PMID: 9174625May 1, 1997Paper

Pathogenesis of myasthenia gravis

Virchows Archiv : an International Journal of Pathology
Alexander MarxH K Müller-Hermelink

Abstract

Various studies over the last 25 years in Man and animal models have revealed many steps in the pathogenesis of myasthenia gravis (MG) which is now considered the classical organ specific, autoantibody mediated and T cell dependent human autoimmune disease. Though not a disease entity, MG is associated with pathological alterations of the thymus in about 80% of cases. These are described here with reference to distinct models of autoimmunization against the acetylcholine receptor (AChR). In MG with thymitis, intrathymic production of AChR-specific autoantibodies is the result of a classical antigen-driven immune reaction that occurs completely inside the thymus and probably involves AChR on myoid cells as the triggering (myasthenogenic) antigen. Genetic factors contribute essentially to the pathogenesis of this form of MG. In thymoma-associated MG genetic factors are probably of marginal significance. Neither intratumour autoantibody production nor T cell activation seem to occur and the AChR is not the myasthenogenic antigen. Instead, abnormal neurofilaments that share epitopes with the AChR and other auto-antigen targets in paraneoplastic MG are expressed in thymomas and may trigger autoantigen-specific, non-tolerogenic T cel...Continue Reading

Citations

Aug 12, 1999·Annals of Neurology·A SchultzA Marx
Jun 2, 2012·Der Nervenarzt·M SchneiderF Then Bergh
May 29, 2012·Current Oncology Reports·Mark MikhailTarek Mekhail
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Nov 7, 2003·The Annals of Thoracic Surgery·Manuel López-CanoManuel Armengol-Carrasco
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Sep 4, 2012·Current Opinion in Neurology·Paola CavalcanteRenato Mantegazza
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Oct 10, 2014·Continuum : Lifelong Learning in Neurology·Donald B Sanders, Jeffrey T Guptill
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May 23, 2015·The International Journal of Neuroscience·Yongxiang YangZhuyi Li
Oct 8, 1998·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·M B Oldstone
Nov 1, 2003·Annals of the New York Academy of Sciences·Alexander MarxPhilipp Ströbel
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