Kawasaki Disease
Overview
Plain-Language Overview
Kawasaki Disease is an illness that mainly affects children and involves inflammation of the blood vessels throughout the body. It primarily targets the medium-sized arteries, especially the coronary arteries that supply blood to the heart. This inflammation can cause symptoms like a high fever, rash, swollen hands and feet, red eyes, and swollen lymph nodes. The disease can lead to serious heart problems if not treated promptly, including the formation of coronary artery aneurysms. Early recognition and treatment are important to reduce the risk of long-term heart damage. The exact cause is unknown, but it is thought to involve an abnormal immune response to an infection or environmental trigger. Overall, it is a condition that affects the cardiovascular system and can have lasting effects on heart health.
Clinical Definition
Kawasaki Disease is an acute, self-limited vasculitis of unknown etiology that predominantly affects medium-sized arteries, with a predilection for the coronary arteries. It is characterized by systemic inflammation leading to endothelial damage and potential development of coronary artery aneurysms, which are the major cause of morbidity and mortality. The disease primarily occurs in children under 5 years old and is thought to result from an abnormal immune activation possibly triggered by infectious agents in genetically susceptible individuals. Clinical features include prolonged fever, conjunctival injection, mucocutaneous changes, extremity changes, rash, and cervical lymphadenopathy. The pathophysiology involves infiltration of vascular walls by inflammatory cells, leading to destruction of the internal elastic lamina and vessel wall weakening. Early diagnosis and treatment with intravenous immunoglobulin (IVIG) and aspirin reduce the risk of coronary complications. The disease is a leading cause of acquired heart disease in children in developed countries.