Kawasaki's Disease

  • Medical Author:
    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

  • Medical Editor: Catherine Burt Driver, MD
    Catherine Burt Driver, MD

    Catherine Burt Driver, MD

    Catherine Burt Driver, MD, is board certified in internal medicine and rheumatology by the American Board of Internal Medicine. Dr. Driver is a member of the American College of Rheumatology. She currently is in active practice in the field of rheumatology in Mission Viejo, Calif., where she is a partner in Mission Internal Medical Group.

Symptoms of Rheumatoid Arthritis

Kawasaki's disease facts

  • Kawasaki's disease is a syndrome of unknown cause that mainly strikes young children.
  • Kawasaki's disease symptoms and signs include
    • fever,
    • redness of the eyes, hands, feet, mouth, and tongue.
  • The disease can be treated with high doses of aspirin (salicylic acid) and gammaglobulin.
  • Kawasaki's disease symptoms usually resolve within a month or two, but the disease should be considered a "lifelong disease" because monitoring for late-onset heart artery changes is necessary.
  • Some children with Kawasaki's disease suffer damage to the coronary arteries.

What is Kawasaki's disease, and how do doctors diagnose it?

Kawasaki's disease is an uncommon illness in children that is characterized by high fever of at least five days' duration together with at least four of the following five findings:

  1. Inflammation with reddening of the whites of the eyes (conjunctivitis) without pus
  2. Redness or swelling of the feet or hands, or generalized skin peeling
  3. Rash
  4. Lymph node swelling in the neck
  5. Cracking, inflamed lips or throat, or red "strawberry" tongue

The above criteria are used to make a diagnosis of Kawasaki's disease. The terminology "incomplete Kawasaki's disease" is sometimes used for patients with only some features of classical Kawasaki's disease. Most patients are under 5 years of age with a peak incidence at 18 months of age.

What is mucocutaneous lymph node syndrome?

Mucocutaneous lymph node syndrome is the original name for Kawasaki's disease. The original name was quite descriptive because the disease is characterized by the typical changes in the mucous membranes that line the lips and mouth and by the enlarged and tender lymph nodes. Kawasaki's disease is also sometimes referred to as Kawasaki disease.

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Kawasaki's Disease Symptoms and Signs

High fever is a prominent symptom in children with Kawasaki disease. The fever is persistent and lasts for at least five days. Nonspecific symptoms can precede or accompany the fever, including

  • fussiness,
  • nausea,
  • vomiting,
  • runny nose,
  • cough,
  • diarrhea,
  • joint pain,
  • abdominal pain,
  • and decreased intake of food and liquids.

What are causes and risk factors of Kawasaki's disease?

The cause is not known. Microorganisms and toxins have been suspected, but none has been identified to date. It is common for Kawasaki’s disease to occur after a preceding infection, such as tonsillitis, ear infection, pneumonia, urine infection, or gastrointestinal infection. Genetic factors and the immune system seem to play roles in the disease, which is characterized by immune activation. The disease is more common in those of Japanese or Korean descent.

Who develops Kawasaki's disease?

Kawasaki disease typically affects children under 5 years of age. Only rarely does it affect children over 8 years of age, in which case it is sometimes referred to as atypical Kawasaki's disease.

What are the usual symptoms and signs of Kawasaki's disease?

The usual symptoms and signs of Kawasaki's disease include

The rash appears in a glove-and-sock fashion over the skin of the hands and feet. The rash becomes hard, swollen (edematous), and then peels off.

What are unusual signs and symptoms of Kawasaki's disease?

Most of the common symptoms described above will resolve without complications, even if untreated. Less common findings include inflammation of the lining of the heart (pericarditis), joints (arthritis), or covering of the brain and spinal cord (meningitis), and the other heart complications listed below in the section on serious complications. The outlook (prognosis) for an individual affected is primarily determined by the seriousness of any heart complications.

What is the difference between Kawasaki's disease and Kawasaki's syndrome?

They are the same. Kawasaki's disease is also referred to as Kawasaki's syndrome. It was first described in the late 1960s in Japan by the renowned pediatrician Tomisaku Kawasaki.

Is Kawasaki's disease contagious?

No. Kawasaki's disease is not believed to be a contagious illness.

How can Kawasaki's disease cause serious complications?

