摘要
目的分析干燥综合征(SS)合并视神经脊髓炎谱系疾病(NMOSD)患者的临床特征、治疗及预后情况。方法检索中国学术期刊数据库(万方)、中国期刊全文数据库(中国知网)、中文科技期刊数据库(维普)、PubMed数据库,检索时间为2011年至2021年,以“干燥综合征、视神经脊髓炎谱系疾病”“干燥综合征神经系统病变”“Sjogren syndrome”“NMOSD”为关键词,分析SS合并NMOSD患者的临床表现、辅助检查及治疗预后情况。结果SS合并NMOSD女性占比较高(94.44%);临床表现常见为视力下降(61.11%),乏力(59.49%),麻木(39.24%),大小便障碍(21.51%),恶心、呕吐(16.45%);实验室检查常存在中枢神经系统水通道蛋白4(AQP4)抗体阳性(87.34%)、抗SSA抗体阳性(93.75%),脑脊液蛋白升高(50.67%);受累部位:以视神经(49.44%)及脊髓(62.01%)病变常见,还可累及脑干、大脑、脑白质。激素及免疫抑制剂治疗有效,但部分患者可能残留视力下降、乏力等症状,复发率为49.69%。结论SS合并NMOSD视力下降及麻木乏力症状多见,常出现视神经及脊髓病变,AQP4抗体对诊断意义大,早期激素和免疫抑制剂治疗可能减少复发,改善预后。
Objective To analyze the clinical features,treatment and prognosis of patients with Sjögren's syndrome(SS)with optic neuromyeli-tis spectrum disease(NMOSD).Methods China Science Periodical Database(Wanfang),China National Knowledge Infrastructure(CNKI),Chi-nese Science and Technology Journal Database(VIP)and PubMed database were searched from 2011 to 2021,using"Sjögren's syndrome and optic neuromyelitis spectrum disease""Sjögren's syndrome neuropathy""NMOSD"as keywords,the clinical manifestations,auxiliary examination and treatment prognosis of SS patients with NMOSD were analyzed.Results The proportion of female SS with NMOSD was higher(94.44%),the com-mon clinical manifestations were vision loss(61.11%),fatigue(59.49%),numbness(39.24%),defecation disorder(21.51%),nausea and vomiting(16.45%).In laboratory tests,there were always positive aquaporin 4(AQP4)antibody(87.34%)and anti-SSA antibody(93.75%)in the central nervous system,and increased CSF protein(50.67%).Affected sites:optic nerve(49.44%)and spinal cord(62.01%)were common lesions,and brain-stem,brain and white matter were also involved.Hormone and immunosuppressive therapy were effective,but patients may have residual symptoms such as vision loss and weakness,and the relapse rate was 49.69%.Conclusion SS combined with NMOSD has common symptoms of vision loss,numbness and fatigue,and optic nerve and spinal cord lesions are common,AQP4 antibody is of great significance in diagnosis,and early hormone and immunosuppressive therapy may reduce recurrence and improve prognosis.
作者
赵臻怡
文钟
林晓静
ZHAO Zhenyi;WEN Zhong;LIN Xiaojing(Department of Rheumatology,Affiliated Hospital of North Sichuan Medical College,Nanchong,Sichuan,637000,China;Department of Hematology,Affiliated Hospital of North Sichuan Medical College,Nanchong,Sichuan,637000,China)
出处
《当代医学》
2024年第6期121-124,共4页
Contemporary Medicine
