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免疫吸附或血浆置换治疗自身免疫性脑炎:一项初步研究。

Immunoadsorption or plasma exchange in the treatment of autoimmune encephalitis: a pilot study.

作者信息

Heine Josephine, Ly Lam-Thanh, Lieker Ina, Slowinski Torsten, Finke Carsten, Prüss Harald, Harms Lutz

机构信息

Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.

German Center for Neurodegenerative Diseases (DZNE) Berlin, Charitéplatz 1, 10117, Berlin, Germany.

出版信息

J Neurol. 2016 Dec;263(12):2395-2402. doi: 10.1007/s00415-016-8277-y. Epub 2016 Sep 7.

Abstract

Therapeutic apheresis has emerged as a major treatment option for autoantibody-associated inflammatory diseases of the nervous system. This includes patients with autoimmune encephalitides caused by antibodies against neuronal proteins. Plasma exchange (PE) and immunoadsorption (IA) constitute two possibilities to eliminate pathogenic antibodies from patients' plasma, but their efficacy and safety has not been prospectively assessed in larger patient groups of autoimmune encephalitides. In a prospective observational case control study, we, therefore, investigated the disease courses and treatment effects of 21 patients with autoimmune encephalitis associated with NMDAR, LGI1, CASPR2, GAD, mGluR5 and Hu antibodies. Patients were randomly assigned to receive PE (n = 11) or IA (n = 10). Symptoms were evaluated using the modified Rankin Scale (mRS). Side effects or adverse events were recorded. Both interventions, IA (p = 0.014) and PE (p = 0.01), resulted in significant reduction of the median mRS. With IA, 60 % of the patients improved clinically by at least 1 mRS score, none worsened. PE led to a comparable symptom reduction in 67 % of the cases. During 83 PE sessions, three adverse events were documented, while no side effects occurred under IA. Symptom improvement was significantly associated with younger age (r = -0.58), but not with disease duration. Therapeutic apheresis was most effective for neuronal surface antigens (83.3 %), followed by intracellular-synaptic antigens (66.7 %). Both IA and PE resulted in moderate to marked clinical improvement, with a low rate of adverse events. Apheresis is well tolerated and effective also as first-line therapy in autoimmune encephalitis, particularly in patients with antibodies targeting neuronal surfaces.

摘要

治疗性血液成分单采术已成为神经系统自身抗体相关炎性疾病的一种主要治疗选择。这包括由抗神经元蛋白抗体引起的自身免疫性脑炎患者。血浆置换(PE)和免疫吸附(IA)是从患者血浆中清除致病抗体的两种方法,但它们的疗效和安全性尚未在更大规模的自身免疫性脑炎患者群体中进行前瞻性评估。因此,在一项前瞻性观察性病例对照研究中,我们调查了21例与NMDAR、LGI1、CASPR2、GAD、mGluR5和Hu抗体相关的自身免疫性脑炎患者的病程和治疗效果。患者被随机分配接受PE(n = 11)或IA(n = 10)。使用改良Rankin量表(mRS)评估症状。记录副作用或不良事件。IA(p = 0.014)和PE(p = 0.01)这两种干预措施均导致mRS中位数显著降低。接受IA治疗的患者中,60%在临床上至少改善了1个mRS评分,无病情恶化。PE在67%的病例中导致了类似的症状减轻。在83次PE治疗过程中,记录到3例不良事件,而IA治疗未出现副作用。症状改善与年龄较轻显著相关(r = -0.58),但与病程无关。治疗性血液成分单采术对神经元表面抗原最为有效(83.3%),其次是细胞内突触抗原(66.7%)。IA和PE均导致中度至显著的临床改善,不良事件发生率较低。血液成分单采术耐受性良好,作为自身免疫性脑炎的一线治疗也有效,特别是对于靶向神经元表面抗体的患者。

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