【摘要】 目的分析他汀相关免疫介导性坏死性肌病(immune-mediated necrotising myopathy,IMNM)患者的临床表现、实验室检查、电生理和肌肉病理学特点。方法回顾性分析中国人民解放军总医院2014年9月至11月2例他汀相关IMNM患者临床表现、实验室检查、电生理和肌肉活检病理特点,以及治疗、随访情况。结果 2例患者均为女性、急性或亚急性起病,发病年龄分别为55和63岁,病程分别为5和3个月。2例患者既往体健,服用他汀类药物半年后出现四肢乏力、抬头困难等不适,1例合并呼吸系统受累,1例患者可见较多肌纤维空泡和凝固性坏死,伴吞噬现象。另1例可见少量肌纤维变性、坏死,伴有少数核内移纤维。1例患者经糖皮质激素激素、另1例经糖皮质激素联合免疫抑制剂治疗有效。结论他汀相关IMNM是一种复杂的异质性疾病,呈亚急性或隐匿性起病,病情轻重不一;主要表现为肢带肌乏力,可合并有不同肌群受累;肌肉病理改变主要为形态多样的肌纤维变性坏死;激素及免疫抑制剂治疗有效。更多还原

【Abstract】 Objective To describe the clinical,laboratory examinations,electrophysiology and muscle biopsy of two cases of immune-mediated necrotizing myopathy( IMNM) associated with statins. Methods We retrospectively analyzed the clinical, laboratory examinations, electrophysiology and muscle biopsy characteristics of two IMNM patients associated with statins in our departments from September to November,2014. Results The two patients were both women( aged 55 and 63 years old) with subacute onset diseases. Disease durations were 5months and 3months respectively. They were generally in good condition taking statins as anti-cholesteremic agent for about half a year. The major presentations were limb weakness and cervical flexor muscle anergy. One patient had pulmonary involvement. Muscle biopsy showed that one had widely distributed myofibre cavity and coagulation necrosis with endocytosis,the other had major myofibre degeneration and necrosis with few nuclear ingression. One patient was treated with corticosteroids and the other was treated with combined corticosteroids and immunosuppressive agents. Conclusions IMNM associated with statins is a complex and heterogeneous disease,which can be manifested as acute or subacute myalgia and weaknesswith different muscle groups involvement. Muscle biopsy findings show diverse myofibre degeneration and necrosis. Corticosteroids and/or immunosuppressive agent treatment can get good response.更多还原