Department of Clinical and Experimental Medicine, Neurological Clinic, University of Pisa, Pisa, Italy.
Department of Surgical Pathology, Medical, Molecular, and Critical Area, University of Pisa, Pisa, Italy.
Acta Myol. 2021 Jun 30;40(2):105-108. doi: 10.36185/2532-1900-050. eCollection 2021 Jun.
Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) related myositis is a form of immune-mediated necrotizing myopathy (IMNM). Anti-HMGCR autoantibodies target HMGCR, a glycoprotein linked to the endoplasmic reticulum implied in the cholesterol synthesis pathway, and exert a pathogenic effect on skeletal muscle cells. More than 60% of patients affected by HMGCR-related myositis shares statin-exposure in their medical history. Patients commonly experience CK levels elevation, myalgia, muscle weakness and soreness at variable extent, which manifest acutely or sub acutely with a progressively worsening course, in some cases mimicking limb-girdle muscular dystrophies (LGMD) phenotype and treatment is based on an immunosuppressive strategy. Here we present the peculiar case of a previously statins-exposed 72 y.o. asymptomatic man with persistent moderate hyperCKemia and high levels of anti-HMGCR, in which pharmacotherapy has not been initiated yet, while a wait-and-see approach has been adopted instead.
抗 3-羟基-3-甲基戊二酰辅酶 A 还原酶(HMGCR)相关肌炎是一种免疫介导的坏死性肌病(IMNM)。抗 HMGCR 自身抗体针对 HMGCR,这是一种与内质网相关的糖蛋白,与胆固醇合成途径有关,并对骨骼肌细胞产生致病作用。超过 60%的 HMGCR 相关肌炎患者在其病史中有他汀类药物暴露。患者常出现肌酸激酶(CK)水平升高、肌痛、肌肉无力和不同程度的酸痛,表现为急性或亚急性,逐渐加重,在某些情况下类似于肢带型肌营养不良(LGMD)表型,治疗基于免疫抑制策略。在这里,我们介绍了一个特殊的病例,一个之前使用过他汀类药物的 72 岁无症状男性,持续中度高肌酸激酶血症和高水平的抗 HMGCR,目前尚未开始药物治疗,而是采用了观望的方法。