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Ann N Y Acad Sci. 2018 Jan;1412(1):137-145. doi: 10.1111/nyas.13519. Epub 2017 Nov 10.

Thymus involvement in early-onset myasthenia gravis.

Cron MA1,2,3, Maillard S1,2,3, Villegas J1,2,3, Truffault F1,2,3, Sudres M1,2,3, Dragin N1,2,3, Berrih-Aknin S1,2,3, Le Panse R1,2,3.

Author information

  • 1INSERM U974, Paris, France.
  • 2UPMC Sorbonne Universités, Paris, France.
  • 3AIM, Institut de myologie, Paris, France.

Abstract

It has long been established that the thymus plays a central role in autoimmune myasthenia gravis (MG) because of either thymoma or thymic hyperplasia of lymphoproliferative origin. In this review, we discuss thymic changes associated with thymic hyperplasia and their implications in the development of an autoimmune response against the acetylcholine receptor (AChR).The hyperplastic MG thymus displays all the characteristics of tertiary lymphoid organs (TLOs): neoangiogenic processes with high endothelial venule and lymphatic vessel development, chemokine overexpression favoring peripheral cell recruitment, and ectopic germinal center development. As thymic epithelial cells or myoid cells express AChR, a specific antigen presentation can easily occur within the thymus in the presence of recruited peripheral cells, such as B cells and T follicular helper cells. How the thymus turns into a TLO is not known, but local inflammation seems mandatory. Interferon (IFN)-β is overexpressed in MG thymus and could orchestrate thymic changes associated with MG. Knowledge about how IFN-β is induced in MG thymus and why its expression is sustained even long after disease onset would be of interest in the future to better understand the etiological and physiopathological mechanisms involved in autoimmune MG.

KEYWORDS:

chemokines; germinal centers; interferon type-I; miRNAs; pathogen infection

PMID:
29125185
DOI:
10.1111/nyas.13519
[PubMed - in process]

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