For more information, visit http://www.nhlbi.nih.gov/health/health-topics/topics/kd/

What Is Kawasaki Disease?

Kawasaki (KAH-wah-SAH-ke) disease is a rare childhood disease. It's a form of a condition called vasculitis (vas-kyu-LI-tis). This condition involves inflammation of the blood vessels.

In Kawasaki disease, the walls of the blood vessels throughout the body become inflamed. The disease can affect any type of blood vessel in the body, including the arteries, veins, and capillaries.

Sometimes Kawasaki disease affects the coronary arteries, which carry oxygen-rich blood to the heart. As a result, some children who have Kawasaki disease may develop serious heart problems.

Overview

The cause of Kawasaki disease isn't known. The body's response to a virus or infection combined with genetic factors may cause the disease. However, no specific virus or infection has been found, and the role of genetics isn't known.

The disease can't be passed from one child to another. Your child won't get it from close contact with a child who has the disease. Also, if your child has the disease, he or she can't pass it to another child.

Kawasaki disease affects children of all races and ages and both genders. It occurs most often in children of Asian and Pacific Island descent. The disease is more likely to affect boys than girls. Most cases occur in children younger than 5 years old.

One of the main symptoms of Kawasaki disease is a fever that lasts longer than 5 days. The fever remains high even after treatment with standard childhood fever medicines.

Children who have the disease also may have red eyes, red lips, and redness on the palms of their hands and soles of their feet. These are all signs of inflamed blood vessels.

Early treatment helps reduce the risk of Kawasaki disease affecting the coronary arteries and causing serious problems.

Outlook

Kawasaki disease can't be prevented. However, most children who have the disease usually recover within weeks of getting symptoms. Further problems are rare.

The disease affects some children's coronary arteries, which can cause serious problems. These children need long-term care and treatment.

Researchers continue to look for the cause of Kawasaki disease and better ways to diagnose and treat it. They also hope to learn more about long-term health risks, if any, for people who have had the disease.

Other Names for Kawasaki Disease

Kawasaki syndrome
Mucocutaneous lymph node syndrome

What Causes Kawasaki Disease?

Kawasaki disease can't be passed from one child to another. Your child won't get it from close contact with a child who has the disease. Also, if your child has the disease, he or she can't pass it to another child.

Who Is at Risk for Kawasaki Disease?

Kawasaki disease affects children of all races and ages and both genders. It occurs most often in children of Asian and Pacific Island descent.

The disease is more likely to affect boys than girls. Most cases occur in children younger than 5 years old. Kawasaki disease is rare in children older than 8.

What Are the Signs and Symptoms of Kawasaki Disease?

Major Signs and Symptoms

One of the main symptoms during the early part of Kawasaki disease, called the acute phase, is fever. The fever lasts longer than 5 days. It remains high even after treatment with standard childhood fever medicines.

Other classic signs of the disease are:

Swollen lymph nodes in the neck
A rash on the mid-section of the body and in the genital area
Red, dry, cracked lips and a red, swollen tongue
Red, swollen palms of the hands and soles of the feet
Redness of the eyes

Other Signs and Symptoms

During the acute phase, your child also may be irritable and have a sore throat, joint pain, diarrhea, vomiting, and stomach pain.

Within 2 to 3 weeks of the start of symptoms, the skin on your child's fingers and toes may peel, sometimes in large sheets.

How Is Kawasaki Disease Diagnosed?

Kawasaki disease is diagnosed based on your child's signs and symptoms and the results from tests and procedures.

Specialists Involved

Pediatricians often are the first to suspect a child has Kawasaki disease. Pediatricians are doctors who specialize in treating children.

If the disease has affected your child's coronary (heart) arteries, a pediatric cardiologist will confirm the diagnosis and give ongoing treatment. Pediatric cardiologists treat children who have heart problems.

Other specialists also may be involved in treating children who have Kawasaki disease.

Signs and Symptoms

The doctor will check your child for the classic signs and symptoms of Kawasaki disease.

