早期血浆置换与利妥昔单抗联合治疗抗SRP抗体阳性免疫介导坏死性肌病的疗效初探  

Treatment Efficacy of Early Therapeutic Plasma Exchange Combined with Rituximab in Newly Diagnosed Patients with Anti-signal Recognition Particle Antibody-positive Immune-mediated Necrotizing Myopathy

作　　者：成纳川 朱鑫方 朱雯华 赵重波[1,2,3] 赵彦胤 CHENG Na-chuan;ZHU Xin-fang;ZHU Wen-hua;ZHAO Chong-bo;ZHAO Yan-yin(Department of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China;National Center for Neurological Diseases,Shanghai 200040,China;Rare Disease Center,Huashan Hospital,Shanghai 200040,China;Department of Blood Transfusion,Huashan Hospital,Shanghai 200040,China)

机构地区：[1]复旦大学附属华山医院神经内科,上海200040 [2]国家神经疾病医学中心,上海200040 [3]复旦大学附属华山医院罕见病中心,上海200040 [4]复旦大学附属华山医院输血科,上海200040

出　　处：《中国临床神经科学》2024年第5期502-506,共5页Chinese Journal of Clinical Neurosciences

基　　金：国家自然科学基金资助项目(编号:81801242)。

摘　　要：目的探究抗信号识别颗粒(SRP)抗体阳性免疫介导坏死性肌病(IMNM)初治患者早期运用血浆置换(TPE)与利妥昔单抗联合治疗的效果。方法对3例初治抗SRP抗体阳性IMNM患者予TPE治疗,术后静脉输注利妥昔单抗100 mg(第1天)+500 mg(第2天)并予激素小剂量(<0.75 mg·kg^(-1)·d^(-1))口服,激素阶梯减量。在患者治疗前基线及治疗后第1、3、6个月采集徒手肌力检查(MMT)评分(MMT-8及MMT-26)、健康评估问卷的残疾指数(DI)、血清肌酸激酶(CK)等临床资料进行分析。结果3例患者均为女性,平均年龄47.3岁,平均病程11.3个月,确诊抗SRP抗体阳性IMNM前均未接受免疫治疗,治疗前四肢近端肌力2~3级,MMT-8平均59/80分,DI平均1.5分;血清CK平均值3391 U·L^(-1),经TPE与利妥昔单抗联合治疗后患者激素顺利阶梯减量,血清CK呈逐月下降趋势;运动功能第6个月四肢近端肌力3~4级,MMT-8平均71/80分,DI平均0.3分,治疗过程中无严重不良事件。结论对初治抗SRP抗体阳性IMNM患者采用早期TPE与利妥昔单抗联合治疗能快速缓解肌肉炎症活动,并可维持较长时间的免疫抑制作用,避免大剂量激素应用及激素减量过程中的炎症再活动。Aim To investigate the therapeutic effect of early therapeutic plasma exchange(TPE)combined with rituximab in newly diagnosed patients with anti-signal recognition particle(SRP)antibodypositive immune-mediated necrotizing myopathy(IMNM).Methods Three newly diagnosed anti-SRP antibody-positive IMNM patients underwent TPE followed by intravenous rituximab administration,with initial doses of 100 mg at day 1 and 500 mg at day 2,accompanied by a low oral steroid regimen(<0.75mg·kg^(-1)·d^(-1)).A steroid taper was subsequently initiated.Clinical data,including manual muscle testing(MMT)scores(MMT-8 and MMT-26),disability index(DI)from health assessment questionnaires,and serum creatine kinase(CK)levels,were collected at baseline and at 1,3,and 6 months posttreatment.Results All the three patients were female,with an average age of 47.3 years and an average disease duration of 11.3 months.Prior to the diagnosis of anti-SRP-positive IMNM,none had received immunotherapy.Pre-treatment proximal limb muscle strength was graded 2 to 3,with an average MMT-8 score of 59/80 and an average DI of 1.5.The average serum CK level was 3391 U·L^(-1).Following therapeutic plasma exchange and intravenous rituximab treatment,patients successfully tapered steroids,and serum CK levels showed a monthly decreasing trend.By the sixth month,proximal limb muscle strength was improved to grades 3 to 4,with an average MMT-8 score of 71/80 and an average DI of 0.3.No serious adverse events occurred during the treatment period.Conclusion Early therapeutic plasma exchange combined with rituximab in newly diagnosed patients with anti-SRP-positive IMNM can rapidly alleviate muscle inflammation and provide long-lasting immunosuppressive effects,thereby avoiding the use of high-dose steroids and the risk of inflammation recurrence during steroid tapering.

关 键 词：免疫介导坏死性肌病 坏死性肌炎 血浆置换 利妥昔单抗 抗信号识别颗粒 血清肌酸激酶 

分 类 号：R746[医药卫生—神经病学与精神病学]