Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjogren's syndrome
Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjogren's syndrome
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伴有和不伴有干燥综合征的视神经脊髓炎患者的表现和结果的比较
DOI：
10.1007/s10072-016-2751-2
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发表时间：
2017
影响因子：
3.3
通讯作者：
Zhang Wei Xi
中科院分区：
文献类型：
--
作者：
Zhong Yu Hua;Zhong Zhi Gang;Zhou Zhou;Ma Zhen Yu;Qiu Meng Yao;Peng Fu Hua;Zhang Wei Xi
视神经脊髓炎(NMO)患者通常伴有自身免疫性疾病，最常见但不限于干燥综合征(SS)。本研究的目的是比较有无SS的NMO患者的临床和实验室特征，并探讨有无SS的NMO患者的预后。有SS的NMO患者23例，无SS的NMO患者42例。比较临床和实验室资料，包括NMO的年复发率和从发病到扩展残疾状态量表(EDSS)评分4.0和6.0的时间。伴SS者抗核抗体、抗SS-A/Ro抗体和抗SS-B/La抗体阳性率分别为91.3%和35.7%(P<0.001)，87.0%和2.3%(P<0.001)，34.8%和0.0%(P<0.001)。伴有SS的NMO患者血清免疫球蛋白(IgA、IgM和Ig G)明显高于无SS的患者。两组患者的年复发率和发病至EDSS评分分别为4.0和6.0的时间无显著差异。AQP-4抗体状态、脊髓病变长度、脑病变长度等指标两组间差异无统计学意义。这些结果表明，SS中的NMO更可能代表了SS的共存，而不是SS直接参与中枢神经系统的结果。NMO伴SS组的自身免疫反应较无SS组更为强烈，但并未导致更严重的预后，提示我们应重视NMO中抗核抗体的潜在益处。
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.