Kawasaki disease

• Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown cause that primarily affects children younger than 5 years of age.
• Clinical features include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat.

Other names

Mucocutaneous lymph node syndrome; Kawasaki syndrome

History

The disease was first described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were reported in Hawaii in 1976.

Signs of Kawasaki disease

Incidence

• KD is a leading cause of acquired heart disease in the United States. Serious complications include coronary artery dilatations and aneurysms.
• The standard treatment, intravenous immunoglobulin and aspirin, substantially decreases the development of these coronary artery abnormalities.
• KD occurs worldwide, with the highest incidence in Japan, and it most often affects boys and younger children. KD may have a winter-spring seasonality, and community-wide outbreaks have been reported occasionally.
• CDC uses a passive KD surveillance system based on voluntary reporting of KD cases by health care providers and local and state health authorities. This system provides CDC with additional information, such as case symptoms and presence or absence of coronary artery abnormalities, that may not be available in hospital discharge data.
• In the continental United States, population-based and hospitalization studies estimate an incidence of KD ranging from about 9 to 20 per 100,000 children under 5 years of age.

Signs and symptoms and time course of Kawasaki disease

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.
• 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.
• Other theories suggest that the disease is caused by a response from the body's immune system.
• KD is not contagious; it cannot be passed from one person to another.

Inheritance

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, but it is still rare for more than one child in a family to develop the disease.

Risk factors

• Asian and Asian-American populations
• Risk factors include being a male gender, being between six months and five years of age, and having a family history of KD.
• 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, but it is still rare for more than one child in a family to develop the disease.

X-ray showing aneurysmal enlargement of the coronary arteries, which is a complication in a Kawasaki syndrome

Signs and symptoms

Clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat.

Typical and atypical presentations

For epidemiologic surveillance, CDC defines a case of KD as illness in a patient with fever of 5 or more days duration (or fever until the date of administration of intravenous immunoglobulin if it is given before the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:

• Rash
• Cervical lymphadenopathy (at least 1.5 cm in diameter)
• Bilateral conjunctival injection
• Oral mucosal changes
• Peripheral extremity changes

Angiography showing ectatic LAD, with largest aneurysm = 6.5 mm in diameter

Patients whose illness does not meet the above KD case definition but who have fever and coronary artery abnormalities are classified as having atypical or incomplete KD.

Treatment

• The standard treatment, intravenous immunoglobulin and aspirin, substantially decreases the development of these coronary artery abnormalities.
• 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.
• 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.

Complications

KD is a leading cause of acquired heart disease in the United States. Serious complications include coronary artery dilatations and aneurysms.

External links

• Kawasaki disease - Stanford Children's Health
• Kawasaki disease research program
• Kawasaki disease foundation
• Kawasaki disease information from Seattle Children's Hospital Heart Center

NIH genetic and rare disease info

• NIH info on Kawasaki disease

Kawasaki disease is a rare disease.

Kawasaki disease Resources
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