 Kawasaki Disease
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Kawasaki disease is the most common form of vasculitis which primarily affects children. The disease produces irritation and inflammation of many tissues of the body, including the hands, feet, whites of the eyes, mouth, lipsand throat. High fever and swelling of the lymph nodes in the neck also are characteristic of this illness. The inflammation is uncomfortable, but resolves with time. The main threat from Kawasaki disease comes from its effect on the heart and blood vessels. Heart-related complications can be temporary or may affect the child long-term. The heart, particularly the coronary arteries, is affected in as many as 20 percent of children with Kawasaki disease.
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Kawasaki Disease was first described by a doctor in Japan in 1967. Another name for Kawasaki disease is mucocutaneous lymph node syndrome.
For in-depth visual information on this condition, visit the Children's Hospital Boston Multimedia Library.
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It is not clear what causes Kawasaki disease. Scientists believe a virus may be responsible, but current research is still underway. Kawasaki disease does not appear to be contagious, nor does it appear to be hereditary. It was once thought that Kawasaki disease was linked to recent rug or carpet cleaning, but no studies have shown this to be a cause of the disease.
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It is rare for more than one child in a family to develop the disease. As a result, less than 2 percent of persons with Kawasaki disease develop the disease more than once.
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Kawasaki disease occurs more often in Japan than in any other country. In the United States, children of Asian or Asian American heritage are affected more often than other races, although Kawasaki disease can occur in any racial or ethnic group.
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The vast majority of children who develop Kawasaki disease are under age 5. The average age child seen with the illness is 2 years old. Boys are about 1.5 times as likely to get Kawasaki disease as girls.
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Kawasaki disease is fairly common in the United States. According to the American Heart Association, the illness is a major cause of heart disease in children. About 4,000 cases occur in the United States each year, and the incidence is on the rise. Kawasaki disease has replaced acute rheumatic fever as the leading cause of acquired heart disease in children in the United States and Japan.
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Kawasaki disease can be a very uncomfortable illness, since it causes fever as well as irritation and inflammation in many tissues of the body. These symptoms usually run their course and resolve within a few weeks. The primary concern with Kawasaki disease is heart and blood vessel involvement.
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The coronary arteries are the vessels that provide the heart muscle with an oxygen-rich blood supply. Kawasaki disease can weaken the wall of one or more of the coronary arteries, causing it to bulge or balloon out. This weakened, ballooned area is called an aneurysm.
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Blood clots can form in the ballooned area and possibly block blood flow through the coronary artery. When this happens, the heart muscle will no longer receive an adequate supply of oxygen-rich blood, and the heart muscle can be damaged. On rare occasions, the aneurysm can burst.
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The illness may also cause the heart muscle (myocardium), and the membrane covering the heart (pericardium) to be irritated and inflamed. Irregular heart rhythms and heart valve problems also may occur with Kawasaki disease.
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In most cases, the effects on the heart caused by Kawasaki disease are temporary, and resolve within five or six weeks. Coronary artery problems, however, may sometimes persist for longer periods of time.
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The following are the most common symptoms of Kawasaki disease. Each child may experience symptoms differently. Symptoms may include:
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- moderate to high fever (101 degrees F to 104 degrees F) that rises and falls for up to three weeks
- irritability
- swollen lymph glands in the neck
- spotty, bright red rash on the back, chest, abdomen and/or groin
- bloodshot eyes
- sensitivity to light
- swollen, coated tongue
- dry, red, cracked lips
- dark red interior surfaces of the mouth
- red, swollen palms of hands and soles of feet
- peeling skin around the nailbeds, hands or feet
- swollen, painful joints
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The symptoms of Kawasaki disease may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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Your child's physician will obtain a medical history and perform a physical examination. Several of the above mentioned symptoms need to be present for your child's physician to consider Kawasaki disease as the cause for the illness.
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Diagnostic tests may also include:
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- Electrocardiogram (ECG or EKG) — A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias) and detects heart muscle damage.
- Echocardiogram — A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor, that produce a moving picture of the heart and heart valves.
- X-ray — A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film.
- Complete Blood Count (CBC) — A measurement of the size, number and maturity of different blood cells in a specific volume of blood. The physician will look for an elevation in the numbers of white blood cells, which normally multiply in the presence of infection.
- Erythrocyte Sedimentation Rate (ESR or sed rate) — A measurement of how quickly red blood cells fall to the bottom of a test tube. When swelling and inflammation are present, the blood's proteins clump together and become heavier than normal. Thus, when measured, they fall and settle faster at the bottom of the test tube. Generally, the faster the blood cells fall, the more severe the inflammation.
- Urinalysis — Testing of a urine sample for protein, red blood cells, white blood cells, or casts to indicate kidney disease associated with several rheumatic diseases.
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Specific treatments for Kawasaki disease will be determined by your child's physician based on:
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- your child's age, overall health and medical history
- your child's tolerance for specific medications, procedures or therapies
- extent of the disease
- expectations for the course of the disease
- your opinion or preference
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Prior to the diagnosis of Kawasaki disease by your child's physician, the goal of treatment is to provide comfort for your child. Fever reduction and increasing fluids are very helpful. Acetaminophen or ibuprofen is often used for fever.
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Once the diagnosis of Kawasaki disease is made, your child's physician may prescribe aspirin to help decrease the inflammation that the illness produces, as well as to prevent clots from forming. Always check with your child's physician before giving your child aspirin. Some children benefit from an intravenous (IV) medication called gamma globulin, which may decrease the risk of the heart being affected. Your child may need to stay in the hospital for a few days if IV gamma globulin is prescribed.
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Currently, there is no known way to prevent Kawasaki disease.
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About 80 percent of all children have a full recovery after Kawasaki disease resolves. If an aneurysm is detected, echocardiograms will be repeated periodically, sometimes for several years after the illness. Some heart problems may not be evident right away, so it is important to keep follow-up appointments with your child's physician, even if your child is feeling well.
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Complementing the Cardiovascular Program of Children's Hospital Boston is the nation's most intensive clinical and basic research program focused on pediatric heart disease. Children's is a world leader in opening new avenues of "translational research," bringing laboratory advances to the bedside and doctor's office as quickly as possible. All senior medical staff members of the Cardiovascular Program participate in clinical research activities.
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Contact Children's Hospital Boston Cardiovascular Program physicians for a second opinion.
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