The thymus in myasthenia gravis: Site of "innate autoimmunity"?

Paola Cavalcante, Rozen le Panse, Sonia Berrih-Aknin, Lorenzo Maggi, Carlo Antozzi, Fulvio Baggi, Pia Bernasconi, Renato Mantegazza

Research output: Contribution to journalArticle

Abstract

Myasthenia gravis (MG) is an autoimmune disorder caused, in most cases, by autoantibodies against components of the neuromuscular junction, frequently the acetylcholine receptor (AChR), and less often the muscle-specific kinase receptor. The thymus plays a major role in the pathogenesis of MG with anti-AChR antibodies: it shows marked pathologic alterations (hyperplastic or tumoral) in most AChR-positive patients and contains the elements required to initiate and sustain an autoimmune reaction (AChR autoantigen, AChR-specific T cells, and autoantibody-secreting plasma cells). In this study we review early and more recent findings implicating the thymus as site of AChR autosensitization in MG and briefly discuss the therapeutic role of thymectomy. We also summarize data showing that the MG thymus is in a state of chronic inflammation, and we review emerging evidence of a viral contribution to the onset and maintenance of the thymic autoimmune response.

Original languageEnglish
Pages (from-to)467-484
Number of pages18
JournalMuscle and Nerve
Volume44
Issue number4
DOIs
Publication statusPublished - Oct 2011

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Keywords

  • Inflammation
  • Innate immunity
  • Myasthenia gravis
  • Thymus
  • Viral infection

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)
  • Physiology

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