title: The thymus in autoimmune Myasthenia Gravis: Paradigm for a tertiary lymphoid organ.
abstract: 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.
authors.affiliation: Unité mixte de recherche CNRS UMR7215/Inserm U974/UPMC UM76/AIM, thérapie des maladies du muscle strié, institut de myologie, groupe hospitalier Pitié-Salpêtrière, 105, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
authors.forename: J-M
authors.initials: JM
authors.lastname: Weiss
authors.forename: P
authors.initials: P
authors.lastname: Cufi
authors.forename: R
authors.initials: R
authors.lastname: Le Panse
authors.forename: S
authors.initials: S
authors.lastname: Berrih-Aknin
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000378:metabolism; D006801:Humans; D008221:Lymphoid Tissue / Q000254:growth & development / Q000276:immunology*; D009157:Myasthenia Gravis / Q000473:pathology / Q000503:physiopathology*; D013950:Thymus Gland / Q000473:pathology / Q000502:physiology*; D013952:Thymus Hyperplasia / Q000276:immunology / Q000378:metabolism; D014777:Virus Diseases / Q000150:complications / Q000276:immunology
chemical_list: D001323:Autoantibodies
keywords: Autoimmune Myasthenia Gravis; Interferons; Interférons; Myasthénie autoimmune; Néogenèse lymphoïde tertiaire; Tertiary lymphoid neogenesis; Thymus; Voie de signalisation de l’ARNdb; dsRNA signaling