水通道蛋白4和视神经脊髓炎

视神经脊髓炎(neuromyelitis optica,NMO)是一种主要累及视神经和脊髓的复发性中枢神经系统炎性脱髓鞘病变,其主要病理特征为星形胶质细胞足板水通道蛋白-4(aquaporin-4,AQP4)缺失及激活补体在血管周围沉积。NMO免疫球蛋白G(NMO-immunoglobulin G,NMO-Ig G)是NMO的特异性血清学标记,NMO-Ig G与星形胶质细胞足板AQP4结合后介导的细胞毒性损伤是NMO的主要发病机制。NMO可以是一种独立疾病,也可以与结缔组织病并存。由于脊髓炎或视神经炎会导致患者永久性残疾或视力丧失,风湿科医师应采用必要的检查手段对容易复发或转变为NMO风险较大的患者及早做出诊断,对表现为脊髓炎或复发性视神经炎的结缔组织病患者应常规进行血清NMO-Ig G的检测。

Aquaporin-4 and Neuromyelitis Optica

Neuromyelitis optica （NMO） is a recurrent inflammatory demyelinating disorder of the central nervous system that mainly targets spinal cord and optic nerve,and is characterized by loss of aquaporin （AQP） 4 and perivascular deposition of activated complement.NMO-IgG is a specific serological marker of NMO,the combination of NMO-IgG with AQP-4 that mediated damage is the main pathogenesis of NMO.NMO may be an independent disease,but it can also be associated with connective tissue diseases.For the sake of potential permanent disability and sight loss following isolated attacks of myelitis or optic neuritis,rheumatologist should make great effort to identify individuals at risk for disease relapse or who are at risk for progression to full-spectrum NMO.We propose that AQP4-IgG testing should always be performed in patients with connective tissue diseases who present with myelitis or recurrent optic neuritis.