Myasthenia Gravis: An Acquired Interferonopathy?

Cells. 2022 Apr 4;11(7):1218. doi: 10.3390/cells11071218.


Myasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IFN-I) signature is clearly detected in the thymus. The origin of this chronic IFN-I expression in the thymus is not yet defined. IFN-I subtypes are normally produced in response to viral infection. However, genetic diseases called interferonopathies are associated with an aberrant chronic production of IFN-I defined as sterile inflammation. Some systemic autoimmune diseases also share common features with interferonopathies. This review aims to analyze the pathogenic role of IFN-I in these diseases as compared to AChR-MG in order to determine if AChR-MG could be an acquired interferonopathy.

Keywords: adaptive immunity; autoimmunity; germinal center; innate immunity; interferon type I; myasthenia gravis; pathogen infection; sterile inflammation; thymoma; thymus.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies
  • Graft vs Host Disease*
  • Humans
  • Myasthenia Gravis*
  • Receptors, Cholinergic
  • Thymoma* / complications
  • Thymoma* / pathology
  • Thymus Neoplasms*


  • Autoantibodies
  • Receptors, Cholinergic