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Kawasaki Disease

(Kawasaki's Disease)


Christopher P. Raab

, MD, Sidney Kimmel Medical College at Thomas Jefferson University

Reviewed/Revised Feb 2023 | Modified Sep 2023
Topic Resources

Kawasaki disease causes inflammation of blood vessels throughout the body.

  • The cause of Kawasaki disease is unknown but may be associated with an infection.

  • Children typically have fever, rash, and a red strawberry tongue, and some develop heart complications that rarely can be fatal.

  • The diagnosis is based on established criteria.

  • With prompt treatment, nearly all children recover.

  • Children are given high doses of immune globulin and aspirin.

Kawasaki disease causes inflammation in the walls of blood vessels (vasculitis Overview of Vasculitis Vasculitic disorders are caused by inflammation of the blood vessels (vasculitis). Vasculitis can be triggered by certain infections or drugs or can occur for unknown reasons. People may have... read more Overview of Vasculitis ) throughout the body. Inflammation of blood vessels in the heart causes the most serious problems. The inflammation also can spread to other parts of the body such as the pancreas and kidneys.

Most children with Kawasaki disease are between 1 and 5 years of age, although infants and older children and adolescents can be affected. Roughly one and one half times as many boys as girls are affected. The illness is more common among children of Japanese descent.

Several thousand cases of Kawasaki disease are estimated to occur in the United States every year. Kawasaki disease occurs year-round but most often in spring or winter.

The cause of Kawasaki disease is unknown, but evidence suggests a virus or other infectious organism triggers an abnormal immune system response in genetically predisposed children.

Symptoms of Kawasaki Disease

The illness begins with fever that is usually above 102.2° F (39° C) and that rises and falls over 1 to 3 weeks. The child's temperature does not return to normal unless medications that lower the temperature (such as acetaminophen and ibuprofen) are given. Within a day or two, the eyes become red but do not have any discharge.

The skin on the fingers and toes begins to peel about 10 days after the illness starts. The lymph nodes in the neck are often swollen and slightly tender. The illness may last from 2 to 12 weeks or longer.

Complications of Kawasaki disease

If children are not treated, heart problems may develop, usually beginning 1 to 4 weeks after the illness starts. A few children develop the most serious heart problem, a bulge in the wall of a coronary artery (coronary artery aneurysm Overview of Aortic Aneurysms and Aortic Dissection The aorta, which is about 1 inch (2.5 centimeters) in diameter, is the largest artery of the body. It receives oxygen-rich blood from the left ventricle of the heart and distributes it to all... read more ). These aneurysms can rupture or provoke a blood clot, leading to a heart attack and sudden death. Treatment greatly reduces the risk of heart complications.

Other problems may include painful inflammation of the tissues lining the brain (meningitis), ears, eyes, liver, joints, urethra, and gallbladder. These symptoms eventually resolve without causing permanent damage.

Diagnosis of Kawasaki Disease

  • Established criteria

  • Electrocardiography and ultrasonography of the heart

  • Laboratory tests

Doctors diagnose Kawasaki disease when children have at least 4 out of 5 defined symptoms (see sidebar ).

How Do Doctors Diagnose Kawasaki Disease?

Doctors diagnose children with Kawasaki disease if they have had a fever for 5 days or more and if they have at least 4 of the following 5 symptoms:

  • Red eyes with no discharge

  • Reddened, dry, cracked lips and a red strawberry tongue

  • Swelling, redness, and peeling of the hands and feet

  • Red, patchy rash over the trunk

  • Swollen, tender lymph nodes in the neck

Blood tests and cultures of the blood and throat are also done to rule out other disorders that cause similar symptoms (such as measles, scarlet fever, juvenile idiopathic arthritis, and multisystem inflammatory syndrome in children [MIS-C]).

Doctors who specialize in treating children's heart disorders (pediatric cardiologists) or infectious diseases are often consulted.

Once Kawasaki disease is diagnosed, children have electrocardiography Electrocardiography Electrocardiography (ECG) is a quick, simple, painless medical test that measures the heart’s electrical impulses. During an ECG, the heart's electrical impulses are measured, amplified, and... read more Electrocardiography (ECG) and ultrasonography of the heart (echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography is a type of medical imaging that uses high-frequency (ultrasound) waves to produce a moving image of internal organs and other tissues. Echocardiography is ultrasonography of... read more Echocardiography and Other Ultrasound Procedures ) to look for coronary artery aneurysms, leaking heart valves, inflammation of the sac that surrounds the heart (pericarditis Overview of Pericardial Disease Pericardial disease affects the pericardium, which is the flexible two-layered sac that envelops the heart. The pericardium helps keep the heart in position, helps prevent the heart from overfilling... read more Overview of Pericardial Disease ), or inflammation of the heart muscle (myocarditis Myocarditis Myocarditis is inflammation of the muscle tissue of the heart (myocardium) that causes tissue death. Myocarditis may be caused by many disorders, including infection, toxins and drugs that affect... read more ). Sometimes abnormalities do not appear right away, so these tests are repeated 2 to 3 weeks, 6 to 8 weeks, and perhaps 6 to 12 months after symptoms began. If ECG or echocardiography test results are abnormal, doctors may do a stress test Stress Testing Stress testing measures the heart's function with electrocardiography (ECG) while the heart is under stress, either from exercise or from a medication used to stress the heart. Stressing the... read more Stress Testing . If an aneurysm is seen during echocardiography, children may have heart catheterization Cardiac Catheterization and Coronary Angiography Cardiac catheterization is a procedure that can measure heart function through a catheter inserted into a vein or artery and guided into the heart. Coronary angiography, which can be done during... read more Cardiac Catheterization and Coronary Angiography .

