B lymphocytes in neuromyelitis optica

Jeffrey L. Bennett, Kevin C. O'Connor, Amit Bar-Or, Scott S. Zamvil, Bernhard Hemmer, Thomas F. Tedder, H. Christian Von Budingen, Olaf Stuve, Michael R. Yeaman, Terry J. Smith, Christine Stadelmann

Research output: Contribution to journalReview article

59 Scopus citations

Abstract

Neuromyelitis optica (NMO) is an inflammatory autoimmune disorder of the CNS that predominantly affects the spinal cord and optic nerves. A majority (approximately 75%) of patients with NMO are seropositive for autoantibodies against the astrocyte water channel aquaporin-4 (AQP4). These autoantibodies are predominantly IgG1, and considerable evidence supports their pathogenicity, presumably by binding to AQP4 on CNS astrocytes, resulting in astrocyte injury and inflammation. Convergent clinical and laboratory-based investigations have indicated that B cells play a fundamental role in NMO immunopathology. Multiple mechanisms have been hypothesized: AQP4 autoantibody production, enhanced proinflammatory B cell and plasmablast activity, aberrant B cell tolerance checkpoints, diminished B cell regulatory function, and loss of B cell anergy. Accordingly, many current off-label therapies for NMO deplete B cells or modulate their activity. Understanding the role and mechanisms whereby B cells contribute to initiation, maintenance, and propagation of disease activity is important to advancing our understanding of NMO pathogenesis and developing effective disease-specific therapies.

Original languageEnglish (US)
Pages (from-to)e104
JournalNeurology: Neuroimmunology and NeuroInflammation
Volume2
Issue number3
DOIs
StatePublished - Jun 2015

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Bennett, J. L., O'Connor, K. C., Bar-Or, A., Zamvil, S. S., Hemmer, B., Tedder, T. F., Von Budingen, H. C., Stuve, O., Yeaman, M. R., Smith, T. J., & Stadelmann, C. (2015). B lymphocytes in neuromyelitis optica. Neurology: Neuroimmunology and NeuroInflammation, 2(3), e104. https://doi.org/10.1212/NXI.0000000000000104