The thymus in autoimmune Myasthenia Gravis: Paradigm for a tertiary lymphoid organ.
Rev Neurol (Paris)
; 169(8-9): 640-9, 2013.
Article
en En
| MEDLINE
| ID: mdl-24008049
In autoimmune Myasthenia Gravis (MG), a neuromuscular disease generally mediated by autoantibodies against the acetylcholine receptor (AChR), the muscle is the target organ of the autoimmune attack, while the thymus seems to be the primary production site of the autoantibodies. In the majority of patients with anti-AChR antibodies, it is characterized by the presence of germinal centers, which contain B cells that produce anti-AChR antibodies. In this review, we summarize recent results regarding neoangiogenic processes, cell infiltration and modified chemokine expression in the MG thymus, which are typical features of secondary lymphoid organs. The structural and functional changes in the MG thymus therefore allow us to declare it to be an archetype for tertiary lymphoid neogenesis providing optimal settings for the interaction between lymphocytes and antigen presenting cells in order to elicit an immune response. We further discuss factors that may have a key role in the transformation of the MG thymus into a tertiary lymphoid organ, such as IFN type I and dsRNA signaling. These factors could also be of importance in other autoimmune diseases, especially those characterized by tertiary lymphoid neogenesis.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Timo
/
Tejido Linfoide
/
Miastenia Gravis
Límite:
Animals
/
Humans
Idioma:
En
Revista:
Rev Neurol (Paris)
Año:
2013
Tipo del documento:
Article
País de afiliación:
Francia
Pais de publicación:
Francia