Mendell J R, Garcha T S, Kissel J T
Department of Neurology, College of Medicine of the Ohio State University, Columbus 43210, USA.
Curr Opin Neurol. 1996 Jun;9(3):226-34. doi: 10.1097/00019052-199606000-00014.
The complement system has been shown to play a pathogenic role in only a small number of muscle diseases. A complement-induced microangiopathy is important in dermatomyositis and in the rare disorder, necrotizing myopathy with pipestem capillaries. Recent studies demonstrate the reversibility of microvascular damage in dermatomyositis by intravenous immune globulin which appears to intercept the assembly and deposition of membrane attach complex. In myasthenia gravis, complement-mediated lysis directed at the acetylcholine receptor leads to a cascade of events reducing the number of receptors, simplifying the complex architecture of the junctional folds and decreasing the available surface for the insertion of new receptors. The newest condition in which a role for complement has been demonstrated is X-linked vacuolated myopathy. The condition is of interest because membrane attack complex deposits result in shedding of complement-laden membrane fragments in contrast to a cytolytic process as the consequence of assembly of the terminal complement components.
补体系统仅在少数肌肉疾病中发挥致病作用。补体诱导的微血管病在皮肌炎和罕见的伴有管状毛细血管的坏死性肌病中很重要。最近的研究表明，静脉注射免疫球蛋白可使皮肌炎中的微血管损伤可逆，这似乎能阻断膜攻击复合物的组装和沉积。在重症肌无力中，针对乙酰胆碱受体的补体介导的溶解会引发一系列事件，减少受体数量，简化突触皱襞的复杂结构，并减少新受体插入的可用表面积。补体发挥作用的最新疾病是X连锁空泡性肌病。这种疾病之所以令人关注，是因为膜攻击复合物沉积导致富含补体的膜碎片脱落，这与末端补体成分组装导致的细胞溶解过程不同。
