Recent developments and controversies in Kawasaki disease

In the developed world, Kawasaki disease is currently the leading cause of pediatric acquired heart disease. To date, the etiologic agent remains unknown. Many hypotheses regarding the etiology exist, and debate continues as to whether the inflammatory response of Kawasaki disease results from a superantigen or a conventional antigen. A variety of growth factors, proteinases, and cytokines have been identified that are involved in the pathogenesis of coronary artery disease in Kawasaki disease. These findings are leading to novel treatment strategies in Kawasaki disease, including platelet glycoprotein receptor inhibitors and monoclonal antibody to tumor necrosis factor-α. The role of corticosteroids remains controversial, and ongoing clinical trials are evaluating its efficacy. Additional studies have focused on newer non-invasive methods of evaluating children with coronary artery disease as alternatives to coronary catheterization. We review recent developments and controversies in exploring the etiology, pathogenesis, diagnosis, and management of Kawasaki disease.