Kawasaki Disease: Latest Updates on Diagnosis, Risk Assessment, and Treatment

Advances in cardiac imaging and risk assessment are transforming the diagnosis, treatment, and long-term management of Kawasaki Disease, according to a new statement published in Circulation, the flagship journal of the American Heart Association. Kawasaki Disease, a rare but serious condition primarily affecting children under five, inflames blood vessels and remains the leading cause of acquired heart disease in children worldwide. Despite its unknown cause, the condition is suspected to have an infectious origin, although no specific agent has been identified. Health care professionals diagnose Kawasaki Disease through symptoms such as prolonged fever, rash, red eyes, and swelling in the hands and feet. Without timely treatment, a quarter of affected children may develop coronary artery complications.

The statement emphasizes the utility of coronary artery Z-scores, which compare the coronary artery size of affected children to healthy peers to assess risk. New risk scoring methods, particularly for North American children, consider factors such as age, ethnicity, and inflammation levels to identify high-risk patients and guide intensive treatment strategies. Advanced echocardiographic techniques and regular imaging schedules are highlighted as critical for detecting and monitoring coronary abnormalities.

Intravenous immunoglobulin (IVIG) remains the standard treatment, with emerging evidence supporting dose adjustments based on body mass. Low- or medium-dose aspirin may be as effective as high doses, while additional therapies like corticosteroids or infliximab may help in cases resistant to IVIG. Anticoagulants are recommended for severe coronary artery complications, with newer direct oral options showing promise.

The COVID-19 pandemic complicated diagnosis with the emergence of MIS-C, which shares symptoms with Kawasaki Disease. Advances in machine learning and symptom differentiation have helped distinguish these conditions. Finally, the statement underscores the need for lifelong care, particularly for those with coronary aneurysms, and specialized care during pregnancy for women with a history of Kawasaki Disease.

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