贝利尤单抗治疗系统性红斑狼疮合并血栓性微血管病1例并文献复习

Belimumab in the treatment of systemic lupus erythematosus with thrombotic microangiopathy:a case report and literature review

目的 探讨贝利尤单抗治疗系统性红斑狼疮(systemic lupus erythematosus,SLE)并血栓性微血管病(thrombotic microangiopathy,TMA)患者的疗效。方法 总结中山市人民医院风湿免疫科应用贝利尤单抗治疗1例SLE并TMA患者的临床特征,随访患者治疗后的转归,并检索多个医学数据库建库至2023年8月的文献,收集国内外文献报道的使用贝利尤单抗治疗的SLE并TMA患者,并行文献复习。结果 国内外文献报道的使用贝利尤单抗治疗的SLE并TMA患者共8例,结合本例共9例患者,均为女性,年龄(34.3±15.9)岁;9例均有血液系统损害,5例伴神经系统损害,4例伴肾损害,3例曾行肾穿活检,1例为狼疮性肾炎Ⅳ型伴TMA,1例为狼疮性肾炎Ⅳ+Ⅴ型伴TMA,1例为狼疮性肾炎Ⅳ型;3例诊断SLE并TMA,6例诊断SLE并血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)。9例患者均使用激素冲击或大量激素疗法,8例使用血浆置换,3例使用环磷酰胺,1例使用吗替麦考酚酯;生物制剂方面,4例患者单用贝利尤单抗,5例序贯使用利妥昔单抗及贝利尤单抗。9例患者经治疗后临床症状均有改善,无死亡病例。结论SLE患者可并发TMA,病因复杂,标准治疗联合贝利尤单抗对SLE并TMA患者是有效且安全的,贝利尤单抗有望成为SLE并TMA患者的治疗新选择。

Objective To investigate the efficacy of belimumab on the treatment of systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA).Methods The clinical features of SLE patient with TMA treated by belimumab in the Rheumatology Department of Zhongshan People's Hospital were reported,and the outcomes of the patient were followed up.The relevant literature was reviewed and summarized.Results A total of 9 SLE patients with TMA were included.All of them were female with an average age of 34.3±15.9 years.All the patients had hematological damage,while 5 patients of them showed the nervous system damage,and four of them presented with kidney damage.3 patients underwent transrenal biopsy,which revealed type IV lupus nephritis(LN)with TMA,type IV+V LN with TMA,and type IV LN,respectively.Three patients were diagnosed with SLE and TMA,and six patients were diagnosed with SLE and thrombotic thrombocytopenic purpura(TTP).All the patients received glucocorticoid pulse therapy or high-dose glucocorticoids.Eight patients received plasma exchange,three patients received cyclophosphamide,and one patient received mycophenolate.Four patients received belimumab alone,and five patients received rituximab and belimumab sequentially.The clinical symptoms of all patients were improved after treatment,and no death case was reported.Conclusions SLE patients can be complicated with TMA,and the etiology is complex.Belimumab may be a novel and reasonable treatment strategy for SLE patients with TMA.