BACKGROUND Plasma exchange (PE) has usually to be considered as a rescue therapy when intravenous corticosteroids is insufficient in acute attacks of neuromyelitis optica spectrum disorders (NMOSD). The efficacy of PE has not been quantified. This system review and meta-analysis was aimed to evaluate the efficacy of PE therapy in acute attacks of NMOSD. METHODS Studies evaluating the efficacy of PE in patients with NMOSD were identified from PubMed and Embase. Changes of Expanded Disability Status Scale (EDSS) score between before and after PE therapy, and the rate of response to PE, were defined as the main efficacy outcomes. Meta-regression was performed to identify the sources of heterogeneity. Subgroup meta-analysis were performed based on the interval of initiation PE after attack onset and AQP4-IgG serostatus of patients. RESULTS Twenty-four studies containing 528 patients with NMOSD were included in this meta-analysis. As a rescue therapy when patients failed to respond to intravenous corticosteroids (PE rescue), PE treatment resulted in a reduction in the mean EDSS score by 1.69 (95% CI: 0.88-2.50), with a response rate of 75%(95%CI: 66%-83%). As a first-line therapy being used alone or simultaneously with intravenous corticosteroids (PE first-line), PE resulted in a reduction in the mean EDSS score by 2.34 (95% CI: 1.69-2.98), with a response rate of 71%(95%CI: 44%-93%). Overall, PE resulted in a reduction in the mean EDSS score by 1.83 (95% CI: 1.19-2.47), with a response rate of 74% (95%CI: 66%-82%). Subgroup analysis suggested that earlier PE initiation and AQP4-IgG seronegative patients seemed to be associated with a superior response to PE therapy. CONCLUSION Plasma exchange, whether used as rescue or as first-line therapy, is an effective therapeutic method in patients during acute attacks of NMOSD.

中文翻译：

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血浆置换治疗视神经脊髓炎谱系疾病急性发作的疗效：系统评价和荟萃分析

背景 当视神经脊髓炎谱系疾病 (NMOSD) 急性发作时静脉内皮质类固醇不足时，通常必须考虑血浆置换 (PE) 作为挽救疗法。PE 的疗效尚未量化。本系统回顾和荟萃分析旨在评估 PE 治疗对 NMOSD 急性发作的疗效。方法 评估 PE 在 NMOSD 患者中的疗效的研究来自 PubMed 和 Embase。PE治疗前后扩展残疾状态量表（EDSS）评分的变化以及对PE的反应率被定义为主要疗效结果。进行元回归以确定异质性的来源。根据发作后开始 PE 的间隔和患者的 AQP4-IgG 血清状态进行亚组荟萃分析。结果 本荟萃分析纳入了 24 项研究，其中包含 528 名 NMOSD 患者。作为患者对静脉注射皮质类固醇治疗无效时的抢救疗法（PE 抢救），PE 治疗导致平均 EDSS 评分降低 1.69（95% CI：0.88-2.50），有效率为 75%（95%置信区间：66%-83%）。作为单独或与静脉注射皮质类固醇同时使用的一线疗法（PE 一线），PE 导致平均 EDSS 评分降低 2.34（95% CI：1.69-2.98），反应率为 71% (95% CI: 44%-93%)。总体而言，PE 导致平均 EDSS 评分降低 1.83（95% CI：1.19-2.47），响应率为 74%（95%CI：66%-82%）。亚组分析表明，早期 PE 开始和 AQP4-IgG 血清阴性患者似乎与对 PE 治疗的良好反应有关。