Marx, Alexander and Willcox, N. and Leite, M. I. and Chuang, W. -Y. and Schalke, B. and Nix, W. and Stroebel, P. (2010) Thymoma and paraneoplastic myasthenia gravis. AUTOIMMUNITY, 43 (5-6). pp. 413-427. ISSN 0891-6934, 1607-842X
Full text not available from this repository. (Request a copy)Abstract
Parancoplastic autoimmune diseases associate occasionally with small cell lung cancers and gynecologic tumors. However, myasthenia gravis (MG) occurs in at least 30% of all patients with thymomas (usually present at MG diagnosis). These epithelial neoplasms almost always have numerous admixed maturing polyclonal T cells (thymocytes). This thymopoiesis- and export of mature CD4(+)T cells-particularly associates with MG, though there are rare/puzzling exceptions in apparently pure epithelial WHO type A thymomas. Other features potentially leading to inefficient self-tolerance induction include defective epithelial expression of the autoimmune regulator (AIRE) gene and/or of major histocompatibility complex class II molecules in thymomas, absence of myoid cells, failure to generate FOXP3(+) regulatory T cells, and genetic polymorphisms affecting T-cell signaling. However, the strong focus on MG/neuromuscular targets remains unexplained and suggests some biased autoantigen expression, T-cell selection, or autoimmunization within thymomas. There must be further clues in the intriguing serological and cellular parallels in some patients with late-onset MG but without thymomas and in others with AIRE mutations-and in the contrasts with early-onset MG, as discussed here.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | THYMIC EPITHELIAL TUMORS; REGULATORY T-CELLS; NICOTINIC ACETYLCHOLINE-RECEPTOR; SYNDROME TYPE-I; MYOID CELLS; RYANODINE RECEPTOR; AUTOIMMUNIZING MECHANISMS; GENETIC ABERRATIONS; SINGLE-INSTITUTION; STRIATED-MUSCLE; Autoimmunity; thymoma; myasthenia gravis |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 20 Jul 2020 06:05 |
| Last Modified: | 20 Jul 2020 06:05 |
| URI: | https://pred.uni-regensburg.de/id/eprint/24345 |
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