Kawasaki disease

Información en español Title

Other Names:

Mucocutaneous lymph node syndrome; Kawasaki syndrome

Categories:

Blood Diseases; Heart Diseases; Immune System Diseases; Blood Diseases; Heart Diseases; Immune System Diseases; Kidney and Urinary Diseases See More

Summary Summary

Kawasaki disease is a disease that involves inflammation of the blood vessels. It is typically diagnosed in young children, but older children and adults can also develop this condition. Kawasaki disease begins with a fever that lasts at least five days. Other classic symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes.^[1] Sometimes the disease affects the coronary arteries which carry oxygen-rich blood to the heart, which can lead to serious heart problems. Kawasaki disease occurs most often in people of Asian and Pacific Island descent.^[2] The cause of Kawasaki disease is unknown.^[1]^[2] An infection along with genetic factors may be involved.^[2]^[3] Treatment includes intravenous gamma globulin and high doses of aspirin in a hospital setting.^[1]^[2] Prognosis is generally very good, but in cases of heart complications it depends on the severity of the coronary disease.^[3]

Last updated: 3/9/2017

Symptoms Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Cheilitis	Inflammation of the lips	0100825
Conjunctivitis	Pink eye	0000509
Erythema		0010783
Lymphadenopathy	Swollen lymph nodes	0002716
Proteinuria	High urine protein levels Protein in urine [ more ]	0000093
Recurrent pharyngitis	Recurrent sore throat	0100776
Skin rash		0000988
Vasculitis	Inflammation of blood vessel	0002633
30%-79% of people have these symptoms
Abdominal pain	Pain in stomach Stomach pain [ more ]	0002027
Abnormal heart valve morphology		0001654
Abnormality of nail color	Abnormality of nail colour	0100643
Arthritis	Joint inflammation	0001369
Diarrhea	Watery stool	0002014
Edema	Fluid retention Water retention [ more ]	0000969
Fatigue	Tired Tiredness [ more ]	0012378
Fever		0001945
Glossitis	Inflammation of the tongue Smooth swollen tongue [ more ]	0000206
Leukocytosis	Elevated white blood count High white blood count Increased blood leukocyte number [ more ]	0001974
Pericarditis	Swelling or irritation of membrane around heart	0001701
5%-29% of people have these symptoms
Arrhythmia	Abnormal heart rate Heart rhythm disorders Irregular heart beat Irregular heartbeat [ more ]	0011675
Arthralgia	Joint pain	0002829
Ascending tubular aorta aneurysm	Bulging of wall of large artery located above heart	0004970
Cholecystitis	Gallbladder inflammation	0001082
Congestive heart failure	Cardiac failure Cardiac failures Heart failure [ more ]	0001635
Cranial nerve paralysis		0006824
Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis		0011658
Hepatitis	Liver inflammation	0012115
Interstitial pulmonary abnormality	Abnormality in area between air sacs in lung	0006530
Irritability	Irritable	0000737
Jaundice	Yellow skin Yellowing of the skin [ more ]	0000952
Meningitis		0001287
Migraine	Intermittent migraine headaches Migraine headache Migraine headaches [ more ]	0002076
Myocarditis	Inflammation of heart muscle	0012819
Nausea and vomiting		0002017
Ptosis	Drooping upper eyelid	0000508
Sterile pyuria		0100586

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 11/1/2019

Do you have updated information on this disease? We want to hear from you.

Cause Cause

The cause of Kawasaki disease (KD) is unknown. The disease results when cells move into the tissues and buildup there, leading to vascular damage, but what causes the cell buildup in the first place is unknown. 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 identified, and the role of genetics is not well understood.^[2]^[4]

Genetic factors appear to be important to this disorder, as suggested by the increased frequency of the disease in Asian and Asian-American populations and among family members of an affected child. A number of gene variants (polymorphisms) are associated with an increased risk of developing (susceptibility) KD, and some of these variants are also associated with coronary artery lesions and aneurysm formation.[12298]

Other theories suggest that the disease is caused by a response from the body's immune system.^[5]

KD is not contagious; it cannot be passed from one person to another.^[2] Other risk factors include being a male gender, being between six months and five years of age, and having a family history of KD.^[5]

Last updated: 3/9/2017

Inheritance Inheritance

A susceptibility to Kawasaki disease (KD) appears to be passed through generations in families, but the inheritance pattern is unknown. Children of parents who have had KD have twice the risk of developing the disease compared to the general population. Children whose siblings have had KD are ten times more likely to develop KD than the general population^[4], but it is still rare for more than one child in a family to develop the disease.^[6]

Last updated: 3/9/2017

Treatment Treatment

Intravenous gamma globulin is the standard treatment for Kawasaki disease and is administered in high doses. Children with Kawasaki disease usually improve significantly within 24 hours of treatment with IV gamma globulin (IVIG). Aspirin is often given in combination with the IV gamma globulin as part of the treatment plan in the beginning of the disease.^[1] Additional treatments include glucocorticoids, which may reduce the level of inflammatory markers and fever more rapidly, and decrease the rate of initial treatment failure in certain high-risk patients.^[3] You can read about complications, treatment, and prevention of Kawasaki disease on the American Heart Association website.

