国人系统性红斑狼疮合并纵向脊髓炎的临床分析(附1例病例报道并文献回顾)

Longitudinal myelitis with systemic lupus erythematosus in China (one case report and literature review)

目的报道系统性红斑狼疮（SLE）合并纵向脊髓炎（LM）1例,并通过文献复习对我国患者的临床特点进行分析总结。方法报道1例58岁女性患者,因肺部感染及肢体肌力减退就诊,经确诊后给予丙种球蛋白、激素以及免疫抑制剂治疗后症状部分缓解,但最终因合并症死亡。检索2000年~2013年国内SLE合并LM的相关病例报道。结果临床资料较为详细的国内报道中SLE合并LM14例（含本例）,其中女性10例,男性4例。所有患者均存在胸髓受累,其中合并颈髓受累7例,合并腰髓受累5例。经治疗6例好转,4例缓解不明显,4例因不同合并症死亡。结论 SLE合并LM严重影响患者的生存质量及预后,在SLE或脊髓炎等疾病诊断过程中应重点识别患者的临床类型,并应通过AQP4抗体的检测与视神经脊髓炎相鉴别,早期免疫支持治疗可显著改善患者的预后。

Objective We reported one case and reviewed literature of additional cases in China to summarize and analyze the clinical manifestations. Method We reported a 58-year-old woman in whom spinal cord dysfunction developed after pulmonary infection resulting in tetraparesis. After diagnosis, immunoglobulin, High-dose glucoconicoid therapy and immunosuppression started to bring an improvement, while she eventually died of complication. We also reviewed cases of SLE with LM from 2000 to 2013 in China. Result 14 cases with detailed clinical data were finally reviewed. There were 10 females and 4 males that all thoracic level were involved, while 6 cases had cervical involement and 2 affected the lumbar. Six patients had partial recovery, while 4 had poor response and 4 died from different complications. Conclusion LM with SLE currently resulted in severe and disabling sequelae. We should focus on the identification of the clinical patterns. AQP4 detection can rule out the mimics of NMO. Early immunesupport therapy may significantly improve the prognosis of patients.