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抗3-羟基-3-甲基戊二酰辅酶A还原酶(HMGCR)肌病伴急性收缩性心力衰竭

Anti-HMGCR myopathy presenting with acute systolic heart failure.

作者信息

Pitlick Mitchell, Ernste Floranne

机构信息

Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Rheumatology, Mayo Clinic, Rochester, Minnesota, USA.

出版信息

BMJ Case Rep. 2019 May 8;12(5):e230213. doi: 10.1136/bcr-2019-230213.

DOI:10.1136/bcr-2019-230213
PMID:31068355
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6506079/
Abstract

Necrotising autoimmune myopathy (NAM) is an immune-mediated myopathy that may be associated with statin use, malignancy or an autoimmune connective tissue disease, but it can also be idiopathic. Anti-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy is an extremely rare side effect of statin use, occurring in approximately 2-3 out of every 100 000 patients who use statins. Patients typically present with subacute proximal muscle weakness and creatine kinase levels >10 times the upper limit of normal. The diagnosis is suggested by muscle biopsy showing necrotic fibres with minimal inflammation along with positive anti-HMGCR antibodies. Treatment nearly always requires multiple immunosuppressive agents, the earlier use of which is associated with improved outcomes. Reports of statin-induced NAM leading to heart failure are limited. We present the case of a 69-year-old woman with statin-induced NAM who presented with acute systolic heart failure. Early initiation of high-dose corticosteroids and IVIG resulted in significant improvement in her symptoms.

摘要

坏死性自身免疫性肌病(NAM)是一种免疫介导的肌病,可能与他汀类药物使用、恶性肿瘤或自身免疫性结缔组织病有关,但也可能是特发性的。抗羟甲基戊二酰辅酶A还原酶(HMGCR)肌病是他汀类药物使用极为罕见的副作用,每10万名使用他汀类药物的患者中约有2 - 3人会出现。患者通常表现为亚急性近端肌无力,肌酸激酶水平高于正常上限10倍以上。肌肉活检显示坏死纤维且炎症轻微,同时抗HMGCR抗体呈阳性,提示可作出诊断。治疗几乎总是需要多种免疫抑制剂,早期使用与改善预后相关。他汀类药物诱导的NAM导致心力衰竭的报道有限。我们报告了一例69岁女性他汀类药物诱导的NAM患者,该患者出现急性收缩性心力衰竭。早期大剂量使用皮质类固醇和静脉注射免疫球蛋白使她的症状有显著改善。

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本文引用的文献

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Statin-Associated Autoimmune Myopathy.他汀类药物相关自身免疫性肌病
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An Unusual Case of Statin-Induced Myopathy: Anti-HMGCoA Necrotizing Autoimmune Myopathy.他汀类药物诱导的肌病罕见病例:抗HMGCoA坏死性自身免疫性肌病
J Gen Intern Med. 2015 Dec;30(12):1879-83. doi: 10.1007/s11606-015-3303-9. Epub 2015 Apr 9.