Children with Kawasaki's disease can develop inflammation of the arteries of various parts of the body. This inflammation of the arteries is called vasculitis. Arteries that can be affected include the arteries that supply blood to the heart muscle (the coronary arteries). Vasculitis can cause weakening of the blood vessels and lead to areas of vessel widening (aneurysms). The weakening occurs because of destruction of the elastic tissue in the walls of the blood vessels. Coronary aneurysms occur because of such injury to the blood vessels in Kawasaki's disease.

Because of the potential for heart injury and coronary aneurysms, special tests are performed to examine the heart. Children are typically evaluated with an electrocardiogram (EKG) and an ultrasound test of the heart (echocardiogram). Monitoring for this problem is crucial throughout life as it can develop as a late aftereffect of Kawasaki's disease. Other arteries that can become inflamed include the arteries of the lungs, neck, and abdomen. These effects can lead to breathing problems, headaches, and abdominal pain, respectively. Blood tests to measure the degree of inflammation (such as C-reactive protein, or CRP, and erythrocyte sedimentation rate, or ESR) are often used to monitor the activity of the disease.

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What is the treatment for Kawasaki's disease?

Children affected by Kawasaki's disease are hospitalized. Kawasaki's disease is treated with high doses of aspirin (salicylic acid) to reduce inflammation and to mildly thin the blood to prevent blood clot formation. Also used in treatment is gammaglobulin administered through the vein (intravenous immunoglobulin or IVIG), together with fluids. This treatment has been shown to decrease the chance of developing aneurysms in the coronary arteries, especially when used early in the illness. Sometimes cortisone medications are given. Persisting joint pains are treated with anti-inflammatory drugs, such as ibuprofen (Advil) or naproxen (Aleve).

Plasma exchange (plasmapheresis) has been reported as effective in patients who were not responding to aspirin and gammaglobulin. Plasmapheresis is a procedure whereby the patient's plasma is removed from the blood and replaced with protein-containing fluids. By taking out portions of the patient's plasma, the procedure also removes antibodies and proteins that are felt to be part of the immune reaction that is causing the inflammation of the disease.

Kawasaki's disease that is not responding to the traditional cornerstones of treatment, aspirin and gammaglobulin infusions, can be deadly. Treatments being investigated include doxycycline, statin drugs (Lipitor, Mevacor, others), anakinra (Kineret), and cyclosporine (Neoral).

What is the prognosis for children with Kawasaki's disease?

Kawasaki's disease generally resolves on its own after four to eight weeks, and with early treatment, full recovery is usual.

However, the outcome is not so favorable in every child. Rarely, Kawasaki's disease can cause death from blood clots forming in abnormal areas of widening (aneurysms) of the heart arteries (coronary arteries). Aneurysms of the arteries to the heart (coronary arteries) can occur early or late, even when the children are adults. Those children with larger aneurysms have a worse prognosis because of this risk. Those whose echocardiograms do not demonstrate widening of the coronary arteries throughout life do best. Those with any signs of aneurysm changes require very close monitoring. Children less than 6 months of age and those with high levels of inflammation on blood testing are at highest risk.

The earlier the diagnosis is made and treatment is begun the better the outcome.

Researchers are searching for methods of detecting which children are at risk for the development of aneurysms of the coronary arteries. Further research is under way to investigate a variety of criteria for atypical variants of Kawasaki's disease that do not have classical presentations.

Is it possible to prevent Kawasaki's disease?

Because the cause of Kawasaki's disease has not been determined, there are no measures known that can prevent the disease. Early evaluation and treatment can decrease the chance for long-term problems.

REFERENCES:

American College of Rheumatology National Meeting, Boston, 2007.

Klippel, J.H., et al. Primer on the Rheumatic Diseases. New York: Springer, 2008.

Ruddy, Shaun, et al., eds. Kelley's Textbook of Rheumatology. Philadelphia: W.B. Saunders Co., 2000.

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Reviewed on 11/19/2015
References
REFERENCES:

American College of Rheumatology National Meeting, Boston, 2007.

Klippel, J.H., et al. Primer on the Rheumatic Diseases. New York: Springer, 2008.

Ruddy, Shaun, et al., eds. Kelley's Textbook of Rheumatology. Philadelphia: W.B. Saunders Co., 2000.

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