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双重滤过血浆置换在降低神经免疫性疾病中免疫球蛋白和致病抗体水平的疗效:来自中国的单中心回顾性分析。

Effectiveness of double-filtration plasmapheresis in reducing immunoglobulin and culprit antibody levels in neuroimmune disorders: A single-center retrospective analysis from China.

机构信息

Department of Neurology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, PR China.

Department of Neurology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, PR China.

出版信息

J Neuroimmunol. 2024 Nov 15;396:578463. doi: 10.1016/j.jneuroim.2024.578463. Epub 2024 Oct 4.

DOI:10.1016/j.jneuroim.2024.578463
PMID:39396401
Abstract

OBJECTIVE

This study aims to evaluate the effectiveness of double-filtration plasmapheresis (DFPP) in reducing immunoglobulins and culprit antibodies in neuroimmune disorders.

METHODS

A retrospective analysis was conducted on 51 patients with neuroimmune diseases treated with DFPP, immunotherapy, and symptomatic treatment. Immunoglobulin and antibody levels were measured pre- and post-treatment, along with neurological function assessments using scales like the modified Rankin Scale (mRS), Expanded Disability Status Scale (EDSS), Clinical Assessment Scale for Autoimmune Encephalitis (CASE), and Myasthenia Gravis-specific scales.

RESULTS

The cohort included patients with neuromyelitis optica spectrum disorder (NMOSD), autoimmune encephalitis (AIE), myasthenia gravis (MG), anti-myelin oligodendrocyte glycoprotein associated disease (MOGAD), and paraneoplastic neurological syndromes (PNS). DFPP significantly reduced immunoglobulin levels (IgG, IgA, IgM) by ∼70 %. Most patients showed decreased antibody titers and significant neurological improvement. The median mRS score improved from 2 (IQR 2-3) to 1 (IQR 1-2) post-treatment, with further improvement at 90 days. Notable improvements were observed across various scales specific to NMOSD, MOGAD, AIE, and MG. Minor adverse events were reported, with no serious adverse events.

CONCLUSIONS

DFPP is effective in reducing immunoglobulin and antibody levels, leading to improved neurological function in neuroimmune disorders. Further large-scale studies are warranted to confirm these findings.

摘要

目的

本研究旨在评估双重滤过血浆置换(DFPP)在降低神经免疫性疾病中免疫球蛋白和致病抗体方面的有效性。

方法

对 51 例接受 DFPP、免疫治疗和对症治疗的神经免疫性疾病患者进行回顾性分析。在治疗前后测量免疫球蛋白和抗体水平,并使用改良 Rankin 量表(mRS)、扩展残疾状态量表(EDSS)、自身免疫性脑炎临床评估量表(CASE)和重症肌无力专用量表等对神经功能进行评估。

结果

该队列包括视神经脊髓炎谱系疾病(NMOSD)、自身免疫性脑炎(AIE)、重症肌无力(MG)、抗髓鞘少突胶质细胞糖蛋白相关疾病(MOGAD)和副肿瘤性神经系统综合征(PNS)患者。DFPP 可显著降低免疫球蛋白水平(IgG、IgA、IgM)约 70%。大多数患者的抗体滴度降低,神经功能显著改善。治疗后 mRS 评分中位数从 2(IQR 2-3)改善至 1(IQR 1-2),90 天后进一步改善。NMOSD、MOGAD、AIE 和 MG 的专用量表均观察到显著改善。报告了轻微不良事件,但无严重不良事件。

结论

DFPP 可有效降低免疫球蛋白和抗体水平,改善神经免疫性疾病的神经功能。需要进一步的大规模研究来证实这些发现。

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