Asked by Chad, Medford, Oklahoma
I had Kawasaki syndrome when I was young. My question is, can you outgrow Kawasaki syndrome? I have always wondered about it, and no one has been able to tell me yes or no.
Dr. Otis Brawley
Chief Medical Officer,
American Cancer Society
Kawasaki disease is sometimes called mucocutaneous lymph node syndrome. It is a vasculitis, an inflammation of blood vessels.
It is a rare disease that affects people of Asian ancestry more than those of Caucasian or African ancestry. Eighty percent of those who get the condition develop it before age 5.
Kawasaki disease usually presents in an acute phase with flulike symptoms that last 10 to 14 days. There can be fever, lethargy, breathing difficulty, vomiting and diarrhea. Patients also usually have a red skin rash, swollen lymph nodes and inflammation of the inside surfaces of the mouth and the conjunctiva of the eyes.
These latter symptoms help distinguish it from the far more common viral illnesses of childhood. Blood vessel inflammation is of greatest concern in this disease. It commonly affects the arteries of the heart (coronary arteries).
It can lead to blockage of these arteries that supply oxygenated blood to the heart muscle. Blockage can cause myocardial infarction (heart attack) and heart rhythm disturbances. Weakening of the coronary arteries (aneurysm) can also be a problem.
Kawasaki disease occasionally causes inflammation of the heart muscle (myocarditis) or weakening of the heart muscle (cardiomyopathy) or decreases the heart's ability to pump blood (congestive heart failure).
An echocardiogram is an ultrasound imaging of the heart. It is useful for assessing the damage done by this disease. Since the mid-1980s, diagnosed Kawasaki disease in the acute phase has been treated with intravenous immune globulin and aspirin. In most cases the treatment causes early cessation of the acute phase.
Patients with prolonged or recalcitrant symptoms are often treated with steroids. Between one in 300 and one in 1,000 people who get this disease die in the acute phase. There was little study of the disease before 1980. As a result, there are not good data on the long-term prognosis, especially for people who had a mild or moderate case of disease.
Most patients appear to be well after the acute phase. Outer signs of the disease disappear. It does appear that long-term prognosis is related to the extent of heart damage in the acute phase.
Children without cardiovascular abnormalities detected in the acute phase appear to do well up to 20 years later. But these patients may be at higher than normal risk of developing atherosclerotic heart disease. Their coronary arteries may have minute damage to the walls that makes it easier for cholesterol to stick to it and form occlusions.
I must stress that experts are concerned about this risk. It may be true, but it has not been proven true. Those with inflamed coronary arteries during the acute phase will eventually have arteries that appear normal in routine medical imaging. Some studies suggest these patients may have higher than normal risk of cardiovascular disease in early adulthood and later.
Patients who form coronary artery aneurysms of any size in the acute phase are definitely at higher risk for coronary artery disease in early adulthood and later in life. Smaller aneurysms do usually regress and appear normal after the acute phase, but risk of coronary artery occlusion in later life is still increased. Larger (greater than 8 millimeters in diameter) aneurysms remain a lifelong problem affecting normal blood flow and causing blood clots. They can also cause cholesterol plaques to form that lead to blockages.
While Kawasaki disease has been seen in all populations, there seems to be a higher risk among people in eastern Asia, compared with those in Western Europe. There is some thought there may be a genetic or familial predisposition, but there is also some evidence that the condition is the manifestation of an infectious disease. Indeed, both may be true.
There is one Japanese study that suggests a sibling of a child who has had Kawasaki disease is 10 times more likely to develop the disease than a child whose siblings have not had it. There are also data showing that the child of a parent who has had it is at higher risk than children without a family history.
There are treatments that can lower the risk of cardiovascular disease in patients with a history of Kawasaki disease. A person with a history of the disease should definitely have an ongoing relationship with a physician (pediatrician, family practice physician or internal medicine specialist). That doctor may want the patient followed by a cardiologist.
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