The doctor will rule out other diseases that cause similar symptoms. These diseases include Rocky Mountain spotted fever, scarlet fever, and juvenile rheumatoid arthritis.

Generally, your child will be diagnosed with Kawasaki disease if he or she has a fever that lasts longer than 5 days plus four other classic signs or symptoms of the disease.

However, not all children have classic signs and symptoms of Kawasaki disease. Tests and procedures can help confirm whether a child has the disease.

Tests and Procedures

Echocardiography

If the doctor thinks that your child has Kawasaki disease, he or she will likely recommend echocardiography (EK-o-kar-de-OG-ra-fee), or echo. This painless test uses sound waves to create pictures of the heart and coronary arteries.

Echo also can help show the disease's effects over time, if any, on your child's coronary arteries. Often, the disease's effects on the coronary arteries don't show up until the second or third week after the first symptoms appear. Thus, this test is done regularly after the diagnosis.

Some children who have Kawasaki disease don't have the classic signs and symptoms of the acute phase. Doctors may not diagnose these children until 2 to 3 weeks after the onset of the disease. This is when another common sign of Kawasaki disease occurs—peeling of the skin on the fingers and toes.

If your child is diagnosed at this point, he or she will likely need echo right away to see whether the disease has affected the coronary arteries.

Other Diagnostic Tests

Doctors also use other tests to help diagnose Kawasaki disease, such as:

Blood tests. The results from blood tests can show whether the body's blood vessels are inflamed.
Chest x ray. This painless test creates pictures of structures inside the chest, such as the heart and lungs. A chest x ray can show whether Kawasaki disease has affected the heart.
EKG (electrocardiogram). This simple test detects and records the heart's electrical activity. An EKG can show whether Kawasaki disease has affected the heart.

How Is Kawasaki Disease Treated?

Medicines are the main treatment for Kawasaki disease. Rarely, children whose coronary (heart) arteries are affected may need medical procedures or surgery.

The goals of treatment include:

Reducing fever and inflammation to improve symptoms
Preventing the disease from affecting the coronary arteries

Initial Treatment

Kawasaki disease can cause serious health problems. Thus, your child will likely be treated in a hospital, at least for the early part of treatment.

The standard treatment during the disease's acute phase is high-dose aspirin and immune globulin. Immune globulin is a medicine that's injected into a vein.

Most children who receive these treatments improve greatly within 24 hours. For a small number of children, fever remains. These children may need a second round of immune globulin.

At the start of treatment, your child will receive high doses of aspirin. As soon as his or her fever goes away, a low dose of aspirin is given. The low dose helps prevent blood clots, which can form in the inflamed small arteries.

Most children treated for Kawasaki disease fully recover from the acute phase and don't need any further treatment. They should, however, follow a healthy diet and adopt healthy lifestyle habits. Taking these steps can help lower the risk of future heart disease. (Following a healthy lifestyle is advised for all children, not just those who have Kawasaki disease.)

Children who have had immune globulin should wait 11 months before having the measles and chicken pox vaccines. Immune globulin can prevent those vaccines from working well.

Long-Term Care and Treatment

If Kawasaki disease has affected your child's coronary arteries, he or she will need ongoing care and treatment.

It's best if a pediatric cardiologist provides this care to reduce the risk of severe heart problems. A pediatric cardiologist is a doctor who specializes in treating children who have heart problems.

Medicines and Tests

When Kawasaki disease affects the coronary arteries, they may expand and twist. If this happens, your child's doctor may prescribe blood-thinning medicines (for example, warfarin). These medicines help prevent blood clots from forming in the affected coronary arteries.

Blood-thinning medicines usually are stopped after the coronary arteries heal. Healing may occur about 18 months after the acute phase of the disease.

In a small number of children, the coronary arteries don't heal. These children likely will need routine tests, such as:

Echocardiography. This test uses sound waves to create images of the heart.
EKG (electrocardiogram). This test detects and records the heart's electrical activity.
Stress test. This test provides information about how the heart works during physical activity or stress.

