Department of Neurology, University of Pecs, Pecs, Hungary.
J Neuroimmunol. 2011 Feb;231(1-2):43-54. doi: 10.1016/j.jneuroim.2010.10.020. Epub 2010 Nov 19.
Although myasthenia gravis (MG) has long been considered a well-established autoimmune disease associated with autoantibodies, which are convincingly pathogenic, accumulating data indicate both clinical and biological heterogeneity similar to many other putative autoimmune disorders. In a subset of patients, thymus plays a definite role: thymic autoimmunity results in generation of autoantibodies within the thymus, which cross-react with antigens at the neuromuscular junction, or thymoma leads to deficient central tolerance and impaired T cell selection. Heterogeneity on the autoantibody level may be associated with genetic heterogeneity and clinical phenotypes with different treatment responses.
尽管重症肌无力 (MG) 长期以来一直被认为是一种与自身抗体相关的明确的自身免疫性疾病,这些自身抗体具有明显的致病性,但越来越多的数据表明,其存在与许多其他假定的自身免疫性疾病相似的临床和生物学异质性。在一部分患者中,胸腺起着明确的作用:胸腺自身免疫导致自身抗体在胸腺内产生,这些自身抗体与神经肌肉接头的抗原发生交叉反应,或胸腺瘤导致中枢耐受缺陷和 T 细胞选择受损。在自身抗体水平上的异质性可能与遗传异质性和不同治疗反应的临床表型相关。