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免疫介导坏死性肌病发病机制及诊治分析
关却卓玛,杨立川,秦伟,付平
0
(四川大学华西医院肾脏内科)
摘要:
目的 探讨免疫介导坏死性肌病的发病机制与临床诊治思路。方法 选取我院肾内科2016年3月收治的1例经肌活检证实为免疫介导坏死性肌病的女性肾病综合征患者,给予糖皮质激素治疗,同时积极控制其肾病综合征,并对其住院诊治过程及门诊随访结果进行分析。结果 糖皮质激素治疗后,肌病迅速得到缓解。随访22个月,肾病在泼尼松联合免疫抑制剂治疗半年,小剂量激素维持半年后得到临床缓解,停药后肾病稳定,随访期间肌病未再复发。结论 免疫介导坏死性肌病为免疫介导的肌病,骨骼肌活检是确诊的主要手段,该病对糖皮质激素治疗的反应良好。但在治疗过程中应重视可能引起免疫紊乱的共病治疗。
关键词:  免疫介导坏死性肌病  特发性炎性肌病  骨骼肌活检  IgA肾病  急性肾损伤  免疫紊乱
DOI:
基金项目:四川省科技支撑计划项目(2017SZ0113)
Pathogenesis and clinical diagnosis and treatment of -immune mediated necrotic myopathy
Guanquezhuoma,YANG Lichuan,QIN Wei,FU Ping
(Immune-mediated necrotizing myopathy;Idiopathic inflammatory myopathies;Skeletal muscle biopsy;IgA nephropathy;Acute kidney injury;Immunologic derangement)
Abstract:
Objective To investigate the pathogenesis, clinical diagnosis and treatment of immunemediated necrotic myopathy(IMNM). Methods A female patient diagnosed as IMNM by skeletal muscle biopsy was treated with corticosteroids. The hospitalization and outpatient follow-up information were analyzed. Results The patient was followed up for 22 months,and myopathy was quickly relieved after corticosteroids treatment and nephropathy was clinically relieved after prednisone combined with immunosuppressive therapy. The nephrotic syndrome was stable and the myopathy was not recurring after stopping the drug for a long time. Conclusion IMNM is caused by autoimmune abnormalities. Skeletal muscle biopsy is the main method for diagnosis, and IMNM responds well to glucocorticoid therapy. And during the treatment, attention also should be paid to the treatment of comorbidities that may cause immune disorders further.
Key words:  Immune-mediated necrotizing myopathy  Idiopathic inflammatory myopathies  Skeletal muscle biopsy  IgA nephropathy  Acute kidney injury  Immunologic derangement

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