Relation of streptococcal pyrogenic exotoxin C as a causative superantigen for Kawasaki disease

Pediatr Res. 2003 Mar;53(3):403-10. doi: 10.1203/01.PDR.0000049668.54870.50.


We previously reported that the frequency of TCRBV2 and TCRBV6S5-bearing T-cells was high in patients in the acute phase of Kawasaki disease (KD) and that streptococcal pyrogenic exotoxin C (SPE-C) was a potent stimulator of these TCRBV-bearing T-cells. To further elucidate the pathogenesis of KD, we examined the T-cell receptor (TCR) repertoire, human leukocyte antigen (HLA)-DRB1 genotype, and antibody responses to recombinant(r) SPE-C in patients with KD. We also performed in vitro stimulation with rSPE-A and rSPE-C of peripheral blood mononuclear cells from healthy donors and characterized the reacting T-cells. The percentage of T-cells bearing TCRBV2 and TCRBV6S5 was high in patients in the acute stage of KD. rSPE-C stimulation of PBMC from healthy donors induced expansion of TCRBV2 and TCRBV6S5-bearing T-cells. Furthermore, serum levels of anti-SPEC antibodies, which did not display antimitogenic activity, were higher in patients with acute KD than in age-matched controls. The frequencies of the DRB1*04051, 0406, and 0901 were high, whereas that of the DRB1*1101 was low among patients with KD as compared with the healthy adults.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibodies, Bacterial / blood
  • Bacterial Proteins*
  • Child, Preschool
  • Exotoxins / analysis*
  • Exotoxins / immunology
  • Female
  • Gene Frequency
  • Genotype
  • HLA-DR Antigens / genetics
  • HLA-DRB1 Chains
  • Humans
  • Infant
  • Male
  • Membrane Proteins*
  • Mucocutaneous Lymph Node Syndrome / genetics
  • Mucocutaneous Lymph Node Syndrome / immunology*
  • Mucocutaneous Lymph Node Syndrome / microbiology*
  • Streptococcal Infections / complications*
  • Streptococcal Infections / genetics
  • Streptococcal Infections / immunology*
  • Superantigens / analysis
  • Superantigens / immunology
  • T-Lymphocytes / microbiology


  • Antibodies, Bacterial
  • Bacterial Proteins
  • Exotoxins
  • HLA-DR Antigens
  • HLA-DRB1 Chains
  • Membrane Proteins
  • SpeA protein, Streptococcus pyogenes
  • Superantigens
  • erythrogenic toxin