Overview of Kawasaki Disease

A Type of Arthritis and Form of Systemic Vasculitis

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What Is Kawasaki Disease?

Kawasaki disease is one of more than 100 types of arthritis, according to the Arthritis Foundation. Kawasaki disease is a form of systemic vasculitis that develops in young children -- 80% of Kawasaki patients are under 5 years old.

Tomasaku Kawasaki first identified the disease in 1967 in Japan. Before being designated as Kawasaki disease, the condition was known as mucocutaneous lymph node syndrome.

It is also referred to as Kawasaki's disease, Kawasaki's syndrome and infantile polyarteritis.

Kawasaki Disease Symptoms

Kawasaki disease may be associated with the development of coronary arteritis (inflammation of the coronary arteries) and the formation of aneurysms. In the United States, Kawasaki disease is considered the leading cause of acquired heart disease in children.

Kawasaki disease develops quickly and runs an aggressive course over a period of weeks. Typically, the condition then resolves. Even though it resolves, though, there can be cardiac complications years later.

Other symptoms associated with Kawasaki disease include:

  • spiking fevers that last 5 or more days (required to make ​a diagnosis of Kawasaki Disease)
  • inflamed conjunctiva (eyes) without pus
  • red chapped lips
  • red mucous membranes in the mouth
  • red tongue that has the appearance of a strawberry
  • rashes on the trunk of the body
  • red palms of hands and red soles of feet
  • edema of hands and feet
  • peeling skin on hands, feet and genitals
  • swollen lymph node usually near the neck
  • joint pain and swelling often on both sides of body

Other unusual findings that can occur with Kawasaki disease include aseptic meningitis, sterile pyuria and urethritis, diarrhea, abdominal pain, pericardial effusion, obstructive jaundice and hydrops of the gallbladder.

Cause of Kawasaki Disease

Some suspect there is an infectious cause of Kawasaki disease, but none has been proven. There are several immunologic irregularities linked with Kawasaki disease, and some believe it is an autoimmune condition.

Prevalence of Kawasaki Disease

In the United States, Kawasaki disease affects 4,000 children each year. It is more prevalent in Japan -- where it was discovered -- with 5,000 to 6,000 cases each year.

Treatment for Kawasaki Disease

Once the disease is diagnosed, quick treatment is recommended so that damage to coronary arteries and heart can be prevented. Intravenous immune globulin (IVIG) given in high doses is considered standard treatment for Kawasaki disease. High-dose aspirin is typically a part of the treatment plan as well. Glucocorticoids are usually not used to treat Kawasaki disease.

With early treatment for Kawasaki disease, full recovery is possible for most, but 2% of Kawasaki disease patients die from complications of coronary artery inflammation.

It is recommended that patients who have had Kawasaki disease have an EKG (echocardiogram) every one or two years to check for residual heart problems.

Sources:

Vasculitides. Kawasaki's Disease (Chapter 21). Primer on the Rheumatic Diseases. 13 Edition. Published by Arthritis Foundation. Klippel J. et al.

It's Not Just Growing Pains. Kawasaki Disease Chapter 17. Thomas J.A. Lehman, MD. Oxford University Press. 2004.

Kawasaki Disease. MedlinePlus. NLM and NIH. 11/29/2006.
http://www.nlm.nih.gov/medlineplus/ency/article/000989.htm

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