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Infectious Disease and Microbiology
Kawasaki syndrome (Kawasaki disease, KD)
Kawasaki syndrome is an acute, self-limited systemic vasculitis that primarily affects young children, especially those under 5 years of age. It is also known as mucocutaneous lymph node syndrome. Although the disease often resolves on its own, it is clinically significant because it can lead to serious cardiovascular complications, particularly coronary artery aneurysms.
The disease occurs worldwide but is most common in Japan, with most cases seen in children younger than 5 years. It can occur throughout the year, with peaks during winter and spring. Risk factors include recent respiratory infections and environmental exposures such as dust and mites. Genetic susceptibility also plays a role, with involvement of pathways such as caspase-3 and TGF-beta.
Kawasaki syndrome involves inflammation of medium and small blood vessels. In the early stage, there is endothelial swelling and inflammation, which later progresses to widespread systemic inflammation affecting multiple organs, especially the heart. Although the exact cause remains unknown, the condition is believed to be triggered by an infectious agent, possibly through a superantigen-mediated immune response.
Clinically, the diagnosis is based on the presence of fever lasting at least five days along with characteristic features. These include a polymorphous rash, bilateral non-purulent conjunctivitis, oral changes such as cracked lips and strawberry tongue, swelling and redness of the hands and feet followed by peeling, and cervical lymphadenopathy. Incomplete forms of the disease may occur and should still be treated if suspected. Other symptoms may include irritability, joint pain, myocarditis, pericarditis, aseptic meningitis, and gastrointestinal symptoms.
Laboratory findings commonly show anemia, leukocytosis, and thrombocytosis, particularly in later stages. Inflammatory markers such as ESR and CRP are elevated, and hypoalbuminemia and elevated liver enzymes may also be present. Echocardiography is essential to evaluate for coronary artery involvement, while ECG may reveal arrhythmias or other cardiac changes.
Management should be initiated promptly to reduce the risk of complications. The mainstay of treatment is intravenous immunoglobulin (IVIG), given as a single high-dose infusion, with a repeat dose if fever persists. Aspirin is also used, initially at high doses for its anti-inflammatory effect and later at lower doses for its antiplatelet properties. In resistant cases, corticosteroids or agents such as infliximab may be used. Patients with coronary complications may require anticoagulation and specialist cardiac care.
The prognosis is generally good when treated early. Without treatment, a significant proportion of patients develop coronary artery aneurysms, but this risk is markedly reduced with timely therapy. Long-term follow-up with echocardiography is important, especially in those with cardiac involvement.
Complications are mainly cardiovascular and include coronary artery aneurysms, myocardial infarction, myocarditis, and pericarditis, with myocardial infarction being the most common cause of death. Non-cardiac complications may include hearing loss, arthritis, pneumonitis, and macrophage activation syndrome.
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