, an ultrasound imaging test that looks at the structure and function of the heart.

How we treat Kawasaki disease

The Kawasaki Disease Program at Columbia is led by a pediatric rheumatologist with expertise in this disease, who works closely with pediatric cardiologists and infectious disease specialists throughout treatment and follow up. Most children fully recover from Kawasaki disease, especially when treatment begins within 10 days of the onset of symptoms.

Your child’s treatment will involve a short hospital stay at NewYork-Presbyterian Morgan Stanley Children’s Hospital. The initial phase of treatment involves intravenous gamma globulin (IVIG), which is administered intravenously (through the vein). IVIG helps reduce fever and prevent damage to the heart and blood vessels.

We will also closely monitor your child’s heart during his or her hospital stay.

Depending on how your child responds to IVIG, we may also administer intravenous corticosteroids and/or the immunosuppressive drug, infliximab.

For children who develop coronary problems, we generally prescribe a low dose of aspirin for six to eight weeks, or longer.

Kawasaki disease and heart problems

Most children recover fully from Kawasaki disease. If your child develops heart problems due to Kawasaki disease, we will refer you to a pediatric cardiologist for long-term follow up care.

The most serious heart complication from Kawasaki disease is a coronary artery aneurysm—a dilation of part of the artery responsible for bringing oxygen to the heart. If your child develops a coronary artery aneurysm, follow up will include regular imaging tests, including echocardiograms. We may also prescribe blood thinners to prevent clots from forming in the arteries.