Objective: To investigate the pathogenesis and management of the hydrocephalus in patients with systemic lupus erythematosus (SLE). Methods: Eight cases of hospitalized lupus patients with hydrocephalus in Peking Union Medical College Hospital from Jan 1990 to Mar 2017 were analyzed retrospectively.We collected the patients' medical records including medical history, CT and/or MRI images and analysis of cerebrospinal fluid via lumbar puncture in order to investigate the pathogenesis of the hydrocephalus in patients with SLE and summarize treatment experience. Results: All the 8 patients with SLE complicated with hydrocephalus were treated with steroids or immunosuppressive agents, and 5 cases were diagnosed with the central nervous system infection. Three cases received ventriculoperitoneal shunt, 5 cases received treatment of medicine.They all were followed up for 2-12 months, of which 3 cases were markedly effective, 1 case effective and 4 cases dead. Conclusion: The pathogenesis of lupus combined with hydrocephalus may have a certain relationship with the central infection, should take effective anti-infection treatment.We recommend cerebrospinal fluid shunt surgery intervention.
目的： 探讨系统性红斑狼疮(SLE)并发脑积水的可能机制及治疗方法。 方法： 回顾性总结北京协和医院1990年1月至2017年3月收治的8例SLE合并脑积水患者的病史、CT和(或)MRI影像资料、腰椎穿刺化验等临床资料，分析SLE患者并发脑积水的发病机制，总结治疗经验。 结果： 8例SLE合并脑积水的患者均接受激素或免疫抑制剂治疗，5例合并中枢神经系统感染。其中3例行脑室腹腔分流术，5例内科保守治疗，随访2～12个月，显效3例，有效1例，死亡4例。 结论： SLE合并脑积水的发病机制可能与中枢感染存在一定关系，应积极抗感染治疗；推荐行脑脊液分流手术干预。.
Keywords: Autoimmune Diseases; Hydrocephalus; Systemic lupus erythematosus; Ventdculoperitoneal shunt.