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蛋白 A 免疫吸附治疗难治性抗 N-甲基-D-天冬氨酸受体脑炎:一项单中心前瞻性研究。

Protein A immunoadsorption for the treatment of refractory anti-N-methyl-d-aspartate receptor encephalitis: A single-center prospective study.

机构信息

Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.

Guangdong Provincial Key Laboratory of Hemoadsorption Technology, Guangzhou, China.

出版信息

J Neurol Sci. 2021 Sep 15;428:117568. doi: 10.1016/j.jns.2021.117568. Epub 2021 Jul 6.

Abstract

OBJECTIVE

The aim of this study was to evaluate the efficacy and safety of protein A immunoadsorption (IA) for anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis resistant to intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG).

METHODS

We prospectively evaluated patients with refractory anti-NMDAR encephalitis, treated with protein A IA. Demographic data, clinical characteristics, modified Rankin Score (mRS), and anti-NMDAR antibodies were documented before and after IA and at follow-up. Clinical improvement was defined as a decrease of mRS ≥1. Adverse events were recorded throughout the study.

RESULTS

Ten patients with mRS ≥3 were enrolled and treated with protein A IA; treatment was performed for an average of 5.2 times per patient. Among the nine patients with positive serum anti-NMDAR, the titer decreased in seven patients, of which two became negative. The cerebrospinal fluid (CSF) anti-NMDAR titer decreased in all patients, and one became negative. Anti-NMDAR levels were tested in two patients at follow-up and found to have declined continuously. All patients exhibited clinical improvement with a mRS decline ≥1 after IA treatment (median mRS: 5.0 [range, 3.0-5.0] vs. 4.0 [range, 2.0-4.0], p = 0.014), and the median mRS decreased to 1.0 (range, 0-3.0) at follow-up. After IA, all patients exhibited accelerated recovery. No adverse events were observed during IA treatment.

CONCLUSION

Protein A IA may be effective for treating IVMP/IVIG-resistant anti-NMDAR encephalitis and well tolerated. It is necessary to initiate larger-scale prospective controlled studies to validate the efficacy and safety of protein A IA in anti-NMDAR encephalitis.

摘要

目的

本研究旨在评估蛋白 A 免疫吸附(IA)治疗静脉注射甲基强的松龙(IVMP)和静脉注射免疫球蛋白(IVIG)耐药性抗 N-甲基-D-天冬氨酸受体(NMDAR)脑炎的疗效和安全性。

方法

我们前瞻性评估了接受蛋白 A IA 治疗的难治性抗 NMDAR 脑炎患者。记录了患者的人口统计学数据、临床特征、改良 Rankin 量表(mRS)评分、抗 NMDAR 抗体水平,分别在 IA 治疗前后及随访时进行。临床改善定义为 mRS 评分至少降低 1 分。整个研究过程中记录不良反应。

结果

共纳入 10 例 mRS 评分≥3 的患者,平均每位患者接受蛋白 A IA 治疗 5.2 次。在 9 例血清抗 NMDAR 阳性的患者中,7 例患者滴度下降,其中 2 例转为阴性。所有患者的脑脊液(CSF)抗 NMDAR 滴度均下降,1 例转为阴性。在 2 例随访患者中检测到抗 NMDAR 水平持续下降。所有患者在 IA 治疗后均出现临床改善,mRS 评分降低≥1(中位数 mRS:5.0 [范围,3.0-5.0] 与 4.0 [范围,2.0-4.0],p=0.014),随访时中位数 mRS 降至 1.0(范围,0-3.0)。IA 治疗后,所有患者均加速康复。IA 治疗期间未观察到不良反应。

结论

蛋白 A IA 可能对治疗 IVMP/IVIG 耐药性抗 NMDAR 脑炎有效,且耐受性良好。有必要开展更大规模的前瞻性对照研究,以验证蛋白 A IA 在抗 NMDAR 脑炎中的疗效和安全性。

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