系统性红斑狼疮合并脊髓病变10例临床分析及文献回顾

Myelopathy associated with systemic lupus erythematosus： a clinical analysis of 10 cases and reviewof literature

目的探讨系统性红斑狼疮（SLE）合并脊髓病变的临床特点、治疗方法及预后。方法回顾性分析北京大学人民医院风湿免疫科1990-2011年住院的10例合并脊髓病变的SLE患者的临床资料，并进行文献复习。结果10例SLE合并脊髓病变的患者均为女性，年龄23-53岁，病程1-18年。3例患者行脊髓MRI检查，其中1例表现为胸8、胸9椎体内有小类圆形长T2信号影，2例表现为正常信号。10例患者中7例接受甲泼尼龙冲击联合免疫抑制剂治疗，2例单用甲泼尼龙冲击治疗，1例接受甲泼尼龙、环磷酰胺及血浆置换治疗，4例完全缓解，4例部分缓解，2例无明显缓解。结论脊髓病变是SLE较少见的严重并发症之一，多于SLE早期发病，发病年龄小，预后差。糖皮质激素冲击联合环磷酰胺治疗有效，早期积极干预有助改善预后。

Objective To analyze the clinical features, therapy and outcome of systemic lupus erythematosus （SLE） combined with lupus myelopathy （LM）. Methods Ten SLE patients combined with LM treated in Department of Rheumatology and Immunology, People＇s Hospital from 1990 to 2011 were retrospectively analyzed and 43 cases of SLE combined with LM reported home and abroad were reviewed. Results All the ten patients were women with age of 23-53 （ 36. 9 ± 3.4 ） years old and duration of 1-18 years. MRI of spinal cord revealed long T2 signal in one case, and normal in two cases. Seven patients received methylprednisolone pulse plus eyclophosphamide （CTX） , two were given glueoeortieoid pulse only, and one was given moderate dosage of glueoeorticoid, CTX and plasma exchange （PE）. The results revealed that four patients received complete recovery, four received partial recovery, and two received no improvement. Conclusions LM is a rare but severe complication of SLE with poor prognosis, which usually occurs in early phase of young SLE patients. Pulse methylprednisolone and CTX may be effective. Early and active treatment may improve the outcome.