摘要
目的总结原发性干燥综合征（pSS）合并视神经脊髓炎（NMO）疾病谱（NMOSD）的临床表现、影像学、脑脊液和自身抗体特点。方法回顾性分析11例诊断明确、资料完整的pSS合并NMOSD的临床资料。结果pSS合并NMOSD患者11例，女性10例，男性1例，年龄20～65（354-15）岁；中位病程10个月（1个月一16年）。以神经系统受累为首发表现者8例；视神经和脊髓受累间隔时间2个月～3年；8例视神经损害。磁共振成像（MRI）：颈髓为最常受累的脊髓节段10例，颈胸髓同时受累6例。抗核抗体（ANA）阳性10例，抗SSA抗体阳性11例，抗SSB抗体阳性5例；7例患者行血清水通道蛋白4（AQP-4）抗体检测，6例阳性，1例阴性。结论NMO是pSS中枢神经系统受累的一种表现形式，可以作为其首发症状，对有视神经、纵向广泛脊髓受累的患者应进行自身抗体、NMO．IgG／AQP-4抗体及影像学检查，以进行合理诊治，减少误诊、漏诊。
Objective To investigate the clinical features of neuromyelitis optica （NMO） and NMO spectrum disorders （ NMOSD ） with primary Sjogren＇ s syndrome （ pSS ） . Methods Eleven inpatients diagnosed as NMO secondary to pSS in Peking Union Medical College Hospital were retrospectively analyzed. Results Eleven patients of pSS with NMO were enrolled in this study, including 10 females and 1 male. The mean age was（ 35 ± 15 ）years old. The course of disease ranged from one month to 16 years, with a median of 10 months. The interval between optic nerve and spinal cord involvement was 3 months to 2 years. Eight patients had NMOSD as initial presentations of pSS. Eight patients had history of optic neuropathy. The most frequently involved spinal segment was cervical cord （10 cases） shown by magnetic resonance. Serum NMO-IgG antibodies were tested in 7 patients and 6 of them were positive. Conclusion NMOSD may present as an important and initial clinical manifestation of pSS, which suggesting the involvement of central nervous system. Autoimmune antibodies, NMO-IgG and imaging were supposed to be done for further evaluation of prognosis and therapy regimens.
出处
《中华内科杂志》
CAS
CSCD
北大核心
2013年第9期745-748,共4页
Chinese Journal of Internal Medicine
关键词
干燥综合征
视神经脊髓炎
Sjogren＇s syndrome
Neuromyelitis optica
