Roxanis Ioannis, Micklem Kingsley, McConville John, Newsom-Davis John, Willcox Nick
Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK.
J Neuroimmunol. 2002 Apr;125(1-2):185-97. doi: 10.1016/s0165-5728(02)00038-3.
In early-onset myasthenia gravis (EOMG), the thymus usually shows medullary lymph node-type infiltrates, rearranged bands of hyperplastic epithelium and focal fenestrations in the intervening laminin borders. This resemblance to autoimmune target organs may reflect autoantigen expression by rare thymic myoid cells, long ago implicated circumstantially as agents provocateurs. In this quantitative study, they were frequently seen at the laminin fenestrations; if so, germinal centers (GC) were significantly commoner nearby, our most EOMG-specific finding (not seen in a distinct MG patient subset). As autoantibodies became detectable, myoid cell involvement apparently progressed. Our unifying hypothesis--that an early autoantibody attack on myoid cells provokes local GC formation--helps to resolve many puzzles.
在早发型重症肌无力(EOMG)中,胸腺通常表现为髓质淋巴结样浸润、增生上皮的重排带以及中间层粘连蛋白边界处的局灶性窗孔。这种与自身免疫靶器官的相似性可能反映了罕见胸腺肌样细胞的自身抗原表达,很久以前就有人间接认为它们是激发因素。在这项定量研究中,它们经常出现在层粘连蛋白窗孔处;如果是这样,生发中心(GC)在附近明显更常见,这是我们最具EOMG特异性的发现(在一个独特的重症肌无力患者亚组中未见到)。随着自身抗体变得可检测到,肌样细胞的受累显然在进展。我们的统一假设——即早期自身抗体对肌样细胞的攻击引发局部GC形成——有助于解决许多谜题。