Defining the role of T lymphocytes in the immunopathogenesis of neuromyelitis optica spectrum disorder

Discov Med. 2020 Mar-Apr;29(157):91-102.

Abstract

Auto-reactive T cells are fundamental to many autoimmune processes, including neuromyelitis optica spectrum disorder (NMOSD). Several lines of evidence indicate that an antibody against aquaporin-4 (AQP4) is present in NMOSD patients. Further, this AQP4 antibody is pathogenic and can cause profound neurological damage. T cells are fundamental to many autoimmune processes, including NMOSD. Here we review work from animal models to discuss mechanisms by which auto-reactive T cells modulate the process by which antibodies cross the blood-brain barrier and orchestrate the local inflammatory milieu underlying NMOSD pathophysiology. We also examine clinical studies that document the presence of AQP4-specific T cells and the unique cytokine profile of NMOSD patients. This work encourages a renewed and broadened attention to the fundamental role of T cells in neuroautoimmune conditions which will hopefully lead to new therapies and better patients' outcomes.

MeSH terms

  • Animals
  • Aquaporin 4 / genetics
  • Aquaporin 4 / immunology*
  • Autoantibodies / immunology*
  • Blood-Brain Barrier / pathology*
  • Disease Models, Animal
  • Female
  • Humans
  • Mice, Knockout
  • Neuromyelitis Optica / immunology*
  • Neuromyelitis Optica / pathology
  • T-Lymphocytes / immunology*
  • T-Lymphocytes / metabolism

Substances

  • AQP4 protein, human
  • Aqp4 protein, mouse
  • Aquaporin 4
  • Autoantibodies