Liang Emily, Rastegar Mandana
University of California Los Angeles David Geffen School of Medicine, Los Angeles, California, USA.
Department of Medicine, University of California Los Angeles David Geffen School of Medicine, Los Angeles, California, USA.
BMJ Case Rep. 2018 Apr 24;2018:bcr-2017-223870. doi: 10.1136/bcr-2017-223870.
Immune-mediated necrotising myopathy (IMNM) is a type of inflammatory myopathy characterised by acute or subacute severe proximal muscle weakness, significantly elevated creatine kinase levels, and prominent myofibre necrosis and regeneration with little or no inflammation. A subtype of IMNM identified by anti-HMG-CoA reductase (HMGCR)antibodies has been shown to be associated with statin exposure. Treatment of IMNM consists of immunosuppression with steroids, steroid-sparing agents, intravenous immune globulin and/or biologics. We present here a case of anti-HMCGR-associated IMNM and review the pathophysiology, diagnosis and treatment to increase physician awareness of this rare and debilitating condition.
免疫介导的坏死性肌病（IMNM）是一种炎性肌病，其特征为急性或亚急性严重近端肌无力、肌酸激酶水平显著升高，以及明显的肌纤维坏死和再生，炎症轻微或无炎症。已证明由抗3-羟基-3-甲基戊二酰辅酶A还原酶（HMGCR）抗体识别的IMNM亚型与他汀类药物暴露有关。IMNM的治疗包括使用类固醇、类固醇替代药物、静脉注射免疫球蛋白和/或生物制剂进行免疫抑制。我们在此报告一例抗HMGCR相关的IMNM病例，并回顾其病理生理学、诊断和治疗方法，以提高医生对这种罕见且使人衰弱疾病的认识。
