Kawasaki Disease: Beyond IVIG and Aspirin

Kawasaki disease (KD) is a vasculitis syndrome of unknown etiology, affecting medium-sized vessels and occurring primarily in young children. It is a self-limited illness classically presenting with fever, conjunctivitis, mucocutaneous lesions, and cervical adenopathy. However, vasculitis of coronary arteries with aneurysm formation may occur, leading to morbidity and rarely mortality. Prompt diagnosis of KD is essential as early treatment with intravenous immune globulin (IVIG) decreases the occurrence and severity of coronary vasculitis. This article reviews the clinical characteristics of KD, laboratory assessment, echocardiogram findings, and recommended initial medical therapy. Complications of KD are discussed, including KD refractory to IVIG, the presence of shock or macrophage activation syndrome (MAS), and short/long-term cardiac sequelae. The medical management of these complications is reviewed. Although improved treatment of KD has resulted in a decrease of coronary artery vasculitis over the past 3 decades, KD remains the most common cause of acquired heart disease in children. [Pediatr Ann. 2019;48(10):e400-e405.].