Treatment of Kawasaki Disease

  • High doses of immune globulin and aspirin

Treatment for Kawasaki disease is started as soon as possible. Treatment given within the first 10 days of symptoms significantly reduces the risk of coronary artery damage and speeds the resolution of fever, rash, and discomfort.

For 1 to 4 days, high doses of immune globulin are given by vein, and high doses of aspirin are given by mouth. As soon as the child has no fever for 4 to 5 days, the dose of aspirin is lowered but still given for at least 8 weeks from the beginning of illness. If there are no coronary artery aneurysms and signs of inflammation are gone, aspirin may be stopped. However, children with coronary artery abnormalities require long-term treatment with aspirin.

Children with large coronary aneurysms may be treated with medications that prevent the blood from clotting (anticoagulants).

Prognosis for Kawasaki Disease

With treatment, children recover completely if their coronary arteries are not affected. For children with coronary artery problems, the outcome varies with the severity of the disease. However, with early treatment, almost no children with Kawasaki disease in the United States die.

Without treatment, some children die. Of the children who die, death nearly always occurs in the first 6 months but can occur as long as 10 years later.

About two thirds of the aneurysms go away within 1 year. Large aneurysms are more likely to remain. However, even when aneurysms go away, children have an increased risk of heart problems in adulthood.

Drugs Mentioned In This Article

Generic Name Select Brand Names
7T Gummy ES, Acephen, Aceta, Actamin, Adult Pain Relief, Anacin Aspirin Free, Aphen, Apra, Children's Acetaminophen, Children's Pain & Fever , Children's Pain Relief, Comtrex Sore Throat Relief, ED-APAP, ElixSure Fever/Pain, Feverall, Genapap, Genebs, Goody's Back & Body Pain, Infantaire, Infants' Acetaminophen, LIQUID PAIN RELIEF, Little Fevers, Little Remedies Infant Fever + Pain Reliever, Mapap, Mapap Arthritis Pain, Mapap Infants, Mapap Junior, M-PAP, Nortemp, Ofirmev, Pain & Fever , Pain and Fever , PAIN RELIEF , PAIN RELIEF Extra Strength, Panadol, PediaCare Children's Fever Reducer/Pain Reliever, PediaCare Children's Smooth Metls Fever Reducer/Pain Reliever, PediaCare Infant's Fever Reducer/Pain Reliever, Pediaphen, PHARBETOL, Plus PHARMA, Q-Pap, Q-Pap Extra Strength, Silapap, Triaminic Fever Reducer and Pain Reliever, Triaminic Infant Fever Reducer and Pain Reliever, Tylenol, Tylenol 8 Hour, Tylenol 8 Hour Arthritis Pain, Tylenol 8 Hour Muscle Aches & Pain, Tylenol Arthritis Pain, Tylenol Children's, Tylenol Children's Pain+Fever, Tylenol CrushableTablet, Tylenol Extra Strength, Tylenol Infants', Tylenol Infants Pain + Fever, Tylenol Junior Strength, Tylenol Pain + Fever, Tylenol Regular Strength, Tylenol Sore Throat, XS No Aspirin, XS Pain Reliever
Advil, Advil Children's, Advil Children's Fever, Advil Infants', Advil Junior Strength, Advil Migraine, Caldolor, Children's Ibuprofen, ElixSure IB, Genpril , Ibren , IBU, Ibupak, Midol, Midol Cramps and Body Aches, Motrin, Motrin Children's, Motrin IB, Motrin Infants', Motrin Junior Strength, Motrin Migraine Pain, PediaCare Children's Pain Reliever/Fever Reducer IB, PediaCare Infants' Pain Reliever/Fever Reducer IB, Samson-8, Toxicology Saliva Collection
Anacin Adult Low Strength, Aspergum, Aspir-Low, Aspirtab , Aspir-Trin , Bayer Advanced Aspirin, Bayer Aspirin, Bayer Aspirin Extra Strength, Bayer Aspirin Plus, Bayer Aspirin Regimen, Bayer Children's Aspirin, Bayer Extra Strength, Bayer Extra Strength Plus, Bayer Genuine Aspirin, Bayer Low Dose Aspirin Regimen, Bayer Womens Aspirin , BeneHealth Aspirin, Bufferin, Bufferin Extra Strength, Bufferin Low Dose, DURLAZA, Easprin , Ecotrin, Ecotrin Low Strength, Genacote, Halfprin, MiniPrin, St. Joseph Adult Low Strength, St. Joseph Aspirin, VAZALORE, Zero Order Release Aspirin, ZORprin
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