Kawasaki disease: 40 years after the original report

The cause of Kawasaki disease (KD) remains unknown, although a number of epidemiologic and clinical observations suggest it is triggered by one or more 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), which 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 who fail to respond to IVIG. It appears that some patients with severe KD who are resistant to IVIG may benefit from IV pulse steroid therapy or infliximab infusion. However, a recent multicenter, randomized, controlled trial did not support the addition of a pulsed dose of intravenous methylprednisolone to the conventional IVIG therapy for the primary treatment of KD. It remains to be seen whether other anti-inflammatory agents such as immunosuppressive therapies or new biologics will play a role in the management of patients with KD.