Frontiers of Kawasaki disease research

Abstract

Kawasaki disease (KD), first described by Dr. Tomisaku Kawasaki in 1967 in Japan, is one of the most common pediatric systemic vasculitides of unknown etiology. The most serious clinical issue in KD is formation of coronary artery lesions due to severe inflammation of the coronary arteries. Prevention of this complication is the most important goal of treatment of acute-phase KD. In the 2000s, intravenous immunoglobulin (IVIG) has become the standard treatment for acute KD. IVIG effectively suppresses coronary artery inflammation and has dramatically reduced the incidence of aneurysms. Many clinical and genomic studies have led to further advances in KD treatment. In particular, an immunosuppressant drug, cyclosporine, was recently approved for KD. That was an important accomplishment based on basic genome research, and it represents the direction that future research should take. On the other hand, despite advances in treatment, about 300 patients still develop cardiac sequelae annually in Japan. KD is the leading cause of childhood-onset acquired heart disease in developed countries, including Japan. To overcome this problem, we need to continue developing more disease-specific, effective therapeutic agents based on pathological mechanisms.