Last updated: 3/9/2017

Management Guidelines

Freeman AF, Shulman ST. Kawasaki Disease: Summary of the American Heart Association Guidelines. Am Fam Physician 2006;74:1141-50.

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.
If your physician would like to review your case with a vasculitis specialist, the Vasculitis Foundation lists vasculitis medical consultants that may be able to speak with your healthcare provider. Click on the link above to learn more.
The Vasculitis Foundation has a "Find a Physician" map on its website.

Related Diseases Related Diseases

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis includes autoimmune and autoinflammatory diseases (i.e. systemic lupus erythematosus, polyarteritis nodosa, and sarcoidosis), bacterial infections (i.e. bacterial toxic shock syndrome, leptospirosis) (see these terms), viral infections (i.e. measles, enterovirus, Epstein-Barr virus), and toxin or drug reactions. Visit the Orphanet disease page for more information.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Kawasaki disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Heart Association
7272 Greenville Avenue
Dallas, TX 75231-4596
Toll-free: 800-242-8721
Telephone: 214-570-5978
E-mail: https://www.heart.org/en/forms/general-questions-and-latest-research-information
Website: https://www.heart.org
Kawasaki Disease Foundation
9 Cape Ann Circle
Ipswich, MA 01938
Telephone: 978-356-2070
Fax: 978-356-2079
E-mail: info@kdfoundation.org
Website: http://www.kdfoundation.org/
Kawasaki Support Group
Attn: Mrs. Sue Davidson
13 Norwood Grove
Potters Green
Coventry
CV2 2FR
United Kingdom
Telephone: 024 7661 2178
Fax: 024 7661 2178
E-mail: helpline@kssg.org.uk
Website: http://www.kssg.org.uk
Vasculitis Foundation
P.O. Box 28660
Kansas City, MO 64188
Toll-free: 1-800-277-9474
Telephone: +1-816-436-8211
Fax: +1-816-656-3838
E-mail: https://www.vasculitisfoundation.org/contact-us-2/
Website: https://www.vasculitisfoundation.org/

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

You can obtain information on this topic from the Centers for Disease Control and Prevention (CDC). The CDC is recognized as the lead federal agency for developing and applying disease prevention and control, environmental health, and health promotion and education activities designed to improve the health of the people of the United States.
The Centers for Disease Control and Prevention (CDC) provide more information on reporting cases of Kawasaki syndrome. Click on the CDC link to view this information.

National Center for Zoonotic, Vector-Borne, Enteric Diseases (NCZVED)
1600 Clifton Road, NE
MS D-76
Atlanta, GA 30329-4018
Genetics Home Reference (GHR) contains information on Kawasaki disease. This website is maintained by the National Library of Medicine.
The Vasculitis Foundation offers information on Kawasaki disease to assist patients, family members, and medical professionals in learning more about the condition.
KidsHealth created by The Nemours Foundation's Center for Children's Health Media provides information in both English and Spanish about this condition. Click on the link to access the information page on Kawasaki disease.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Heart, Lung, and Blood Institute (NHLBI) has information on this topic. NHLBI is part of the National Institutes of Health and supports research, training, and education for the prevention and treatment of heart, lung, and blood diseases.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Kawasaki disease. Click on the link to view a sample search on this topic.

Videos/Presentations

Dr. Adriana Tremoulet spoke on Kawasaki Disease (KD), in her lecture "Kawasaki Disease: New Horizons" at the NIH Clinical Center in November 2016. Dr. Tremoulet is an associate professor and associate director at the Kawasaki Disease Research Center at the University of California San Diego Rady Children's Hospital. While the lecture is aimed at medical professionals, it may be of interest to anyone who seeks detailed information on KD.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

My son had Kawasaki disease last year. I am expecting another baby. Is this baby at-risk to develop Kawasaki disease? See answer
Eighteen months ago my daughter was diagnosed with Kawasaki syndrome. Although she has recovered medically, she appears to have some behavioral and developmental problems. Can Kawasaki syndrome cause behavioral problems or slow a child's development? See answer
I know someone who had Kawasaki disease when he was a child. Is there any information about the long-term consequences of this condition in surviving adults? See answer
My child was diagnosed with Kawasaki disease last month, and I found out that another child in the same daycare center was diagnosed with Kawasaki disease one year ago. I know this condition is rare and I thought it was unusual for two kids from the same day care center to have Kawasaki disease. Is there a method for tracking Kawasaki disease? See answer