Clinical characteristics of patients with neuromyelitis optica spectrum disorders with other autoantibodies

doi:10.3969/j.issn.1672-6731.2020.09.007

Abstract

Abstract:

Objective To analyze the clinical characteristics of patients with neuromyelitis optica spectrum disorders (NMOSDs) with or without autoantibodies other than aquaporin 4 (AQP4)-IgG. Methods A total of 90 NMOSDs patients were included from September 2014 to June 2017. First symptoms, first diagnosis, monophasic or polyphasic course of disease, comorbidities, Expanded Disability Status Scale (EDSS) score, treatment regimen and annualized relapse rate (ARR) were recorded. Laboratory tests for cerebrospinal fluid (CSF) white blood cell count and protein quantification, as well as serum autoimmune-related antibodies, including anti-nuclear antibody (ANA), A type and B type Sjögren's syndrome antibody (SSA and SSB), thyroid peroxidase (TPO) and thyroglobulin (TG) antibodies, anti-double stranded DNA antibody (dsDNA), perinuclear antineutrophil cytoplasmic antibody (pANCA), anti-endothelial cell antibody (AECA), Ro-52 antibody, Jo-1 antibody, anti-cardiolipin antibody (ACA), anti-mitochondria antibody M2 subtype (AMA-M2), human leukocyte antigen-B27 (HLA-B27), and myelin oligodendrocyte glycoprotein (MOG) antibody were examined. Brain and whole spine MRI were performed to observe the lesion site, distribution and length of spinal cord lesions. Results Among the 90 patients, 34 (37.78%) were accompanied by autoimmune-related antibodies (positive group), including 19 (55.88%) ANA positive cases, 13 (38.24%) SSA positive cases, 12 (35.29%) TPO antibody positive cases, and 10 (29.41%) TG antibody positive cases. The remaining 56 patients (62.22%) were not accompanied by autoimmune-related antibodies (negative group). In both groups, optic neuritis[52.94% (18/34) vs. 58.93% (33/56)], brain symptoms[26.47% (9/34) vs. 17.86% (10/56)] and myelitis[14.71% (5/34) vs. 21.43% (12/56)] were the main initial symptoms (Fisher exact probability:P=0.504). There were no significant differences in the first diagnosis (χ²=1.634, P=0.201), monophasic or polyphasic course (Fisher exact probability:P=1.000), comorbidities (χ²=1.275, P=0.302), EDSS score at the peak of the first onset (Z=-0.747, P=0.455) and at the inclusion (Z=-0.379, P=0.705), treatment plan (χ²=0.662, P=0.416) and ARR (Z=-0.370, P=0.711). There was no statistically significant difference in CSF white blood cell count (Z=-1.163, P=0.245) and protein quantification (Z=-0.340, P=0.734). MRI showed that 68 cases (75.56%) had intracranial lesions[28 cases (82.35%) in the positive group and 40 cases (71.43%) in the negative group], mainly located in the parietal lobe, frontal lobe and lateral ventricle. There were 62 cases (68.89%) with spinal lesions[21 cases (61.76%) in the positive group and 41 cases (73.21%) in the negative group], which were mainly cervical and thoracic myeloid lesions and medium-length segment lesion. There were no statistically significant differences in intracranial lesions (χ²=1.367, P=0.242), spinal cord lesions (χ²=1.294, P=0.255) or lesion length (Z=-0.647, P=0.517) between 2 groups. Conclusions There were no significant differences between NMOSDs patients with and without other autoimmune-related antibodies in terms of initial symptoms, CSF white blood cell count and protein quantification, MRI lesion distribution characteristics, etc. The value of autoimmune-related antibodies in clinical characteristics of NMOSDs patients remains to be further explored.

Key words: Neuromyelities optica spectrum disorders (not in MeSH), Autoantibodies, Serology, Cerebrospinal fluid, Magnetic resonance imaging

摘要：

目的对比分析伴与不伴其他自身免疫相关抗体阳性[排除水通道蛋白4（AQP4）-IgG]视神经脊髓炎谱系疾病（NMOSDs）的临床特点。方法共90例2014年9月至2017年6月确诊的NMOSDs患者符合入组条件，记录首发症状、首次诊断、单相或多相病程、共病情况、首次发病时峰值和入组时扩展残疾状态量表（EDSS）评分、治疗方案、年复发率（ARR）；实验室测定脑脊液白细胞计数和蛋白定量，以及多种血清自身免疫相关抗体；头部和全脊椎MRI观察病灶部位、分布范围和脊髓病灶长度。结果 90例患者中34例（37.78%）伴血清自身免疫相关抗体阳性（阳性组），并且以抗核抗体（19例占55.88%）、干燥综合征A型抗体（13例占38.24%）、甲状腺过氧化物酶抗体（12例占35.29%）和甲状腺球蛋白抗体（10例占29.41%）阳性为主；其余56例（62.22%）不伴自身免疫相关抗体阳性（阴性组）。两组均以视神经炎[52.94%（18/34）对58.93%（33/56）]、脑部症状[26.47%（9/34）对17.86%（10/56）]和脊髓炎[14.71%（5/34）对21.43%（12/56）]为主要首发症状（Fisher确切概率法：P=0.504），首次诊断（χ²=1.634，P=0.201）、单相或多相病程（Fisher确切概率法：P=1.000）、共病情况（χ²=1.275，P=0.302）、首次发病时峰值（Z=-0.747，P=0.455）和入组时（Z=-0.379，P=0.705）EDSS评分，以及治疗方案（χ²=0.662，P=0.416）和年复发率（Z=-0.370，P=0.711）差异均无统计学意义；脑脊液白细胞计数（Z=-1.163，P=0.245）和蛋白定量（Z=-0.340，P=0.734）差异亦无统计学意义。MRI显示68例（75.56%）有颅内病灶[阳性组28例（82.35%）、阴性组40例（71.43%）]，主要位于顶叶、额叶和侧脑室；62例（68.89%）存在脊髓病灶[阳性组21例（61.76%）、阴性组41例（73.21%）]，均以颈髓和胸髓病灶以及中节段病灶为主；两组颅内病灶分布范围（χ²=1.367，P=0.242），以及脊髓病灶分布范围（χ²=1.294，P=0.255）和病变长度（Z=-0.647，P=0.517）差异均无统计学意义。。结论伴与不伴其他自身免疫相关抗体阳性的NMOSDs患者在首发症状、脑脊液白细胞计数和蛋白定量、MRI病灶分布特征等方面无明显差异，自身免疫相关抗体在NMOSDs患者临床特征方面的价值尚待进一步探讨。

关键词: 视神经脊髓炎谱系疾病(非MeSH词), 自身抗体, 血清学, 脑脊髓液, 磁共振成像

LI Miao-chang, LI Rui, YANG Yu, LU Ting-ting, QIU Wei, WANG Yu-ge. Clinical characteristics of patients with neuromyelitis optica spectrum disorders with other autoantibodies[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2020, 20(9): 794-799.

李妙嫦, 李蕊, 杨渝, 卢婷婷, 邱伟, 王玉鸽. 伴其他自身免疫相关抗体的视神经脊髓炎谱系疾病临床特点分析[J]. 中国现代神经疾病杂志, 2020, 20(9): 794-799.

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