SOLUBLE TERMINAL COMPLEMENT COMPONENTS IN HUMAN MYASTHENIA-GRAVIS
SOLUBLE TERMINAL COMPLEMENT COMPONENTS IN HUMAN MYASTHENIA-GRAVIS
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DOI：
10.1016/0303-8467(93)90103-n
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发表时间：
1993-12-01
影响因子：
1.9
通讯作者：
BREY, RL
中科院分区：
文献类型：
--
作者：
BAROHN, RJ;BREY, RL
重症肌无力（MG）患者的肌膜乙酰胆碱受体（AChR）缺失导致肌无力和神经肌肉接头（NMJ）传递受损，部分原因是补体介导的肌膜损伤。这得到了人MG中NMJ处C9的组织学发现的支持。我们使用ELISA法检测了31例MG患者和健康对照者的42份血浆样本中的SC 5 b-9，以评估血浆中终末补体成分的证据。当绘制在标准阳性稀释曲线上时，在31例患者中的18例（58%）中发现了一个或多个时间点的SC 5 b-9异常升高。随着时间的推移，可从8例患者获得多份样本。部分（但非全部）患者的临床恶化伴随SC 5 b-9值升高。MG严重程度或AChR抗体水平与SC 5 b-9值之间在整个组中没有明显区别。这支持了补体介导的肌膜损伤在人MG发病机制中的潜在作用，但也证明了通过ELISA测量的血浆水平并不总是与疾病活动相关。
The loss of membrane acetylcholine receptor (AChR) leading to muscle weakness and impaired neuromuscular junction (NMJ) transmission in human myasthenia gravis (MG) is in part due to complement mediated muscle membrane damage. This has been supported by the histologic finding of C9 at the NMJ in human MG. We evaluated for evidence of terminal complement components in plasma by using an ELISA for SC5b-9 in 42 separate plasma samples from 31 patients with MG and from healthy controls. Abnormal elevations of SC5b-9 was found in 18 of 31 patients (58%) at one or more time points when plotted on a standard positive dilution curve. Multiple samples were available from 8 patients over time. Clinical deterioration in some, but not all, was accompanied by an increase in SC5b-9 values. There was no clear distinction in the group as a whole between MG severity or AChR antibody levels and SC5b-9 values. This supports the potential role of complement-mediated muscle membrane damage in the pathogenesis of human MG, but also demonstrates that plasma levels as measured by ELISA do not always correlate with disease activity.