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Kawasaki Disease: Illness
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Kawasaki disease is a poorly understood illness. It appears in many respects to be an immune vasculitis, an autoimmune disorder. It is precipitated by unknown outside factors. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels and the heart. The cardiac involvement and complications are, by far, the most important aspect of the disease. Kawasaki disease can cause vasculitis (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms.
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The pathologic hallmark of Kawasaki disease is a generalized vasculitis that is most severe in the extraparenchymal sections of medium-sized musculoelastic arteries, especially the coronary arteries. The primary cause of death is myocardial infarction due to acute thrombosis of inflamed coronary arteries. Coronary artery stenosis can occur and result in death years after the acute illness. Aneurysms can occur in any major artery. Phlebitis occurs but is not of clinical significance.
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The most common and life threatening complication of Kawasaki disease is the development of coronary arterial aneurysms. Coronary arterial aneurysms usually develop within 6-8 weeks from the onset of illness. The other cardiac complications include myocarditis, pericarditis, congestive heart failure, pericardial effusion, valvular insufficiency and arrhythmias.
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Several noncardiac clinical findings may be observed in patients with Kawasaki disease (Table 5).28 For example, infants and young children with this disease are often more irritable than children with other febrile illnesses. Arthritis and arthralgia (more common in older girls) can occur in the first two to three weeks of the illness and typically involve the knees, ankles or hips. About one fourth of patients who undergo lumbar puncture in the acute phase of Kawasaki disease have findings consistent with aseptic meningitis. Other common features include diarrhea, vomiting, abdominal pain and pneumonitis.
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Kawasaki disease is a poorly understood illness. The cause has not been determined. It may be an autoimmune disorder precipitated by unknown outside factors. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart.
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The vast majority of children who develop Kawasaki disease are under age five. The average age child seen with the illness is two years old. It occurs in boys twice as often as in girls.
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