Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Department of Neurology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Front Immunol. 2021 Jul 22;12:705536. doi: 10.3389/fimmu.2021.705536. eCollection 2021.
Neuromyelitis optica (NMO), multiple sclerosis (MS) and autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy are idiopathic inflammatory demyelinating diseases (IIDDs) that mainly present as encephalomyelitis. Heparan sulfate (HS) and hyaluronic acid (HA) are two components of glycocalyx, a carbohydrate-rich layer on the surface of blood vessels that mediates interaction with blood. Degradation of glycocalyx in NMO is poorly understood.
To detect the serum and cerebrospinal fluid (CSF) levels of shed HS and HA and to correlate these levels with disease severity to determine their diagnostic value.
We obtained serum and CSF samples from 24 NMO patients, 15 MS patients, 10 autoimmune GFAP astrocytopathy patients, and 18 controls without non-inflammatory neurological diseases. Soluble HS and HA, and IFNγ, IL17A, and matrix metalloproteinase (MMP) 1 were detected ELISA.
Serum and CSF levels of HS, HA and related cytokines but not of plasma MMP1 were significantly elevated in these diseases. Notably, HS and HA levels were positively correlated with Expanded Disability Status Scale scores.
Our results indicate glycocalyx degradation and inflammation in NMO, MS and autoimmune GFAP astrocytopathy. Moreover, increased shedding of HS or HA may indicate a worse clinical situation. Furthermore, therapeutic strategies that protect glycocalyx may be effective in these diseases.
视神经脊髓炎（NMO）、多发性硬化症（MS）和自身免疫性胶质纤维酸性蛋白（GFAP）星形胶质细胞病是特发性炎症性脱髓鞘疾病（IIDDs），主要表现为脑脊髓炎。硫酸乙酰肝素（HS）和透明质酸（HA）是糖萼的两个组成部分，糖萼是血管表面富含碳水化合物的层，介导与血液的相互作用。NMO 中糖萼的降解知之甚少。
检测血清和脑脊液（CSF）中脱落的 HS 和 HA 水平，并将这些水平与疾病严重程度相关联，以确定其诊断价值。
我们从 24 名 NMO 患者、15 名 MS 患者、10 名自身免疫性 GFAP 星形胶质细胞病患者和 18 名无非炎症性神经疾病的对照者中获得了血清和 CSF 样本。采用 ELISA 法检测可溶性 HS 和 HA 以及 IFNγ、IL17A 和基质金属蛋白酶（MMP）1。
这些疾病的血清和 CSF 中 HS、HA 和相关细胞因子水平升高，而血浆 MMP1 水平无明显升高。值得注意的是，HS 和 HA 水平与扩展残疾状况量表评分呈正相关。
我们的结果表明 NMO、MS 和自身免疫性 GFAP 星形胶质细胞病中存在糖萼降解和炎症。此外，HS 或 HA 的脱落增加可能表明临床情况更糟。此外，保护糖萼的治疗策略可能对这些疾病有效。
