期刊
AUTOIMMUNITY
卷 43, 期 5-6, 页码 413-427出版社
TAYLOR & FRANCIS LTD
DOI: 10.3109/08916930903555935
关键词
Autoimmunity; thymoma; myasthenia gravis
类别
资金
- European Union [LSHB-CT-2003-503410]
- European Union, EuroMyasthenia Network [2005105]
- Deutsche Krebshilfe [106430]
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.
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