Polymyositis mediated by T lymphocytes that express the γ/δ receptor

Background. The invasion and destruction of nonnecrotic muscle fibers by CD8+ cytotoxic T cells is considered a hallmark of polymyositis. In the cases of polymyositis reported so far, the autoinvasive CD8+ T cells expressed the common form of T-cell receptor for the recognition of antigen, the so-called α/β T-cell receptor. We describe a 69-year-old man with polymyositis mediated by CD4—, CD8— T cells expressing the recently discovered, uncommon γ/δ T-cell receptor. Methods. We used immunofluorescence or immunoperoxidase techniques to study frozen sections of muscle from our patient, who had mild weakness of cervical and proximal limb muscles, and from control patients with polymyositis, inclusion-body myositis, dermatomyositis, or granulomatous myopathy with monoclonal antibodies against T-cell-related antigens (CD2, CD3, CD4, CD8, and γ/δ T-cell receptor), B cells (CD22), major histocompatibility complex (MHC) and MHC-related antigens (MHC Class I, CD1a, CD1b, and CD1c), and the 65-kd heat-shock protein. The membrane contacts between the autoinvasive cells and the sarcolemma were investigated by electron microscopy. Results. In the patient described here, but not in 28 others with inflammatory myopathies, myriad γ/δ T cells surrounded and invaded nonnecrotic muscle fibers. All muscle fibers were highly reactive for MHC Class I antigen and the 65-kd heat-shock protein. Treatment with prednisone improved the clinical and histologie findings. Conclusions. Polymyositis can be mediated by γ/δ T cells. This new form of polymyositis appears to be highly responsive to steroids. (N Engl J Med 1991; 324:877–81.)