系统性红斑狼疮合并横贯性脊髓炎患者临床特征

摘要

Objective Transverse myelitis (TM)as a manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE)is a serious complication of systemic lupus erythematosus (SLE).Clinicians need to be vigilant to this special condition.Methods This study retrospectively collected 19 cases of SLE-TMinpatients and outpatients in Peking Union Medical College Hospital during 1993—2013.Descriptive analysis and literature review were performed.Results Among 19 SLE-TM patients,18 were female.The median onset age of SLE was 36 years old (13-70 years).Seventeen patients presented with decreased muscle strength of bilateral lower extremities.Thirteen patients had sensory abnormalities.Fifteen patients had sphincter dysfunction.Six patients had optic neuritis.Eighteen patients had spinal MRI examination,16 cases had abnormal signal of spinal cord.ANA of all patients were positive.APQ4 antibody was detected in only 3 patients in blood and cerebrospinal fluid,2 patients had positive antibody in blood,but all negative in cerebrospinal fluid.SLE-TMpatients were compared with 43 patients in the control group.SLE-TM patients had higher degree of fever than patients in the control group (63.2% vs.7.0%,P <0.05 ),but the presence of photosensitivity was significantly less than that of the control group (5.3% vs.27.9%,P <0.05).The anti-dsDNA positive rate of the SLE-TM group was significantly less than that of the control group (26.3% vs.55.8%,P <0.05).Sixteen patients were given steroid pulse treatment,the rest of them were given large dose steroid therapy.Fifteen patients were on single immunosuppressant of cyclophosphamide (CTX)therapy.Three patients were on combined immunosuppressant treatment.One patient was treated with Rituximab.Six patients had severe neurological deficits remained.Conclusions TMis a serious complication of SLE,and the quality of life of patients is severely impacted.Each segment of the spinal cord could be involved,but thoracic segment is the most frequently involved.Early steroid and immunosuppressive therapy can improve outcomes.%目的：横贯性脊髓炎（transverse myelitis，TM）作为神经精神狼疮（neuropsychiatric systemic lupus erythematosus， NPSLE）的表现之一，是系统性红斑狼疮（systemic lupus erythematosus，SLE）的严重合并症，临床医师需要提高对 SLE 合并TM（SLE-TM）的认识。方法本研究回顾性收集北京协和医院1993至2013年的住院和门诊随访病例，对 SLE 合并 TM患者的临床表现，实验室检查，治疗方法和预后进行分析。并与43例北京协和医院同时期住院无脊髓受累 SLE 患者进行对比。结果SLE-TM患者中18例为女性，1例为男性。平均年龄36岁（13～70岁）。17例患者存在双下肢肌力下降，13例存在感觉平面，15例存在括约肌功能障碍，6例合并视神经炎。18例患者进行脊髓核磁检查，16例有脊髓异常信号。19例患者抗核抗体均为阳性，仅3例进行了血和脑脊液水通道蛋白-4抗体检测，其中2例血水通道蛋白-4抗体为阳性，脑脊液水通道蛋白-4均为阴性。SLE-TM患者伴发热患者多于对照组，差异有统计学意义（63．2％比7．0％，P ＜0．05）；而伴光过敏患者少于对照组（5．3％比27．9％，P ＜0．05）；SLE-TM抗双链 DNA 抗体阳性率少于对照组，差异有统计学意义（26．3％比55．8％，P ＜0．05）；其余自身抗体（包括抗磷脂抗体）和系统性红斑狼疮疾病活动度指数两组间差异无统计学意义。16例患者经过激素冲击治疗，其余均予以大剂量糖皮质激素（1～2 mg·kg －1·d －1）治疗。15例单独应用环磷酰胺治疗，3例应用免疫抑制剂联合治疗，1例应用利妥昔单抗。6例患者有明显神经系统后遗症。结论TM是 SLE 的严重并发症，可明显影响患者功能。各节段脊髓均可受累，其中以胸髓多见。早期经糖皮质激素和免疫抑制剂治疗可改善患者预后。