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系统性红斑狼疮所致脊髓炎:5例患者的临床特征、免疫学特征及磁共振成像

Myelitis in systemic lupus erythematosus: clinical features, immunological profile and magnetic resonance imaging of five cases.

作者信息

Hryb Javier Pablo, Chiganer Edson, Contentti Edgar Carnero, Di Pace José Luis, Lessa Carmen, Perassolo Mónica Beatriz

机构信息

Department of Neurology, Hospital 'Carlos G Durand' , Buenos Aires, Argentina.

Department of Immunology and Histocompatibility, Hospital 'Carlos G Durand' , Buenos Aires, Argentina.

出版信息

Spinal Cord Ser Cases. 2016 Jul 7;2:16005. doi: 10.1038/scsandc.2016.5. eCollection 2016.

Abstract

Myelopathy is one of the neuropsychiatric lupus syndromes. In this article, an original series of related lupus myelitis is reported and analyzed. We employed a retrospective chart review and identified all patients who were admitted to a general hospital in Buenos Aires, Argentina, with SLE and myelitis during the period 2007-2014. Five patients were observed, all women. The mean age was 25.4 years (19-39). In three of five cases, myelitis was one of the initial SLE manifestations. The SLE Disease Activity Index was variable (3/5 with high activity). Time to nadir ranged from 6 to 72 h. All had severe impairment, with motor deficit, sensory level and urinary retention. Magnetic resonance imaging was abnormal in all cases, 3/5 presented a longitudinally extensive myelitis. Serum analysis revealed positive antinuclear antibodies at a high titer in all patients, 4/5 had low complement levels and 3/5 had anti-phospholipids positive. The treatment (methylprednisolone and, in some cases, cyclophosphamide, anticoagulation and/or plasmapheresis) produced partial improvement or no benefits. One patient died due to sepsis. The others showed significant disability at 6 months (European Database for Multiple Sclerosis grading scale=6-8). In view of these results, myelitis associated with lupus shows heterogeneity of the clinical, radiological and serological features. In our experience, the cases were severe and with poor response to treatment. Further studies are required to understand this disease and establish a more efficient treatment.

摘要

脊髓病是神经精神性狼疮综合征之一。在本文中,我们报告并分析了一系列原发性狼疮性脊髓炎病例。我们采用回顾性病历审查,确定了2007年至2014年期间在阿根廷布宜诺斯艾利斯一家综合医院收治的所有系统性红斑狼疮(SLE)合并脊髓炎患者。共观察到5例患者,均为女性。平均年龄为25.4岁(19 - 39岁)。5例中有3例脊髓炎是SLE的首发表现之一。SLE疾病活动指数各不相同(5例中有3例活动度高)。病情至最低点的时间为6至72小时。所有患者均有严重功能障碍,伴有运动功能缺损、感觉平面及尿潴留。所有病例磁共振成像均异常,5例中有3例表现为纵向广泛脊髓炎。血清分析显示所有患者抗核抗体高滴度阳性,5例中有4例补体水平低,5例中有3例抗磷脂抗体阳性。治疗(甲泼尼龙,部分病例使用环磷酰胺、抗凝治疗和/或血浆置换)仅产生部分改善或无效果。1例患者因败血症死亡。其他患者在6个月时显示出明显残疾(欧洲多发性硬化数据库分级量表评分为6 - 8级)。鉴于这些结果,狼疮相关脊髓炎在临床、影像学和血清学特征上表现出异质性。根据我们的经验,这些病例病情严重且治疗反应不佳。需要进一步研究以了解这种疾病并建立更有效的治疗方法。

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