Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS).

Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS).
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DOI:
10.1007/s00415-013-7169-7
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发表时间:
2014-01
影响因子:
6
通讯作者:
Neuromyelitis Optica Study Group (NEMOS)
Neuromyelitis Optica Study Group (NEMOS)
中科院分区:
医学2区
文献类型:
--
作者:
Trebst C;Jarius S;Berthele A;Paul F;Schippling S;Wildemann B;Borisow N;Kleiter I;Aktas O;Kümpfel T;Neuromyelitis Optica Study Group (NEMOS)

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视神经脊髓炎(NMO,Devic综合征),长期以来被认为是多发性硬化症的临床变种,现在被视为一种独特的疾病实体。自从水通道蛋白-4抗体(AQP4-Ab;也称为NMO-IgG)于2004年首次被发现以来,在NMO的诊断和治疗方面取得了重大进展。在这篇综述中,视神经脊髓炎研究小组(NEMOS)总结了最近获得的关于NMO的知识,并根据当前的指南、出版的文献和NEMOS定期会议上的专家讨论,重点介绍了NMO诊断和治疗的新进展。对AQP4-Ab的检测是必要的,也是诊断可疑NMO的最重要的测试,有助于将NMO与其他自身免疫性疾病区分开来。此外,AQP4-Ab检测扩大了我们对NMO谱系障碍(NMOSD)的临床表现的了解。此外,成像技术,特别是大脑和脊髓的磁共振成像,在诊断工作中是必不可少的。值得注意的是,NMO和NMOSD的脑部病变并不少见,不排除诊断,并显示出特征性的模式。其他成像方式,如光学相干断层扫描,被认为是评估视网膜损伤的有用工具。NMO的治疗应及早开始。硫唑嘌呤和利妥昔单抗被建议作为一线治疗,后者越来越被认为是一种对NMO患者具有长期疗效和可接受的安全性的既定治疗方法。其他免疫抑制药物,如甲氨蝶呤、霉酚酸酯和米托蒽醌,被推荐为二线治疗。有希望的新疗法正在以抗IL6受体、抗补体或抗AQP4-Ab生物制剂的形式出现。
Neuromyelitis optica (NMO, Devic’s syndrome), long considered a clinical variant of multiple sclerosis, is now regarded as a distinct disease entity. Major progress has been made in the diagnosis and treatment of NMO since aquaporin-4 antibodies (AQP4-Ab; also termed NMO-IgG) were first described in 2004. In this review, the Neuromyelitis Optica Study Group (NEMOS) summarizes recently obtained knowledge on NMO and highlights new developments in its diagnosis and treatment, based on current guidelines, the published literature and expert discussion at regular NEMOS meetings. Testing of AQP4-Ab is essential and is the most important test in the diagnostic work-up of suspected NMO, and helps to distinguish NMO from other autoimmune diseases. Furthermore, AQP4-Ab testing has expanded our knowledge of the clinical presentation of NMO spectrum disorders (NMOSD). In addition, imaging techniques, particularly magnetic resonance imaging of the brain and spinal cord, are obligatory in the diagnostic workup. It is important to note that brain lesions in NMO and NMOSD are not uncommon, do not rule out the diagnosis, and show characteristic patterns. Other imaging modalities such as optical coherence tomography are proposed as useful tools in the assessment of retinal damage. Therapy of NMO should be initiated early. Azathioprine and rituximab are suggested as first-line treatments, the latter being increasingly regarded as an established therapy with long-term efficacy and an acceptable safety profile in NMO patients. Other immunosuppressive drugs, such as methotrexate, mycophenolate mofetil and mitoxantrone, are recommended as second-line treatments. Promising new therapies are emerging in the form of anti-IL6 receptor, anti-complement or anti-AQP4-Ab biologicals.
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