Kawasaki disease and the T-cell antigen receptor

Hum Immunol. 1998 Jan;59(1):29-38. doi: 10.1016/s0198-8859(97)00233-4.


We investigated the evidence for an infectious etiology of Kawasaki disease (KD), an acute vasculitis of unknown etiology, by assessing the effects of KD on the T cell antigen receptor variable beta region families (V beta). Using 3-color flow cytometry, we studied KD patients pre- and post-intravenous gamma globulin (IVIG) therapy and at > 40 days post therapy, additionally comparing them to matched pediatric control patients (PCC) and their own healthy parents (one parent/KD child). Of all the V beta families examined, only V beta 2 exhibited statistically significant differences, between the pre- and post-IVIG samples and preIVIG and parent samples. No associations were found between V beta 2 findings and T cell memory, activation, or adhesion markers. For 2 KD patients, 4 parents, and 1 PCC participant, > 15% of resting CD8+ lymphocytes and > 15% of blastic CD8+ lymphocytes expressed a single V beta family, which varied by individual, without similar expansions in the CD4+ cell populations. One of the participants with this abnormality was the only one with significant cardiac abnormalities. For all participants with the V beta abnormality, other T-cell abnormalities were extensive and involved both CD4+ and CD8+ cells. We suggest that V beta 2 changes do occur in KD, as previously reported. However, these may not be involved in disease pathogenesis. Other V beta changes also occur. Those occurring in parents may reflect asymptomatic reinfection with an infectious agent causing KD. Further, some KD patients may have restricted cytotoxic T-cell responses to that as yet unidentified agent; this restricted response may be associated with more severe cardiac involvement.

Publication types

  • Clinical Trial
  • Controlled Clinical Trial

MeSH terms

  • Acute Disease
  • CD4-Positive T-Lymphocytes / immunology*
  • CD8-Positive T-Lymphocytes / immunology*
  • Child
  • Child, Preschool
  • Female
  • Flow Cytometry
  • Humans
  • Infant
  • L-Selectin / immunology
  • Male
  • Mucocutaneous Lymph Node Syndrome / immunology*
  • Mucocutaneous Lymph Node Syndrome / therapy
  • Receptor-CD3 Complex, Antigen, T-Cell / immunology*
  • Statistics, Nonparametric
  • gamma-Globulins / administration & dosage


  • Receptor-CD3 Complex, Antigen, T-Cell
  • gamma-Globulins
  • L-Selectin