Schneider-Hohendorf Tilman, Wünsch Christian, Falk Simon, Raposo Catarina, Rubelt Florian, Mirebrahim Hamid, Asgharian Hosseinali, Schlecht Ulrich, Mattox Daniel, Zhou Wenyu, Dawin Eva, Pawlitzki Marc, Lauks Sarah, Jarius Sven, Wildemann Brigitte, Havla Joachim, Kümpfel Tania, Schrot Miriam-Carolina, Ringelstein Marius, Kraemer Markus, Schwake Carolin, Schmitter Thomas, Ayzenberg Ilya, Fischer Katinka, Meuth Sven G, Aktas Orhan, Hümmert Martin W, Kretschmer Julian R, Trebst Corinna, Kleffner Ilka, Massey Jennifer, Muraro Paolo A, Chen-Harris Haiyin, Gross Catharina C, Klotz Luisa, Wiendl Heinz, Schwab Nicholas
Department of Neurology with Institute of Translational Neurology, University of Muenster, 48149 Muenster, Germany.
F. Hoffmann-La Roche Ltd, 4070 Basel, Switzerland.
Brain. 2025 Mar 6;148(3):933-940. doi: 10.1093/brain/awae244.
Epstein-Barr virus (EBV) infection has long been associated with the development of multiple sclerosis (MS). Patients with MS have elevated titres of EBV-specific antibodies in serum and show signs of CNS damage only after EBV infection. Regarding CD8+ T cells, an elevated but ineffective response to EBV was suggested in MS patients, who present with a broader MHC-I-restricted EBV-specific T-cell receptor beta chain (TRB) repertoire compared to controls. It is not known whether this altered EBV response could be subject to dynamic changes, e.g. by approved MS therapies, and whether it is specific for MS. Peripheral blood TRB repertoire samples (n = 1317) of healthy donors (n = 409), patients with MS (n = 710) before and after treatment, patients with neuromyelitis optica spectrum disorder (n = 87), MOG antibody-associated disease (MOGAD) (n = 64) and Susac's syndrome (n = 47) were analysed. Apart from MS, none of the evaluated diseases presented with a broader anti-EBV TRB repertoire. In MS patients undergoing autologous haematopoietic stem-cell transplantation, EBV reactivation coincided with elevated MHC-I-restricted EBV-specific TRB sequence matches. Therapy with ocrelizumab, teriflunomide or dimethyl fumarate reduced EBV-specific, but not CMV-specific MHC-I-restricted TRB sequence matches. Together, these data suggest that the aberrant MHC-I-restricted T-cell response directed against EBV is specific to MS with regard to neuromyelitis optica, MOGAD and Susac's syndrome and that it is specifically modified by MS treatments interfering with EBV host cells or activated lymphocytes.
长期以来,爱泼斯坦 - 巴尔病毒(EBV)感染一直与多发性硬化症(MS)的发生有关。MS患者血清中EBV特异性抗体滴度升高,且仅在EBV感染后才出现中枢神经系统损伤的迹象。关于CD8 + T细胞,MS患者对EBV的反应虽升高但无效,与对照组相比,MS患者呈现出更广泛的主要组织相容性复合体I类(MHC-I)限制性EBV特异性T细胞受体β链(TRB)库。目前尚不清楚这种改变的EBV反应是否会发生动态变化,例如通过已获批的MS疗法,以及它是否是MS所特有的。分析了健康供体(n = 409)、治疗前后的MS患者(n = 710)、视神经脊髓炎谱系障碍患者(n = 87)、MOG抗体相关疾病(MOGAD)患者(n = 64)和Susac综合征患者(n = 47)的外周血TRB库样本(n = 1317)。除MS外,所评估的其他疾病均未呈现出更广泛的抗EBV TRB库。在接受自体造血干细胞移植的MS患者中,EBV重新激活与MHC-I限制性EBV特异性TRB序列匹配升高同时出现。用奥瑞珠单抗、特立氟胺或富马酸二甲酯治疗可降低EBV特异性但不降低巨细胞病毒(CMV)特异性的MHC-I限制性TRB序列匹配。总之,这些数据表明,针对EBV的异常MHC-I限制性T细胞反应在视神经脊髓炎、MOGAD和Susac综合征方面是MS所特有的,并且它会被干扰EBV宿主细胞或活化淋巴细胞的MS治疗特异性改变。