全身性重症肌无力患者血清补体及其调节因子的变化。

Changes in serum complements and their regulators in generalized myasthenia gravis.

机构信息

Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.

Department of Neurology, Kyoto Prefectural University of Medicine, Kyoto, Japan.

出版信息

Eur J Neurol. 2021 Jan;28(1):314-322. doi: 10.1111/ene.14500. Epub 2020 Sep 27.

DOI:10.1111/ene.14500

PMID:32889770

Abstract

OBJECTIVE

To investigate changes in serum complements and their regulators in the pathogenesis of myasthenia gravis (MG).

METHODS

Forty-four patients with acetylcholine receptor antibody-positive MG, as well as 20 patients with non-inflammatory neurological disorders were enrolled. Serum complements (C3, C4 and soluble C5b-9) and complement regulators (vitronectin, clusterin and properdin) were extensively analysed by enzyme-linked immunosorbent assay and their associations with clinical profiles of MG were examined.

RESULTS

Serum C3, C4 and clusterin levels were not significantly different between patients with MG and controls. The patients with MG had higher soluble C5b-9 (P = 0.09) and vitronectin (P = 0.001) levels than the controls; moreover, vitronectin levels decreased after treatment (P = 0.09). Serum properdin (P = 0.03) levels were lower in the patients with MG than in the controls, and negatively correlated with the MG Activities of Daily Living score (rs = -0.26, P = 0.09) and with the presence of bulbar palsy (P = 0.04).

CONCLUSION

Our results show that activation of complements and an altered complement network could contribute to the inflammatory pathogenesis of MG.

摘要

目的

探讨重症肌无力（MG）发病机制中血清补体及其调节因子的变化。

方法

纳入 44 例乙酰胆碱受体抗体阳性的 MG 患者和 20 例非炎症性神经疾病患者。采用酶联免疫吸附试验检测血清补体（C3、C4 和可溶性 C5b-9）和补体调节因子（纤连蛋白、聚类素和备解素），并分析其与 MG 临床特征的关系。

结果

MG 患者与对照组之间血清 C3、C4 和聚类素水平无显著差异。MG 患者可溶性 C5b-9（P=0.09）和纤连蛋白（P=0.001）水平较高，且治疗后纤连蛋白水平下降（P=0.09）。MG 患者血清备解素（P=0.03）水平低于对照组，与 MG 日常生活活动评分（rs=-0.26，P=0.09）和延髓麻痹的存在呈负相关（P=0.04）。