Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA.
Curr Opin Rheumatol. 2019 May;31(3):250-255. doi: 10.1097/BOR.0000000000000603.
This review discusses concepts for diagnosing neuromyelitis optica spectrum disorders (NMOSD), distinguishing NMOSD from other inflammatory diseases of the central nervous system, and highlights recent and forthcoming data on acute and maintenance therapy of NMOSD.
The neurologic manifestations of NMOSD are heterogenous, extending beyond classic presentations of optic neuritis and longitudinally extensive transverse myelitis. NMOSD may be comorbid with rheumatologic diseases, such as systemic lupus erythematosus, but is recognized as a distinct entity. Recent studies of acute treatment of NMOSD support early use of plasmapheresis. Relapse prevention is essential, as relapses can be disabling and patients may have only partial recovery. Current practice generally recommends at least 5 years of maintenance treatment. Recent randomized data demonstrates superiority of rituximab over azathioprine. Phase 3 trials have recently been completed or are underway studying novel therapies employing B-cell depletion, complement inhibition, and cell-based mechanisms (among other mechanisms) for maintenance therapy of NMOSD.
NMOSD is a heterogeneous but well-defined clinical entity, distinct from other neurologic and systemic inflammatory diseases, and treatment is poised for expansion.
本篇综述讨论了视神经脊髓炎谱系疾病（NMOSD）的诊断概念，NMOSD 与中枢神经系统其他炎症性疾病的鉴别，以及 NMOSD 的急性和维持治疗的最新和即将出现的数据。
NMOSD 的神经表现具有异质性，不仅限于经典的视神经炎和长节段横贯性脊髓炎。NMOSD 可能与系统性红斑狼疮等风湿性疾病同时存在，但被认为是一种独特的实体。NMOSD 急性治疗的最新研究支持早期使用血浆置换。预防复发至关重要，因为复发可能导致残疾，且患者可能只有部分恢复。目前的实践通常建议至少进行 5 年的维持治疗。最近的随机数据表明，利妥昔单抗优于硫唑嘌呤。最近已经完成或正在进行 3 期试验，研究用于 NMOSD 维持治疗的新型疗法，这些疗法采用 B 细胞耗竭、补体抑制和基于细胞的机制（以及其他机制）。
NMOSD 是一种具有异质性但明确的临床实体，与其他神经和系统性炎症性疾病不同，治疗方法正在扩展。
