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珍珠与牡蛎:治疗因抗 HMG-CoA 还原酶相关肌病致他汀类药物不耐受患者的胆固醇

Pearls & Oy-sters: Managing Cholesterol in a Patient With Statin Intolerance Due to Anti-HMG-CoA Reductase-Associated Myopathy.

机构信息

From the Program of Immunology (R.N.), Institute of Biomedical Sciences, Faculty of Medicine (E.V.), Universidad de Chile. Santiago; Department of Pathology (K.B., K.C.), Hospital San Juan de Dios, Universidad de Chile. Santiago; and Department of Neurology and Neurosurgery (A.C.V., C.G.), Faculty of Medicine, Hospital Clínico Universidad de Chile. Santiago.

出版信息

Neurology. 2022 Nov 14;99(20):909-913. doi: 10.1212/WNL.0000000000201315.

Abstract

Statins are the first line of treatment for hypercholesterolemia and of prevention of atherosclerotic cardiovascular disease (ASCVD). It is estimated that 1 in 4 Americans over the age of 40 years use statins. In rare cases, patients may develop an autoimmune myopathy associated with antibodies against 3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR). Anti-HMGCR-associated myopathy requires immediate discontinuation of statins plus initiation of immunosuppressive therapy. Suspension of statin treatment worsens low-density lipoprotein-cholesterol (LDL-C) control, leading to an increased risk of ASCVD and necessitating commencement of another treatment for dyslipidemia. Unfortunately, the management of dyslipidemia in these patients is still unclear. In this study, we describe the case of a 65-year-old woman with dyslipidemia treated with atorvastatin, who consulted for long-standing muscle pain associated with symmetrical proximal weakness. Laboratory tests showed elevated levels of creatine kinase and anti-HMGCR antibodies. She was diagnosed with an anti-HMGCR-associated myopathy and was successfully treated with corticosteroids and azathioprine as immunosuppressive therapy, followed by ezetimibe for LDL-C reduction. We present key findings for early recognition and treatment of anti-HMGCR-associated myopathy and give recommendations on how to manage hypercholesterolemia in a patient with statin intolerance due to this disease.

摘要

他汀类药物是治疗高胆固醇血症和预防动脉粥样硬化性心血管疾病(ASCVD)的一线药物。据估计,年龄在 40 岁以上的美国人中有 1/4 使用他汀类药物。在极少数情况下,患者可能会出现与 3-羟基-3-甲基戊二酰辅酶 A 还原酶(HMGCR)抗体相关的自身免疫性肌病。抗 HMGCR 相关肌病需要立即停用他汀类药物并开始免疫抑制治疗。停止他汀类药物治疗会使低密度脂蛋白胆固醇(LDL-C)控制恶化,导致 ASCVD 风险增加,并需要开始另一种血脂异常治疗。不幸的是,这些患者的血脂异常管理仍不清楚。在这项研究中,我们描述了一位 65 岁患有血脂异常的女性患者的病例,她因长期肌肉疼痛和对称性近端无力就诊。实验室检查显示肌酸激酶和抗 HMGCR 抗体水平升高。她被诊断为抗 HMGCR 相关肌病,并成功接受了皮质类固醇和硫唑嘌呤作为免疫抑制治疗,随后使用依折麦布降低 LDL-C。我们提出了早期识别和治疗抗 HMGCR 相关肌病的关键发现,并就如何管理因这种疾病导致他汀类药物不耐受的高胆固醇血症提出了建议。

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