Zhong Yu Hua, Zhong Zhi Gang, Zhou Zhou, Ma Zhen Yu, Qiu Meng Yao, Peng Fu Hua, Zhang Wei Xi
Department of Neurology, The First Affiliated Hospital of Sun Yat-Sen University, No. 58, Zhongshan Road II, Guangzhou, 510080, Guangdong Province, People's Republic of China.
Department of Neurology, The Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, People's Republic of China.
Neurol Sci. 2017 Feb;38(2):271-277. doi: 10.1007/s10072-016-2751-2. Epub 2016 Nov 5.
Patients with neuromyelitis optica (NMO) often have an accompanying autoimmune disease, most commonly, but not limited to Sjögren's syndrome (SS). The aim of this study was to compare clinical and laboratory features between NMO patients with and without SS and to investigate the prognosis of NMO in patients with and without SS. Twenty-three NMO patients with SS and 42 NMO patients without SS were included. Clinical and laboratory profiles were compared, including annual relapse rate and time from onset of NMO to Expanded Disability Status Scale (EDSS) scores of 4.0 and 6.0. More NMO patients with SS than those without SS had anti-nuclear antibody, anti-SS-A/Ro and anti-SS-B/La antibodies (91.3 vs. 35.7%, p < 0.001, 87.0 vs. 2.3%, p < 0.001, and 34.8 vs. 0.0%, p < 0.001, respectively). Serum immunoglobulins (IgA, IgM and IgG) were markedly increased in NMO patients with SS in comparison with those without SS. Annual relapse rate and the time from disease onset to an EDSS score of 4.0 and 6.0 were not significantly different between the two groups. No differences between the two groups were found for the other parameters, including AQP-4 antibody status, length of spinal cord lesion and brain lesions. These results imply that NMO in SS more likely represents coexistence with SS rather than representing the result of direct central nervous system involvement in SS. Autoimmune response appears to be more intense in the NMO group with SS, but did not cause a more severe prognosis in comparison with the group without SS, indicating that we should pay attention to the potential benefit of the antinuclear antibodies in NMO.
视神经脊髓炎（NMO）患者常伴有自身免疫性疾病，最常见但不限于干燥综合征（SS）。本研究旨在比较合并和未合并SS的NMO患者的临床和实验室特征，并调查合并和未合并SS的NMO患者的预后。纳入23例合并SS的NMO患者和42例未合并SS的NMO患者。比较了临床和实验室指标，包括年复发率以及从NMO发病到扩展残疾状态量表（EDSS）评分为4.0和6.0的时间。合并SS的NMO患者中抗核抗体、抗SS-A/Ro抗体和抗SS-B/La抗体阳性的比例高于未合并SS的患者（分别为91.3%对35.7%，p<0.001；87.0%对2.3%，p<0.001；34.8%对0.0%，p<0.001）。与未合并SS的NMO患者相比，合并SS的NMO患者血清免疫球蛋白（IgA、IgM和IgG）明显升高。两组的年复发率以及从疾病发作到EDSS评分为4.0和6.0的时间无显著差异。两组在其他参数方面未发现差异，包括水通道蛋白4抗体状态、脊髓病变长度和脑部病变。这些结果表明，SS中的NMO更可能是与SS共存，而不是SS直接累及中枢神经系统的结果。合并SS的NMO组自身免疫反应似乎更强，但与未合并SS的组相比，并未导致更严重的预后，这表明我们应关注抗核抗体在NMO中的潜在益处。
