Kawasaki disease
Mucocutaneous lymph node syndrome; Infantile polyarteritis; Kawasaki syndromeKawasaki disease is a rare condition that involves inflammation of the blood vessels. It occurs in children.
Causes
Kawasaki disease occurs most often in Japan, where it was first discovered. The disease is seen more often in boys than in girls. Most of the children who develop this condition are younger than age 5.
Kawasaki disease is not well understood and the cause is yet unknown. It may be an autoimmune disorder. The problem affects the mucous membranes, lymph nodes, walls of the blood vessels, and the heart.
Autoimmune disorder
An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...
Read Article Now Book Mark ArticleSymptoms
Kawasaki disease often begins with a fever of 102°F (38.9°C) or higher that does not go away. The fever is often as high as 104°F (40°C). A fever lasting at least 5 days is a common sign of the disorder. The fever may last for up to 2 weeks. The fever often does not come down with normal doses of acetaminophen (Tylenol) or ibuprofen.
Fever
Fever is the temporary increase in the body's temperature in response to a disease or illness. A child has a fever when the temperature is at or abov...
Read Article Now Book Mark ArticleOther symptoms often include:
- Bloodshot or red eyes (without pus or drainage)
- Bright red, chapped, or cracked lips
- Red mucous membranes in the mouth
- "Strawberry" tongue, with white coating on the tongue, or visible red bumps on the back of the tongue
- Red, swollen palms of the hands and the soles of the feet
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Skin rashes on the middle of the body, not blister-like
Skin rashes
Rashes involve changes in the color, feeling or texture of your skin.
Read Article Now Book Mark Article - Peeling skin in the genital area, hands, and feet (mostly around the nails, palms, and soles)
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Swollen lymph nodes in the neck (often only one lymph node is swollen)
Swollen lymph nodes
Lymph nodes are present throughout your body. They are an important part of your immune system. Lymph nodes help your body recognize and fight germ...
Read Article Now Book Mark Article - Joint pain and swelling, often on both sides of the body
Additional symptoms may include:
- Irritability
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Diarrhea, vomiting, and abdominal pain
Vomiting
Nausea is feeling an urge to vomit. It is often called "being sick to your stomach. "Vomiting or throwing-up forces the contents of the stomach up t...
Read Article Now Book Mark ArticleAbdominal pain
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
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Cough and runny nose
Cough
Coughing is an important way to keep your throat and airways clear. But too much coughing may mean you have a disease or disorder. Some coughs are d...
Read Article Now Book Mark ArticleRunny nose
A stuffy or congested nose occurs when the tissues lining the nose become swollen. The swelling is due to inflamed blood vessels. The problem may a...
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Exams and Tests
Tests alone cannot diagnose Kawasaki disease. Most of the time, the health care provider will diagnose the disease when a child has most of the common symptoms.
In some cases, a child may have a fever that lasts more than 5 days, but not all the common symptoms of the disease. These children may be diagnosed with atypical Kawasaki disease.
All children with fever lasting more than 5 days should be checked for Kawasaki disease by a provider. Children with the disease need early treatment for a good outcome.
The following tests may be done:
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Chest x-ray
Chest x-ray
A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.
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Complete blood count
Complete blood count
A complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
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C-reactive protein (CRP)
C-reactive protein
C-reactive protein (CRP) is produced by the liver. The level of CRP rises when there is inflammation in the body. It is one of a group of proteins,...
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Erythrocyte sedimentation rate (ESR)
Erythrocyte sedimentation rate (ESR
ESR stands for erythrocyte sedimentation rate. It is commonly called a "sed rate. "It is a test that indirectly measures the level of certain protei...
Read Article Now Book Mark Article - Ferritin
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Serum albumin
Serum albumin
Albumin is a protein made by the liver. A serum albumin test measures the amount of this protein in the clear liquid portion of the blood. Albumin c...
Read Article Now Book Mark Article - Serum transaminase
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Urinalysis - may show pus in the urine or protein in the urine
Urinalysis
Urinalysis is the physical, chemical, and microscopic examination of urine. It involves a number of tests to detect and measure various compounds th...
Read Article Now Book Mark ArticleProtein in the urine
The urine protein dipstick test measures the presence of all proteins, including albumin, in a urine sample. Albumin and protein can also be measured...
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Throat culture for streptococcus
Throat culture
A throat swab culture is a laboratory test that is done to identify germs that may cause infection in the throat. It is most often used to diagnose ...
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Echocardiogram
Echocardiogram
An echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...
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Electrocardiogram
Electrocardiogram
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
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Tests such as ECG and echocardiography are done to look for signs of myocarditis, pericarditis, and inflammation of the coronary arteries. Arthritis and aseptic meningitis can also occur.
Myocarditis
Myocarditis is inflammation of the heart muscle. The condition is called pediatric myocarditis when it occurs in children.
Read Article Now Book Mark ArticlePericarditis
Pericarditis is a condition in which the sac-like covering around the heart (pericardium) becomes inflamed.
