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Kawasaki disease

Kawasaki disease is a rare condition that involves inflammation of the blood vessels. It occurs in children.

Alternative Names

Mucocutaneous lymph node syndrome; Infantile polyarteritis

Causes, incidence, and risk factors

Kawasaki disease occurs most often in Japan, where it was first discovered. The disease occurs 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 mucus membranes, lymph nodes, walls of the blood vessels, and the heart.

Symptoms

Kawasaki disease often begins with a fever  of 102 °F or higher that does not go away. The fever is often as high as 104 °F. 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.

Other symptoms often include:

  • Very bloodshot or red eyes (without pus or drainage)
  • Bright red, chapped, or cracked lips
  • Red mucous membranes in the mouth
  • Strawberry tongue, white coating on the tongue, or visible red bumps on the back of the tongue
  • Red palms of the hands and the soles of the feet
  • Swollen hands and feet
  • Skin rashes on the middle of the body, NOT blister-like
  • Peeling skin in the genital area, hands, and feet (mostly around the nails, palms, and soles)
  • Swollen lymph nodes in the neck (often only one lymph node is swollen)
  • Joint pain and swelling, often on both sides of the body

Additional symptoms may include:

  • Irritability
  • Diarrhea, vomiting, and abdominal pain
  • Cough and runny nose

Signs and tests

Tests alone cannot diagnose Kawasaki disease. Most of the time, the doctor will diagnose the disease when the patient 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 seen by a health care provider to be checked for Kawasaki disease. Children with the disease need early treatment for a good outcome.

The following tests may be done:

Tests such as ECG and echocardiography are done to look for signs of myocarditis , pericarditis , arthritis , aseptic meningitis, and inflammation of the coronary arteries.

Treatment

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. 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. Some research suggests that adding steroids or TNF inhibitors such as Enbrel to the treatment routine may help. However, there still needs to be better tests to tell which children will benefit from added forms of treatment.

Expectations (prognosis)

Most children can recover fully when the disease is caught and treated early. About 1 in 100 patients die from heart problems caused by the disease. People who have had Kawasaki disease should have an echocardiogram every 1 - 2 years to screen for heart problems.

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.

Calling your health care provider

Call your health care provider if symptoms of Kawasaki disease develop. An ongoing high fever that does not come down with acetaminophen or ibuprofen and lasts more than 24 hours should be checked by a health care provider.

Prevention

There are no known ways to prevent this disorder.

References

Villa-Forte A, Mandell BF. Rheumatic diseases of the cardiovascular system. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 89.

Dominguez SR, Anderson MS. Advances in the treatment of Kawasaki disease. Current Opinion in Pediatrics. 2013;25(1):103-109.

Updated: 4/20/2013

Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.


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