补体蛋白在区分 AQP4-IgG 阳性与 MOG-IgG 阳性视神经脊髓炎谱系疾病中的临床价值。
The clinical value of complement proteins in differentiating AQP4-IgG-positive from MOG-IgG-positive neuromyelitis optica spectrum disorders.
机构信息
Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China.
Department of Neurology, Mayo Clinic, Rochester, MN 55905, United States.
出版信息
Mult Scler Relat Disord. 2019 Oct;35:1-4. doi: 10.1016/j.msard.2019.06.035. Epub 2019 Jun 29.
BACKGROUND
Neuromyelitis optica spectrum disorder (NMOSD) refers to a range of autoimmune inflammatory demyelinating diseases affecting the optic nerves, spinal cord, and periependymal regions of the brain. Classical NMOSD is characterized by the presentation of autoantibodies against the water channel aquaporin-4 (AQP4). However, a subset of patients fulfilling the clinical criteria for NMOSD is negative for AQP4-IgG but positive for autoantibodies against myelin oligodendrocyte glycoprotein (MOG); these patients are associated with different clinical manifestations and pathogenesis.
METHODS
Patients who received a first diagnosis of NMOSD were reviewed retrospectively between April 2015 and December 2018. Patients were classified according to the presence of AQP4-IgG and MOG-IgG in serum and/or cerebrospinal fluid. Clinical characteristics, magnetic resonance imaging findings, disease severity, and serum C3 and C4 levels at the first episode were compared between the groups.
RESULTS
The NMOSD patients with AQP4-IgG and MOG-IgG demonstrated specific, differential clinical features. The AQP4-IgG group featured more women, the presentation of transverse myelitis attacks and simultaneous occurrence of optic neuritis and transverse myelitis were more common, and intrathecal synthesis was more evident. The MOG-IgG group featured younger patients, more acute disseminated encephalomyelitis (ADEM) or ADEM-like attacks, more frequent cerebrospinal fluid pleocytosis, and a better overall outcome. C3 levels were significantly lower in AQP4-IgG-positive patients and higher in MOG-IgG-positive patients relative to healthy controls. C4 levels were significantly lower in the AQP4-IgG-positive NMOSD group when compared to both MOG-IgG-positive patients and controls. C3 and C4 were then combined in a receiver operating characteristic model. The area under the curve of the model was calculated to differentiate the AQP4-IgG-positive group from the MOG-IgG-positive group was 0.787, which was considered moderately predictive.
CONCLUSION
The combination of C3 and C4 could assist in the differential diagnosis of AQP4-IgG-positive NMOSD from MOG-IgG-positive NMOSD.
背景
视神经脊髓炎谱系疾病（NMOSD）是指一系列影响视神经、脊髓和脑室周围区域的自身免疫性炎症性脱髓鞘疾病。经典 NMOSD 的特征是存在针对水通道蛋白 4（AQP4）的自身抗体。然而，一部分符合 NMOSD 临床标准的患者 AQP4-IgG 检测为阴性，但髓鞘少突胶质细胞糖蛋白（MOG）自身抗体检测为阳性；这些患者具有不同的临床表现和发病机制。
方法
回顾性分析 2015 年 4 月至 2018 年 12 月期间首次诊断为 NMOSD 的患者。根据血清和/或脑脊液中 AQP4-IgG 和 MOG-IgG 的存在情况对患者进行分类。比较两组患者的临床特征、磁共振成像表现、疾病严重程度以及首发时血清 C3 和 C4 水平。
结果
NMOSD 患者 AQP4-IgG 和 MOG-IgG 表现出特定的、不同的临床特征。AQP4-IgG 组女性患者更多，横惯性脊髓炎发作和同时发生视神经炎和横惯性脊髓炎更为常见，鞘内合成更为明显。MOG-IgG 组患者更年轻，急性播散性脑脊髓炎（ADEM）或 ADEM 样发作更常见，脑脊液白细胞增多更频繁，总体预后更好。与健康对照组相比，AQP4-IgG 阳性患者的 C3 水平显著降低，MOG-IgG 阳性患者的 C3 水平显著升高。与 MOG-IgG 阳性患者和对照组相比，AQP4-IgG 阳性 NMOSD 组的 C4 水平显著降低。然后将 C3 和 C4 结合在一个接收器工作特征模型中。计算模型区分 AQP4-IgG 阳性组和 MOG-IgG 阳性组的曲线下面积为 0.787，认为具有中等预测价值。
结论
C3 和 C4 的联合检测可辅助诊断 AQP4-IgG 阳性 NMOSD 与 MOG-IgG 阳性 NMOSD。
Zotero 插件