Kawasaki disease and the T-cell antigen receptor.
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.
Jason
J
J
Department of Health and Human Services, Public Health Service, Atlanta, GA, USA.
Montana
E
E
Donald
J F
JF
Seidman
M
M
Inge
K L
KL
Campbell
R
R
D000208:Acute Disease; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007223:Infant; D019041:L-Selectin / Q000276:immunology; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000276:immunology* / Q000628:therapy; D017260:Receptor-CD3 Complex, Antigen, T-Cell / Q000276:immunology*; D018709:Statistics, Nonparametric; D005719:gamma-Globulins / Q000008:administration & dosage
D017260:Receptor-CD3 Complex, Antigen, T-Cell; D005719:gamma-Globulins; D019041:L-Selectin