Abboud Hesham, Probasco John, Irani Sarosh R, Ances Beau, Benavides David R, Bradshaw Michael, Christo Paulo Pereira, Dale Russell C, Fernandez-Fournier Mireya, Flanagan Eoin P, Gadoth Avi, George Pravin, Grebenciucova Elena, Jammoul Adham, Lee Soon-Tae, Li Yuebing, Matiello Marcelo, Morse Anne Marie, Rae-Grant Alexander, Rojas Galeno, Rossman Ian, Schmitt Sarah, Venkatesan Arun, Vernino Steven, Pittock Sean J, Titulaer Maarten
Neurology, Case Western Reserve University, Cleveland, Ohio, USA
Multiple Sclerosis and Neuroimmunology Program, University Hospitals of Cleveland, Cleveland, Ohio, USA.
J Neurol Neurosurg Psychiatry. 2021 Mar 1;92(8):897-907. doi: 10.1136/jnnp-2020-325302.
The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. The most popular bridging therapy was oral prednisone taper chosen by 38% of responders while rituximab was the most popular maintenance therapy chosen by 46%. Most responders considered maintenance immunosuppression after a second relapse in patients with neuronal surface antibodies (70%) or seronegative autoimmune encephalitis (61%) as opposed to those with onconeuronal antibodies (29%). Most responders opted to cancer screening for 4 years in patients with neuronal surface antibodies (49%) or limbic encephalitis (46%) as opposed to non-limbic seronegative autoimmune encephalitis (36%). Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.
本文的目的是评估自身免疫性脑炎管理各步骤的现有证据,并在缺乏证据时提供专家意见。本文将自身免疫性脑炎视为一个广泛的类别,而不是专注于个别抗体综合征。自身免疫性脑炎联盟临床医生网络的核心作者查阅了文献并起草了初稿。在证据缺乏或存在争议的情况下,向所有成员进行了电子调查以征求个人意见。来自17个国家的68名成员回答了调查。最受欢迎的桥接治疗是口服泼尼松逐渐减量,38%的受访者选择了这种方法,而利妥昔单抗是最受欢迎的维持治疗,46%的受访者选择了它。大多数受访者认为,与伴有肿瘤神经元抗体的患者(29%)相比,神经元表面抗体阳性患者(70%)或血清阴性自身免疫性脑炎患者(61%)在第二次复发后应进行维持性免疫抑制治疗。大多数受访者选择对神经元表面抗体阳性患者(49%)或边缘性脑炎患者(46%)进行4年的癌症筛查,而非边缘性血清阴性自身免疫性脑炎患者的这一比例为36%。详细的调查结果在稿件中呈现,诊断和治疗建议总结在结论部分。