Kawasaki Disease
What is Kawasaki disease?
Kawasaki disease is an uncommon illness in children which is characterized by fever of at least 5 days duration together with four of the following five findings: - Inflammation with reddening of the whites of the eyes (conjunctivitis).
- Redness or swelling of the hands or feet; or generalized skin peeling.
- Rash.
- Lymph gland swelling in the neck.
- Cracking inflamed lips or throat; or red "strawberry" tongue.
What are the usual symptoms of Kawasaki disease?
The usual symptoms of Kawasaki disease include fever, reddening of the eyes (conjunctivitis), cracked and inflamed lips and mucous membranes of the mouth, ulcerative gum disease (gingivitis), swollen glands in the neck (cervical lymphadenopathy) and a rash that is raised and bright red. The rash appears in a glove-and-sock fashion over the skin of the hands and feet which becomes hard, swollen (edematous), and then peels off.
What are the less common findings?
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 other heart complications listed below under serious complications. The outlook for an individual affected is primarily determined by the seriousness of any heart complications.
What is the difference between Kawasaki disease and Kawasaki syndrome?
None. Kawasaki disease is also referred to Kawasaki syndrome. It was first described in the late 1960's in Japan by the pediatrician Tomisaku Kawasaki.
What is mucocutaneous lymph node syndrome?
Kawasaki disease or syndrome is also called the mucocutaneous lymph node syndrome, a name that is 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 glands.
What causes Kawasaki disease?
The cause is not known. Microorganisms and toxins have been suspected, but none has been identified to date. Genetic factors and the immune system may play a role.
Who develops Kawasaki disease?
Kawasaki disease typically affects children under 4 years of age. Only rarely does it affect children over 8 years of age.
How can Kawasaki disease cause serious complications?
Children with Kawasaki 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). Coronary aneurysms are reported in up to 25 percent of persons with Kawasaki 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 sound wave test of the heart (echocardiogram). Other arteries that can become inflamed include the arteries of the lungs, neck, and abdomen. These effects can lead to breathing problems, headaches, and pain in the belly, respectively.
How is Kawasaki disease treated?
Kawasaki disease is treated with high doses of aspirin (salicylic acid). Also used in treatment is gamma globulin administered through the vein (intravenously), together with fluids. Sometimes cortisone medications are given. Persisting joint pains are treated with antiinflammatory drugs, such as ibuprofen or naproxen.
Plasma Exchange (Plasmapheresis) has been reported as effective in patients who were not responding to aspirin and gamma globulin. 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 aspirin and gamma globulin treatments can be deadly. This research offers hope to those who are failing treatment.
What is the outlook (prognosis) for children with Kawasaki disease?
Kawasaki disease generally resolves on its own after 4 to 8 weeks.
However, the outcome is not so favorable in every case. Rarely, Kawasaki disease can cause death from blood clots forming in abnormal areas of widening of the heart (coronary) arteries. Those children with larger aneurysms have a worse prognosis because of this risk.
Kawasaki Disease At A Glance - Kawasaki disease is a syndrome of unknown cause that mainly strikes young children.
- Signs of the disease include fever and red eyes, hands, feet, mouth, and tongue.
- The disease can be treated with high doses of aspirin (salicylic acid) and gamma globulin.
- Kawasaki disease usually resolves on its own within a month or two.
- Some children with Kawasaki disease suffer damage to the coronary arteries.
Author: William C. Shiel Jr., MD, FACP, FACR
For more information, please visit the Kawasaki Families' Network .
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