The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes |
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| Authors: | Alexander Marx Frederick Pfister Berthold Schalke Güher Saruhan-Direskeneli Arthur Melms Philipp Ströbel |
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| Affiliation: | 1. Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, D-68167 Mannheim, Germany;2. Department of Neurology, Bezirkskrankenhaus, University of Regensburg, D-93042 Regens-burg, Germany;3. Department of Physiology, University of Istanbul, Istanbul Tip Fakultesi, Temel Bilimler, 34093 CAPA, Istanbul, Turkey;4. Universitätsklinikum Erlangen, Neurologische Klinik Schwabachanlage 6, D-91054 Erlangen, Germany;5. Institute of Pathology, University of Göttingen, Robert-Koch-Str. 40, D-37075 Göttingen, Germany;1. Department of Experimental Neurology, Charité—University Medicine Berlin, Germany;2. Department of Neurology, Charité—University Medicine Berlin, Germany;3. NeuroCure Clinical Research Center, Charité—University Medicine Berlin, Germany;4. Thoracic Surgery Division, Charité—University Medicine Berlin, Germany;5. Department of Medicine, Division of Rheumatology and Clinical Immunology, Charité—University Medicine Berlin, Germany;6. HELIOS Klinikum Emil von Behring, Department of Orthopaedics, Berlin, Germany;7. Department of Regenerative Immunology and Aging, Charité—University Medicine Berlin, Berlin-Brandenburg Center for Regenerative Therapies (BCRT), Berlin, Germany |
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| Abstract: | The thymus plays distinct roles in the pathogenesis of the different Myasthenia gravis (MG) subtypes. Inflammatory, neoplastic and age-related alterations of the thymus are of pivotal relevance for the initiation of anti-acetylcholine receptor (AChR) autoimmunity in early onset MG, thymoma-associated MG and, likely, late onset MG, respectively. By contrast, the thymus is presumably not related to MG that is due to autoantibodies to the muscle specific kinase, MuSK. Finally, the role of the thymus is still obscure in MG defined by antibodies against the agrin receptor LRP4 and in MG without all of the above autoantibdies (triple sero-negative MG) since these MG subtypes have been described only recently and thymectomy has not been their standard treatment. This review aims to give an update on intrathymic mechanisms of tolerance breakdown in MG, including abnormal T cell selection and activation, the role of thymic myoid cells, the autoimmune regulator (AIRE) and regulatory T cells. |
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