2 learning resources available for this topic
Kawasaki disease is an acute systemic vasculitis primarily affecting children under 5 years old, characterized by prolonged fever and inflammation of medium-sized arteries. It is the leading cause of acquired heart disease in children in developed countries, with coronary artery aneurysms being the most serious complication. Early recognition and treatment with IVIG and aspirin significantly reduce the risk of cardiac sequelae.
The exact etiology remains unknown, but an abnormal immune response to an infectious trigger in genetically susceptible individuals is suspected. The disease involves widespread inflammation of medium-sized arteries, particularly affecting the coronary arteries, leading to endothelial dysfunction, smooth muscle cell proliferation, and potential aneurysm formation. The inflammatory process involves activation of T cells, macrophages, and cytokine release, resulting in the characteristic clinical manifestations.
Diagnosis relies on clinical criteria including prolonged fever plus four of five principal features: conjunctival injection, oral mucosal changes, cervical lymphadenopathy, extremity changes, and polymorphous rash. Laboratory findings show elevated inflammatory markers, thrombocytosis, and hypoalbuminemia, while echocardiography is essential for detecting coronary artery involvement. Prompt treatment with high-dose IVIG within 10 days of fever onset, combined with aspirin therapy, is crucial for preventing coronary complications and reducing the inflammatory response.