系统性红斑狼疮合并视神经脊髓炎1例并文献复习

Systemic lupus erythematosus with neuromyelitis optica：a case report and literature review

摘要:

目的：探讨系统性红斑狼疮（systemic lupus erythematosus，SLE）合并视神经脊髓炎（neuromyelitis optica，NMO）的临床特征、治疗及预后。方法：对重庆医科大学附属儿童医院1例确诊为SLE合并NMO的患儿临床表现、实验室检查及治疗进行总结，结合国内外文献报道共31例临床资料进行分析。结果：①患者，男，10岁8个月，以皮疹、关节炎起病，出现进行性肌力下降、视力下降、蛋白尿、血清ANA阳性、NMO-IgG抗体阳性、脊髓MRI全脊髓广泛水肿样异常信号，确诊为SLE合并NMO，经甲泼尼龙、丙球冲击，吗替麦考酚酯口服及利妥昔单抗静脉治疗，视力、肌力逐渐恢复，随访半年无复发。②结合文献，31例患者中最常见的临床表现为视力损害（27/31）、肌力下降（24/31）、感觉异常（21/31）。31例患者均伴血清ANA阳性、脊髓MRI异常信号，NMO-IgG抗体阳性（24/26），dsDNA阳性（17/26），补体下降（13/24）。③31例患者均使用皮质激素治疗，急性期给予甲泼尼龙冲击（27/31）、血浆置换（11/31）及丙球（9/31）等治疗。30例加用免疫抑制剂治疗，包括环磷酰胺冲击治疗15例，利妥昔单抗静脉10例，硫唑嘌呤及吗替麦考酚酯口服分别8例、7例。④12例环磷酰胺冲击治疗患者随访半年以上，其中6例复发（复发31次，包括31次脊髓炎，2次视神经炎）；10例利妥昔单抗治疗患者，8例无复发，4例年复发率减少。结论：SLE合并NMO多表现为反复多相病程，复发比例高，易遗留瘫痪、失明等并发症，血清NMO-IgG抗体及脊髓MRI检查阳性率高。本病一旦确诊，早期使用皮质激素冲击，联合生物制剂利妥昔单抗治疗可降低复发频率，改善病情及预后。

Abstract:

Objective：To analyze the clinical characteristics，treatment and prognosis of systemic lupus erythematosus（SLE） with neu－romyelitis optica（NMO）. Methods：Clinical manifestation，laboratory examination，and diagnosis and treatment of one child with SLE and NMO in our hospital was summarized，and clinical data of 30 reported cases about SLE with NMO at home and abroad were analyzed in combination with our case. Results：①The patient was 10 years and 8 months old，with onset of skin rash and arthritis. He developed progressive muscle weakness，impaired vision，proteinuria，positive serum autoantibody to nuclear antigen（ANA），positive anti-NMO-immunoglobuin G（IgG） antibody and abnormal edema pattern in the whole spinal cord，with final diagnosis of SLE with NMO. He was treated by methylprednisolone pulse and immuneglobulin（IVIG） pulse，oral mycophenolate mofetil（MMF），and intra－venous rituximab（RTX）. After treatment，his vision and myodynamia were gradually recovered，without relapse after six months of fol－low-up. ②Combing with literatures，the most common clinical manifestations among those 31 patients were impaired vision（27/31），muscle weakness（24/31） and paresthesia（21/31）. All patients had positive ANA and abnormal MRI signals of spinal cord，including positive double-stranded DNA（dsDNA） antibody（17/26），hypocomplementemia（13/24） and positive NMO-IgG antibody（24/26）. ③All patients were treated with glucocorticoid；treatment in the acute phase included methylprednisolone pulse（27/31），lasmaphere－sis （11/31），IVIG（9/31） and so on. All 30 patients were treated with immunosuppressants，including cyclophosphamide（CTX） pulse （15/31），intravenous RTX（10/31），azathioprine（AZA）（8/31），and oral MMF（7/31）. ④Those 12 patients treated with CTX had a following up for more than half a year，including relapse of 6 patients（31 times of relapse，including 31 times of myelitis and 2 times of optic neuritis）；among 10 patients treated with RTX，8 patients had no recurrence and 4 patients had a re－duced annual recurrence rate. Conclusion：SLE complicated with NMO is characterized by repeated polyphase course with high re－currence rate，is also prone to complications such as paralysis and blindness and etc. and positive rate of serum NMO-IgG antibody and spinal MRI is high. Early methylprednisolone pulse in combination with RTX can reduce the frequency of recurrence，and signifi－cantly improve the condition and prognosis.