Purpose of Review Provide the most recent updates on the epidemiology, pathogenesis, and treatment advances in Kawasaki disease. Recent Findings Treatment advances in complex, IVIG-refractory cases of Kawasaki disease. Multisystem inflammatory syndrome, a newly reported inflammatory condition with Kawasaki-like features and an association with the 2019 Coronavirus (COVID-19). Summary Kawasaki disease (KD) is a rare systemic inflammatory disease that predominately affects children less than 5 years of age. Pathogenesis of KD remains unknown; the leading theory is that an unknown stimulus triggers an immune-mediated inflammatory cascade in a genetically susceptible child. Classic KD is a clinical diagnosis based on set criteria and excluding other similar clinical entities. Patients who do not fulfill complete diagnostic criteria for KD are often referred to as atypical (or incomplete) KD. The most feared complication of KD is coronary artery abnormality development, and patients with atypical KD are also at risk. Administration of intravenous immunoglobulin (IVIG) and aspirin has greatly reduced the incidence of coronary lesions in affected children. Several other immune-modulating therapies have recently been utilized in complex or refractory cases. Keywords Kawasaki disease. Kawasaki review. Kawasaki treatment. Kawasaki workup. Kawasaki differential. Kawasaki diagnosis. Multi system inflammatory syndrome. Kawasaki-like disease Pathogenesis Genetics Although poorly understood, the predilection for children of East Asian and Pacific Islander descent, even with transmigration, This article is part of the Topical Collection on Vasculitis * Eileen Rife

Kawasaki disease (KD) was first reported in Japan more than 30 years ago, but the original diagnostic clinical criteria defined by Dr. Kawasaki are still authentic. The cause of KD remains unknown. Several epidemiologic and clinical observations suggest that it is caused by one or multiple infectious agents, each of which can result in the clinical manifestation of the disease. Advances have been made in the management of the disease with the introduction of aspirin and intravenous immunoglobulin (IVIG) that have had a significant impact on lowering the rate of coronary artery aneurysms and death from the disease. Questions remain regarding the management of those patients whose disease does not respond to IVIG. Some patients with severe KD who are resistant to IVIG may benefit from IV pulse steroids therapy. It remains to be seen whether steroid or other anti-inflammatory agents play a role in the management of patients with KD. Further research studies are needed to identify the possible cause, define the treatment, and predict the long-term outcome of this enigmatic disease.

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