Kawasaki Disease (cont.)
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
have been reported in up to 25% of those 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
an ultrasound 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) to reduce inflammation and mildly thin the blood to prevent blood clot formation. Also used in treatment is gamma globulin 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. Sometimes cortisone medications are given. Persisting joint pains are treated with anti-inflammatory 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. Medications that block the effects of TNF (tumor necrosis factor), one of the messenger molecules in the inflammatory response, are being studied for use in these situations. Examples of TNF-blocking drugs are infliximab (Remicade) and etanercept (Enbrel). Further research is needed to design treatment programs for those who are failing conventional treatments.
Next: What is the outlook (prognosis) for children with Kawasaki disease? »
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