Polymyositis mediated by γ/δ T cells is a unique disease in which autoaggressive T lymphocytes surround, invade, and destroy muscle fibers. Histochemically, the vast majority of muscle-infiltrating T cells in a patient with polymyositis were reactive with a pan-γ/δ T cell receptor (TCR)-specific monodonal antibody (TCR-δ1+), but unlike >90% of peripheral blood γ/δ T cells, these lymphocytes did not react with Vδ1- or Vγ9-specific antibodies (A13− and TiγA−, respectively). Differential reactivity with two different Vδ2-specific monoclonal antibodies (BB3−/TiV-δ2+) indicated that the infiltrating T cells express a Vδ2-containing TCR with unusual additional structural features. Using conventional and anchored polymerase chain reaction for the analysis of TCR transcripts, we found a striking predominance of one unusual Vδ2-Jδ3 recombination and one Vγ3-Jγ1 recombination. Both the unusual phenotype (TCR-δ1+/A13−/TiγA−/BB3−/TiV-δ2+) and the dominance of distinct TCR transcripts are compatible with the assumption that one T cell done, which expresses a Vγ3-Jγ1-Cγ1/Vδ2-Jδ3-Cδ disulfidelinked TCR, dominates among the infiltrating T cells of the polymyositis muscle specimen analyzed.
ASJC Scopus subject areas
- Immunology and Allergy