Read Article Now Book Mark ArticleArthritis
Arthritis is inflammation or degeneration of one or more joints. A joint is the area where 2 bones meet. There are more than 100 different types of...
Read Article Now Book Mark ArticleTreatment
Children with Kawasaki disease need hospital treatment. Treatment must be started right away to prevent damage to the coronary arteries and heart.
Intravenous gamma globulin is the standard treatment. It is given in high doses as a single infusion. The child's condition often gets much better within 24 hours of treatment with IV gamma globulin.
High-dose aspirin is often given along with IV gamma globulin.
Even with standard treatment, up to 1 in 4 children may still develop problems in their coronary arteries. In sicker children or those with signs of heart disease, adding corticosteroids is recommended. Tumor necrosis factor (TNF) inhibitors such as infliximab (Remicade) or etanercept (Enbrel) are not recommended for initial treatment. However, there still needs to be better tests to tell which children will benefit from these medicines.
Outlook (Prognosis)
Most children can recover fully when the disease is caught and treated early. About 1 in 100 children die from heart problems caused by the disease. People who have had Kawasaki disease should have an echocardiogram every 1 to 2 years to check for heart problems.
Possible Complications
Kawasaki disease can cause inflammation of blood vessels in the arteries, especially the coronary arteries. This can lead to aneurysm. Rarely, it can lead to a heart attack at a young age or later in life.
When to Contact a Medical Professional
Call your provider if symptoms of Kawasaki disease develop. Cracked, red lips and swelling and redness develop in the affected areas such as the palms and soles of the feet. If these problems occur along with an ongoing high fever that does not come down with acetaminophen or ibuprofen, your child should be checked by a provider.
Prevention
There are no known ways to prevent this disorder.
References
Abrams JY, Belay ED, Uehara R, Maddox RA, Schonberger LB, Nakamura Y. Cardiac complications, earlier treatment, and initial disease severity in Kawasaki disease. J Pediatr. 2017;188:64-69. PMID: 28619520 pubmed.ncbi.nlm.nih.gov/28619520/.
American Academy of Pediatrics. Kawasaki disease. In: Committee on Infectious Diseases, American Academy of; Pediatrics, Kimberlin DW, Barnett ED, Lynfield R, Sawyer MH, eds. Red Book: 2021-2024 Report of the Committee on Infectious Diseases. 32nd ed. Itasca, IL: American Academy of Pediatrics; 2021.
McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017;135(17):e927-e999. PMID: 28356445 pubmed.ncbi.nlm.nih.gov/28356445/.
Nyhan A. Cardiology. In: Kleinman K, Mcdaniel L, Molloy M, eds. Harriet Lane Handbook, The. 22nd ed. Philadelphia, PA: Elsevier; 2021:chap 7.
Xue LJ, Wu R, Du GL, et al. Effect and safety of TNF inhibitors in immunoglobulin-resistant Kawasaki Disease: a meta-analysis. Clin Rev Allergy Immunol. 2017;52(3):389-400. PMID: 27550227 pubmed.ncbi.nlm.nih.gov/27550227/.
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Kawasaki disease - edema of the hand - illustration
Kawasaki disease can produce various rashes and skin manifestations. This photograph shows mild swelling (edema) and redness associated with the disease. The hands and feet may be tender to touch and the skin may peel once the redness begins to fade.
Kawasaki disease - edema of the hand
illustration
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Kawasaki's disease - peeling of the fingertips - illustration
Kawasaki's disease causes various rashes and manifestations of the mucous membranes and skin. This photograph shows the skin peeling away from the fingertips. This can also occur on the palms, toes, soles, and around the nails.
Kawasaki's disease - peeling of the fingertips
illustration
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Swollen lymph node - illustration
Lymph nodes play an important part in the body's defense against infection. Swelling might occur even if the infection is trivial or not apparent. Swelling of lymph nodes generally results from localized or systemic infection, abscess formation, or malignancy.
Swollen lymph node
illustration
-
Kawasaki disease - edema of the hand - illustration
Kawasaki disease can produce various rashes and skin manifestations. This photograph shows mild swelling (edema) and redness associated with the disease. The hands and feet may be tender to touch and the skin may peel once the redness begins to fade.
Kawasaki disease - edema of the hand
illustration
-
Kawasaki's disease - peeling of the fingertips - illustration
Kawasaki's disease causes various rashes and manifestations of the mucous membranes and skin. This photograph shows the skin peeling away from the fingertips. This can also occur on the palms, toes, soles, and around the nails.
Kawasaki's disease - peeling of the fingertips
illustration
-
Swollen lymph node - illustration
Lymph nodes play an important part in the body's defense against infection. Swelling might occur even if the infection is trivial or not apparent. Swelling of lymph nodes generally results from localized or systemic infection, abscess formation, or malignancy.
Swollen lymph node
illustration
Review Date: 4/30/2023
Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, New York, NY, and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.