■免疫介导的坏死性肌病(IMNM)是一种罕见的疾病,最早于2004年被描述。由于缺乏大型案例系列,对于IMNM没有正式的治疗建议.
■我们介绍了一例47岁的女性,她的四肢进行性无力,从下肢开始,逐渐影响上肢。她还报告在进行日常活动后出现呼吸困难。她入院时,她的上肢几乎无法移动,即使有支撑也无法站立。她的肌酸激酶(CK)水平显着增加(>3500u/l)。肌电图显示肌源性损伤,抗信号识别颗粒(抗SRP)和抗Ro52抗体高度阳性。右侧肱二头肌的病理活检显示骨骼肌坏死性肌病。她最终被诊断出患有抗SRPIMNN,并给予甲泼尼龙单药治疗和免疫球蛋白联合治疗,但她的症状继续恶化.患者拒绝承担环磷酰胺和利妥昔单抗可能导致的进一步肝功能障碍和血液系统损害,她选择尝试使用Ofatumumab(OFA)。
■接受三剂OFA治疗后,没有任何不良反应,她报告说,她的肌肉力量已经基本恢复,并且能够独立行走。循环系统中的B细胞已经耗尽,和肝功能等血液标志物一直保持在正常范围内。在后续行动中,她的活动耐受性继续提高。
■我们提出了一例SRP-IMNM的严重病例,其中患者对常规免疫疗法的反应较差。然而,早期序贯使用OFA治疗可快速缓解症状.这为SRP-IMNM的治疗提供了新的选择,未来将需要更多的大规模研究来验证我们的结果。
Immune-mediated necrotizing myopathies (IMNM) is a rare disease that was first described in 2004. Due to the lack of large
case series, there are no formal treatment recommendations for IMNM.
We presented a
case of a 47-year-old woman who experienced progressive limb weakness, starting from the lower limbs and gradually affecting the upper limbs. She also reported experiencing dyspnea after engaging in daily activities. When she was admitted to the hospital, her upper limbs were almost unable to move and she could not stand even with support. Her Creatine kinase (CK) level significantly increased (> 3500 u/l). Electromyography showed myogenic damage, anti-Signal recognition particle (anti-SRP) and anti-Ro52 antibodies were highly positive. Pathological biopsy of the right biceps muscle showed necrotizing myopathy in the skeletal muscle. She was ultimately diagnosed with anti-SRP IMNN, and was given monotherapy with methylprednisolone and combination therapy with immunoglobulin, but her symptoms continued to worsen. The patient refused to bear the possible further liver dysfunction and blood system damage caused by Cyclophosphamide and Rituximab, and she chose to try to use Ofatumumab (OFA).
After receiving three doses of OFA treatment without any adverse reactions, she reported that her muscle strength had basically recovered and she was able to walk independently. The B cells in the circulatory system have been depleted, and blood markers such as liver function have consistently remained within normal range. During the follow up, her activity tolerance continued to improve.
We have presented a severe
case of SRP-IMNM in which the patient showed poor response to conventional immunotherapy. However, rapid symptom relief was achieved with early sequential use of OFA treatment. This provides a new option for the treatment of SRP-IMNM, and more large-scale studies will be needed in the future to verify our results.