title: The thymus in myasthenia gravis: Site of "innate autoimmunity"?
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.
authors.affiliation: Department of Neurology, Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico Carlo Besta, Milan, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Cavalcante
authors.forename: Rozen
authors.initials: R
authors.lastname: Le Panse
authors.forename: Sonia
authors.initials: S
authors.lastname: Berrih-Aknin
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Maggi
authors.forename: Carlo
authors.initials: C
authors.lastname: Antozzi
authors.forename: Fulvio
authors.initials: F
authors.lastname: Baggi
authors.forename: Pia
authors.initials: P
authors.lastname: Bernasconi
authors.forename: Renato
authors.initials: R
authors.lastname: Mantegazza
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007113:Immunity, Innate / Q000502:physiology*; D009157:Myasthenia Gravis / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D013950:Thymus Gland / Q000002:abnormalities / Q000276:immunology* / Q000378:metabolism
chemical_list: D001323:Autoantibodies