Ma Xiaoyu, Kermode Allan G, Hu Xueqiang, Qiu Wei
Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou, 510630, China.
Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou, 510630, China; Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Perth, WA, Australia; Department of Neurology, Sir Charles Gairdner Hospital, Queen Elizabeth II Medical Centre, Perth, WA, Australia; Institute of Immunology and Infectious Diseases, Murdoch University, Perth, WA, Australia.
Mult Scler Relat Disord. 2020 Nov;46:102522. doi: 10.1016/j.msard.2020.102522. Epub 2020 Sep 19.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disorder of the central nervous system (CNS) that is mainly associated with serum autoantibodies against aquaporin-4 (AQP4) in astrocytes. The relapsing clinical course of NMOSD, which can be blinding and disabling due to severe visual impairment, spinal cord lesions and a group of brain syndromes, suggests the importance of accurately evaluating the likelihood and severity of relapse at an early stage of the disease. To date, many risk factors have been revealed in association with relapse, and only some of them are supported by substantial evidence. Furthermore, while the clinical use of conventional immunosuppressants is mostly empirical, an increasing number of emerging therapies for monoclonal antibodies have been confirmed by several randomized placebo-controlled trials to be effective and safe for relapse prevention. In this review, we summarize the reported risk factors that may influence the frequency, symptoms, severity and prognosis of relapse in NMOSD, as well as the efficacy and safety of emerging therapies for relapse prevention. All of these results enable us to better recognize patients who are at higher risk of relapse and suggest more effective monoclonal antibody therapies for use in these patients.
视神经脊髓炎谱系障碍（NMOSD）是一种罕见的中枢神经系统（CNS）自身免疫性炎性疾病，主要与针对星形胶质细胞中水通道蛋白4（AQP4）的血清自身抗体有关。NMOSD的复发临床过程可能因严重视力损害、脊髓病变和一组脑综合征而致盲和致残，这表明在疾病早期准确评估复发的可能性和严重程度很重要。迄今为止，已发现许多与复发相关的危险因素，其中只有一些有充分证据支持。此外，虽然传统免疫抑制剂的临床应用大多是经验性的，但越来越多的单克隆抗体新兴疗法已被多项随机安慰剂对照试验证实对预防复发有效且安全。在本综述中，我们总结了已报道的可能影响NMOSD复发频率、症状、严重程度和预后的危险因素，以及预防复发新兴疗法的疗效和安全性。所有这些结果使我们能够更好地识别复发风险较高的患者，并为这些患者推荐更有效的单克隆抗体疗法。
