EVIDENCE FOR SUPERANTIGEN INVOLVEMENT IN CARDIOVASCULAR INJURY DUE TOKAWASAKI SYNDROME

Kawasaki syndrome (KS), the major cause of acquired heart disease in c hildren, is an acute multisystem vasculitis frequently associated with the development of myocarditis and coronary artery abnormalities. Des pite the widely held belief that KS is an infectious disease, its etio logy has remained elusive. Recently, we and others have reported the s elective expansion of V beta 2(+) T cells in the peripheral blood of m ost patients in the acute, but not in the convalescent, phase of KS. T hese data were consistent with the concept that this illness is trigge red by a bacterial superantigen. We report here that a patient who die d of acute KS had selective expansion of V beta 2(+) T cells in her my ocardium and coronary artery. Sequence analysis of TCR beta-chain gene s of V beta 2(+) T cells from the myocardium showed extensive junction al region diversity. These observations, along with the demonstration of V beta 2 expansion in both the CD4(+) and CD8(+) T cell subsets, su pport the concept that the activation of infiltrating V beta 2(+) T ce lls are involved in the cardiovascular damage associated with KS.