T cell activation profiles in Kawasaki syndrome

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Abstract
Superantigens (SAgs) are potent stimulators of T cells bearing specific Vbeta T cell receptors (TCR) and may play a role in the pathogenesis of Kawasaki syndrome (KS), although despite 15 years of intense study this area remains controversial. Because SAgs can cause Vbeta restricted T cell activation in the absence of Vbeta skewing the aims of this study were to describe a flow cytometric protocol to study both CD4 and CD8 Vbeta repertoires, and CD69 expression across the CD4 and CD8 Vbeta repertoire in children with KS. Sixteen children with KS were studied. There was no significant increase in overall peripheral blood CD4 or CD8 T cell activation as determined by CD69 expression. However, Vbeta restricted CD4 and/or CD8 activation was observed in eight of 11 (72%) of the KS patients, a finding not observed in healthy controls. Thirteen of 16 (81%) of the KS patients had evidence of either Vbeta skewing (particularly CD4 Vbeta2 and Vbeta5.1) and/or Vbeta restricted activation. Three patients had Vbeta restricted activation in the absence of skewing. We suggest that these preliminary observations highlight the many layers of complexity when considering T cell activation in KS, which could explain some of the conflicting studies regarding peripheral blood T cell activation and Vbeta skewing. It is likely that in order to move forward with this debate a combination of detailed microbiological, immunological and molecular techniques applied to individual patients will be required ultimately to prove or refute the SAg hypothesis of KS.