Kawasaki disease: an update.

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.