Medical Procedures and Surgery

Rarely, a child who has Kawasaki disease may need cardiac catheterization (KATH-eh-ter-ih-ZA-shun). Doctors use this procedure to diagnose and treat some heart conditions.

A flexible tube called a catheter is put into a blood vessel in the arm, groin (upper thigh), or neck and threaded to the heart. Through the catheter, doctors can perform tests and treatments on the heart.

Very rarely, a child may need to have other procedures or surgery if inflammation narrows his or her coronary arteries and blocks blood flow to the heart. Coronary angioplasty (AN-jee-oh-plas-tee), stent placement, or coronary artery bypass grafting (CABG) may be used.

Coronary angioplasty restores blood flow through narrowed or blocked coronary arteries. A thin tube with a balloon on the end is inserted into a blood vessel in the arm or groin. The tube is threaded to the narrowed or blocked coronary artery. Then, the balloon is inflated to widen the artery and restore blood flow.

A stent (small mesh tube) may be placed in the coronary artery during angioplasty. This device helps support the narrowed or weakened artery. A stent can improve blood flow and prevent the artery from bursting.

Rarely, a child may need to have CABG. This surgery is used to treat blocked coronary arteries. During CABG, a healthy artery or vein from another part of the body is connected, or grafted, to the blocked coronary artery.

The grafted artery or vein bypasses (that is, goes around) the blocked part of the coronary artery. This improves blood flow to the heart.

How Can Kawasaki Disease Be Prevented?

Kawasaki disease can't be prevented. However, most children who have the disease recover—usually within weeks of getting signs and symptoms. Further problems are rare.

Living With Kawasaki Disease

Most children who have Kawasaki disease recover—usually within weeks of getting symptoms. Further problems are rare. Early treatment reduces the risk of serious problems.

What To Expect After Treatment

Most children who are treated for Kawasaki disease fully recover from the acute phase. They don't need further treatment.

They should, however, follow a healthy diet and adopt healthy lifestyle habits. Taking these steps can help lower their risk of future heart disease. (Following a healthy lifestyle is advised for all children, not just those who have Kawasaki disease).

Children treated with immune globulin should wait 11 months before having measles and chicken pox vaccines. Immune globulin can prevent these vaccines from working well.

Ongoing Health Care Needs

If Kawasaki disease has affected your child's coronary arteries, he or she will need ongoing care and treatment. It's best if a pediatric cardiologist provides this care to reduce the risk of severe heart problems. A pediatric cardiologist is a doctor who specializes in treating children who have heart problems.

Support Groups

Joining a support group may help you adjust to caring for a child who has Kawasaki disease. You can see how other parents have coped with the disease. Ask your child's doctor about local support groups or check with an area medical center.

Clinical Trials

The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.

NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.

Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.

By taking part in a clinical trial, your child can gain access to new treatments before they're widely available. Your child also will have the support of a team of health care providers, who will likely monitor his or her health closely. Even if your child doesn't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.

Children (aged 18 and younger) get special protection as research subjects. Almost always, parents must give legal consent for their child to take part in a clinical trial.

When researchers think that a trial's potential risks are greater than minimal, both parents must give permission for their child to enroll. Also, children aged 7 and older often must agree (assent) to take part in clinical trials.

If you agree to have your child take part in a clinical trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw your child from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.

For more information about clinical trials related to Kawasaki disease, talk with your child's doctor. For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.

You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:

Links to Other Information About Kawasaki Disease

NHLBI Resources

Vasculitis (Health Topics)

Non-NHLBI Resources

Kawasaki Disease (MedlinePlus)

Clinical Trials

Children and Clinical Studies
Clinical Trials (Health Topics)
Current Research (ClinicalTrials.gov)
NHLBI Clinical Trials
NIH Clinical Research Trials and You (National Institutes of Health)
ResearchMatch (funded by the National Institutes of Health)