Kawasaki disease affects mainly the blood vessels that supply the heart. Without treatment, these vessels can develop aneurysms, which can damage the heart and lead to problems with the blood flow through the heart.
A doctor diagnoses Kawasaki disease by examining your child and taking blood tests. Tests may include: echocardiograms, which show how well the heart is working; and blood tests that check your child’s blood cells.
What is Kawasaki’s disease?
Kawasaki’s disease, also known as mucocutaneous lymph node syndrome, is a rare type of vasculitis (blood vessel inflammation). It happens mostly in kids 6 months to 5 years old and usually affects the arteries that supply blood to the heart. These arteries can get weak and stretched out, which limits how much blood can flow through to nourish tissues and organs. This illness can lead to coronary artery aneurysms (abnormal bulging or swelling in the walls of the arteries). It’s not clear what causes Kawasaki’s disease, but it might be related to certain viruses or genes. It’s not contagious and doesn’t spread from person to person.
Doctors diagnose Kawasaki’s disease by looking at the symptoms and doing a physical exam. A child may have a fever higher than 102.2 degrees Fahrenheit (39 degrees Celsius) for five days or more. Other symptoms include a rash on the main part of the body or in the genital area; enlarged lymph nodes in the neck; very red eyes without a thick discharge; swollen hands and feet, with the skin on fingers and toes often peeling; and a swollen and red tongue. The rash fades within about a week.
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Some children have a fever but fewer than four of the other symptoms — this is called atypical Kawasaki’s disease or incomplete Kawasaki’s disease. They still need treatment to reduce the risk of future heart problems.
The doctor will probably give your child low dose aspirin for six to eight weeks. They might also give your child immunoglobulins, which are antibodies that help fight infections. These are usually given as an IV (intravenous) injection. They’ll also check your child’s heart with an EKG or echocardiogram.
Some children with Kawasaki’s disease have permanent damage to their coronary arteries and might need surgery or other procedures. But if they get prompt treatment, their long-term outlook is good. They’ll need to see a cardiologist for follow-up visits every year or so. This helps them keep track of the condition and look for any heart problems that might have developed. They might need other tests over time, too, like an X-ray or a magnetic resonance imaging (MRI) scan.
Symptoms
Kawasaki disease usually starts in children under the age of five. It is more common in boys than girls. It is also more common in children of Japanese or Oriental descent. But it can affect children of any ethnicity. The main symptom of Kawasaki’s disease is fever higher than 102.2 degrees Fahrenheit (39 degrees Celsius) for five days or more. Other symptoms include a rash on the main part of the body or in the genital area, red eyes without a thick discharge and swollen lips. The skin on the palms of the hands and the soles of the feet may turn red and then start to peel.
In about 6% of cases, children with Kawasaki disease have problems with their heart blood vessels. They may develop a bulge in the wall of one of their heart blood vessels (coronary artery aneurysm) or the walls of their heart valves can become thin and weak. The valves that help blood move in the right direction through the heart may become leaky, allowing blood to flow backwards (regurgitation).
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Heart problems from Kawasaki disease are much less common with prompt diagnosis and treatment. With treatment, coronary artery aneurysms heal within 1 to 2 years. But if the condition isn’t treated, it can lead to death.
A healthcare professional can diagnose Kawasaki’s disease by carefully examining your child and looking at their signs and symptoms. They’ll ask about any medicines your child takes and when they started. They might also order a blood test, which can show that your child has a high level of certain white blood cells and antibodies called erythrocyte sedimentation rate, which indicate inflammation in the blood vessels.
Your child’s healthcare team will usually treat them with intravenous immunoglobulin (IVIG), which can reduce inflammation and prevent long-term heart damage. They’ll also prescribe a high dose of aspirin. They may want to give your child a medication that helps prevent blood clots (blood thinners), such as heparin, during the illness.
Diagnosis
There isn’t a single test to diagnose Kawasaki disease. But doctors can usually make the diagnosis by examining your child and observing their symptoms. The doctor will also order blood tests to look for inflammation and other signs of Kawasaki disease. These may include a high white blood cell count, anemia and inflammation of the blood vessels. An elevated erythrocyte sedimentation rate (a measure of inflammation in the blood) is also often found in children with Kawasaki disease. Blood tests may also check for other possible causes of the swelling, such as hepatitis or pancreatitis.
A large dose of intravenous immunoglobulin is the treatment of choice for Kawasaki disease. This is a special protein fraction of the blood that helps fight germs in the body. It’s very effective in reducing the fever and preventing coronary artery damage in most children with the condition. It’s especially important to start this therapy within 10 days of the first symptoms of Kawasaki disease.
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Children with Kawasaki disease should also be given low-dose aspirin — a medicine that helps prevent blood clots. The aspirin needs to be given under the supervision of a healthcare professional because aspirin can cause Reye’s syndrome in some children. This is a rare, but life-threatening condition in kids who have had the flu or chickenpox.
In some cases, the doctor might do an echocardiogram or electrocardiogram (ECG) to look for problems with your child’s heart. These tests can help detect coronary artery aneurysms and other heart-related complications of Kawasaki disease.
After a child is treated for Kawasaki disease, they should be followed by a healthcare professional who specializes in heart conditions. This person might be a pediatric cardiologist or a specialist in bone and joint diseases called a rheumatologist. Some children might need to be seen more often, and others might need other tests, such as an MRI or CT scan, to watch for the development of aneurysms or other heart-related issues linked to Kawasaki disease. This might be needed for several years after treatment of the disease. These visits might be done in the clinic or at home, depending on your child’s health and needs.
Treatment
Children with Kawasaki disease who start treatment early have a low risk of having long-term heart problems. Getting treatment within 10 days of the start of symptoms is important.
Doctors don’t know what causes Kawasaki disease, but it probably isn’t contagious and doesn’t spread from person to person. It usually affects kids who are 5 years old or younger, and is most common in winter and spring. It is more common in kids of Asian descent, but it can happen to any child. Boys are 1.5 times more likely to get it than girls.
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Symptoms of the illness usually go away on their own in a few weeks. Most kids who get the disease have no lasting effects from it. But in about 2% to 3% of cases, children have long-term damage to their blood vessels that can lead to heart problems later in life. These can include a balloon-like bulge (aneurysm) in the wall of a coronary artery or the valves that control blood flow to and from the heart.
There is no test to diagnose Kawasaki disease, but your child will have a physical exam and blood tests. These can show anemia, a high white blood cell count and inflammation. They can also show how well the heart is working and look for an enlarged coronary artery.
Once the diagnosis is made, doctors give your child an intravenous dose of immune globulins (IVIG). These are antibodies that help fight infections. They also lower the risk of heart complications from Kawasaki disease, including a bulge in the wall of a heart blood vessel (coronary aneurysm). IVIG is given once.
After treatment, most children feel better quickly and don’t have any heart problems. But children who have problems with their coronary arteries can need long-term follow up with a cardiologist, which may involve echocardiograms or other tests to check for aneurysms or other heart issues. Blood thinners may also be needed. The frequency of these follow up visits will depend on how severe the coronary artery damage is at the time of diagnosis and treatment.
