Ding Hongyun, Li Zhen, Zhang Jianbin
Department of Nephrology.
Department of nutriology, YongChuan Hospital of Chongqing Medical University, ChongQing, China.
Medicine (Baltimore). 2019 Apr;98(16):e15206. doi: 10.1097/MD.0000000000015206.
Cyclosporine A (CsA) is a potent immunosuppressive agent originally used to prevent rejection after organ transplantation but now more frequently used for treatment of refractory autoimmune diseases. It can induce adverse effects, such as nephrotoxicity, gastrointestinal reactions, and gingival hyperplasia whist myopathy with subacute muscular atrophy are rare.
A 55-year-old male patient with idiopathic membranous nephropathy treated with cyclosporine A at 3 mg/kg/d and prednisone at 0.5 mg/kg.d for more than 20 days, gradually developed lower limb weakness, which were progressively aggravated until he was unable to stand or walk. A physical examination show muscle atrophy of both lower extremities, which was more severe in the right thigh muscle than the left, decreased muscular tension of the limbs was also observed.
Light microscopy and Transmission electron microscopy of muscle (quadriceps femoris) biopsy revealed drug-induced myopathy rather than neurogenic damage.
Cyclosporine was withdrawn and replaced with cyclophosphamide tablets, prednisone remain unchanged and other symptomatic therapies were also administered.
His bilateral thigh muscle atrophy showed improvement and lower limb weakness was obviously alleviated and he could stand and walk with the help of others 4 weeks later. Gradually, his thigh muscle atrophy was alleviated so that he was able to walk independently. After follow-up, no similar symptoms were found in the patients.
CsA-induced myopathy with muscular atrophy is rare and serious, which can be identified according to pathological characteristics.
环孢素A(CsA)是一种强效免疫抑制剂,最初用于预防器官移植后的排斥反应,但现在更常用于治疗难治性自身免疫性疾病。它可引起不良反应,如肾毒性、胃肠道反应和牙龈增生,而伴有亚急性肌肉萎缩的肌病较为罕见。
一名55岁男性特发性膜性肾病患者,接受环孢素A 3mg/kg/d及泼尼松0.5mg/kg.d治疗20余天,逐渐出现下肢无力,且进行性加重,直至无法站立或行走。体格检查显示双下肢肌肉萎缩,右大腿肌肉萎缩较左侧更严重,还观察到肢体肌张力降低。
肌肉(股四头肌)活检的光镜和透射电镜检查显示为药物性肌病而非神经源性损伤。
停用环孢素,换用环磷酰胺片,泼尼松剂量不变,并给予其他对症治疗。
4周后,他双侧大腿肌肉萎缩有所改善,下肢无力明显缓解,在他人帮助下能够站立和行走。逐渐地,他的大腿肌肉萎缩减轻,能够独立行走。随访期间,患者未再出现类似症状。
CsA引起的伴有肌肉萎缩的肌病罕见且严重,可根据病理特征进行识别。