Kawasaki disease: A maturational defect in immune responsiveness

Kawasaki disease (KD), an acute febrile disease in children of unknown etiology, is characterized by a vasculitis that may result in coronary artery aneurysms (CAAs). In new patients with KD, a selective and prolonged T cell unresponsiveness to activation via the T cell antigen receptor CD3 was observed, whereas proliferation to other stimuli was intact. This "split T cell anergy" delineated KD from other pediatric infections and autoimmune diseases and correlated with CAA formation (P < .001), A transient immune dysfunction was also suggested by an incomplete responsiveness to measles-mumps-rubella (MMR) vaccination in patients with KD versus controls (P < .0001; odds ratio, 15.6; 95% confidence interval, 4.8-51.1), which was overcome by revaccination(s), The reduced responsiveness to MMR in patients with KD suggests a subtle and predetermining immune dysfunction, An inherent immaturity to dear certain antigens may be an important cause that precipitates KD and the immune dysregulation during acute disease.