Li Jiali, Yan Mingming, Qin Jiao, He Lingyan, Dai Cao, Wen Rui
Department of Rheumatology and Immunology, University of South China Affiliated Changsha Central Hospital, 161 South Shaoshan Road, Changsha, 410008, Hunan, China.
Department of Orthopaedic Surgery, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China.
Thromb J. 2021 Nov 25;19(1):92. doi: 10.1186/s12959-021-00347-x.
Immune-mediated necrotizing myopathy (IMNM) is characterized by proximal muscle weakness, elvated serum muscle enzyme levels, myopathic electromyography findings, and necrotic muscle fiber with few inflammatory cell infiltration in muscle biopsies. Statins, the first line drug to lower triglyceride and cholesterol level in blood, have been reported to be associated with statins-induced necrotizing autoimmune myopathy (SINAM). Although anti-3-hydroxy-3-methylglutarylcoenzyme-A reductase (anti-HMGCR) myopathy is considered as the leading myopathy related to the statins medication, anti-signal recognition particle (SRP) myopathy were also identified in several cases with statin exposure. The risk of deep venous thrombosis (DVT) is substantially high in individuals with autoimmune inflammatory diseases. But few studies have reported the occurrence and recommendation for treatment of DVT in patients with anti-SRP myopathy. Here, we reported a statin-exposed anti-SRP myopathy individual developed DVT who was successfully treated with catheter-directed thrombolysis (CDT) and systemic anticoagulants therapy.
A 56-year-old Chinese female came to the outpatient room with gradually progressive bilateral lower-extremity weakness. Magnetic resonance imaging revealed myopathy in bilateral thighs. Serum anti-SRP antibody was positive. She was diagnosed with anti-SRP myopathy. When treated with corticosteroids and immunosuppressants, the patient developed mild edema and pain of left lower extremity. Angiography and ultrasound revealed diffuse venous thrombosis of left lower extremity. Therapy was initiated with CDT and lower molecular weight heparin, then switched to once daily oral rivaroxaban. Meanwhile, steroids combined with tacrolimus were also carried on while simvastatin was discontinued. One month later, patient's symptoms were resolved and only partial thrombosis in left femoral vein was remained.
The prevalence of DVT in patient with anti-SRP myopathy was rare. No well-established treatment strategy is available to manage the IMNM and DVT at the same time. The systemic anticoagulants therapy combined CDT can be an effective therapeutic approach to address extensive DVT in patient with anti-SRP myopathy.
免疫介导性坏死性肌病(IMNM)的特征为近端肌无力、血清肌肉酶水平升高、肌病性肌电图表现,以及肌肉活检显示坏死性肌纤维且炎症细胞浸润较少。他汀类药物是降低血液中甘油三酯和胆固醇水平的一线药物,据报道与他汀类药物诱导的坏死性自身免疫性肌病(SINAM)有关。尽管抗3-羟基-3-甲基戊二酰辅酶A还原酶(抗HMGCR)肌病被认为是与他汀类药物治疗相关的主要肌病,但在几例使用他汀类药物的患者中也发现了抗信号识别颗粒(SRP)肌病。自身免疫性炎症性疾病患者发生深静脉血栓形成(DVT)的风险相当高。但很少有研究报道抗SRP肌病患者DVT的发生情况及治疗建议。在此,我们报告了1例使用他汀类药物的抗SRP肌病患者发生DVT,经导管直接溶栓(CDT)和全身抗凝治疗成功治愈。
一名56岁中国女性因双侧下肢渐进性无力前来门诊就诊。磁共振成像显示双侧大腿存在肌病。血清抗SRP抗体呈阳性。她被诊断为抗SRP肌病。在使用皮质类固醇和免疫抑制剂治疗时,患者出现左下肢轻度水肿和疼痛。血管造影和超声检查显示左下肢弥漫性静脉血栓形成。开始采用CDT和低分子量肝素治疗,随后改为每日口服一次利伐沙班。同时,继续使用类固醇联合他克莫司,停用辛伐他汀。1个月后,患者症状缓解,仅左股静脉残留部分血栓。
抗SRP肌病患者中DVT的发生率罕见。目前尚无成熟的治疗策略可同时处理IMNM和DVT。全身抗凝治疗联合CDT可能是治疗抗SRP肌病患者广泛DVT的有效治疗方法。