Department of Neurology and the Multiple Sclerosis Center, the Agnes-Ginges Center for Neurogenetics, Hadassah - Medical Center, Ein-Karem, Jerusalem, 91120, Israel.
Department of Neurology and the Multiple Sclerosis Center, the Agnes-Ginges Center for Neurogenetics, Hadassah - Medical Center, Ein-Karem, Jerusalem, 91120, Israel.
J Autoimmun. 2014 Nov;54:93-9. doi: 10.1016/j.jaut.2014.05.004. Epub 2014 Aug 14.
When studying a rare or orphan disease, we hope to shed light on more prevalent syndromes. Neuromyelitis optica (NMO), also known as Devic's disease, is a rare disease with a prevalence of about 4 in 100,000. Since 2005 when the anti-Aquaporin 4 (AQP4) NMO autoantibody was discovered by Lennon's group at the Mayo clinic, an enormous amount of data have been acquired on the pathogenesis of the disease. A review of the literature showed 47 relevant publications in 2004, compared with 353 in 2013. The auto-antigen AQP4 is expressed on the astrocytic foot processes suggesting a role for astrocytes in the pathogenesis of the disease. However, the astrocytes might play a more active role than has previously been suggested in the immune cascade of NMO pathology. Here we will review epidemiological, clinical diagnostic and therapeutic aspects of NMO and highlight the possible role of astrocytes as major direct and indirect players in the pathogenesis of NMO and related CNS inflammatory diseases.
当研究一种罕见或孤儿病时，我们希望能为更常见的综合征提供一些启示。视神经脊髓炎（NMO），也称为 Devic 病，是一种罕见疾病，发病率约为每 10 万人中有 4 人。自 2005 年 Mayo 诊所的 Lennon 小组发现抗水通道蛋白 4（AQP4）NMO 自身抗体以来，已经获得了大量关于该疾病发病机制的数据。文献回顾显示，2004 年有 47 篇相关出版物，而 2013 年则有 353 篇。自身抗原 AQP4 表达在星形胶质细胞足突上，提示星形胶质细胞在疾病发病机制中起作用。然而，星形胶质细胞在 NMO 病理的免疫级联反应中可能扮演着比以前所认为的更积极的角色。在这里，我们将回顾 NMO 的流行病学、临床诊断和治疗方面，并强调星形胶质细胞作为 NMO 和相关中枢神经系统炎症性疾病发病机制中的主要直接和间接参与者的可能作用。
