Kawasaki Disease (KD) Kawasaki Disease (KD), also known as mucocutaneous lymph node syndrome, is an acute febrile illness primarily affecting young children. It is characterized by inflammation of the medium-sized arteries throughout the body, particularly the coronary arteries, and can lead to potentially serious cardiovascular complications if untreated. Etiology and Pathophysiology The exact cause of Kawasaki Disease remains unknown, although it is widely believed to involve an abnormal immune response triggered by an infectious agent in genetically predisposed individuals. It is not contagious. The inflammation primarily affects the arterial walls, leading to vasculitis and subsequent damage, particularly in the coronary arteries. This can result in coronary artery aneurysms or other cardiovascular sequelae. Clinical Features KD typically presents in children under the age of five. The hallmark clinical features include:    Prolonged fever: Persistent fever for more than five days is often the first symptom. Conjunctivitis: Bilateral non-exudative conjunctivitis (inflammation of the eyes) is common. Mucous membrane changes: Changes in the oral mucosa such as red, cracked lips, "strawberry" tongue (prominent papillae), and oral erythema.   Rash: Characteristic polymorphous rash, often erythematous and morbilliform (measles-like). Cervical lymphadenopathy: Enlargement of cervical lymph nodes, usually more than 1.5 cm in diameter. Diagnostic Evaluation Diagnosing Kawasaki Disease relies on clinical criteria due to the absence of specific laboratory or imaging tests. Diagnostic criteria include fever lasting five or more days plus four of the following five features:      Bilateral conjunctival injection Changes in the lips and oral cavity (e.g., erythema, strawberry tongue) Changes in the extremities (e.g., erythema of palms/soles, edema) Polymorphous rash Cervical lymphadenopathy Additional tests may include echocardiography to assess coronary artery involvement and laboratory tests such as complete blood count, inflammatory markers (e.g., ESR, CRP), and sometimes a panel to rule out other conditions. Management and Treatment Early treatment of Kawasaki Disease is crucial to prevent coronary artery complications. Treatment typically involves:    Intravenous immunoglobulin (IVIG): Administered within 10 days of fever onset to reduce inflammation and decrease the risk of coronary artery abnormalities. Aspirin: High doses initially to reduce fever and inflammation, followed by low doses to prevent thrombosis and coronary complications. Monitoring: Regular clinical assessment and echocardiography to monitor coronary artery status. Prognosis With timely treatment, the majority of children with Kawasaki Disease recover fully without significant complications. However, untreated or inadequately treated cases can lead to coronary artery aneurysms, myocardial infarction, or other cardiovascular sequelae, highlighting the importance of early recognition and management. Long-term Considerations Long-term follow-up is essential for children with Kawasaki Disease, particularly those with coronary artery involvement. Some may require ongoing cardiology evaluation and monitoring for potential late complications such as coronary artery stenosis or thrombosis. Conclusion Kawasaki Disease is a unique vasculitis primarily affecting young children, characterized by systemic inflammation and potential coronary artery involvement. Early recognition of its clinical features and prompt initiation of IVIG and aspirin therapy are critical to reduce the risk of cardiovascular complications. Ongoing research continues to improve our understanding of its pathogenesis and optimize treatment strategies, aiming to improve outcomes and quality of life for affected children worldwide.