中国实用神经疾病杂志2026，Vol.29Issue(1)：53-57,5.DOI:10.12083/SYSJ.251273
抗HMGCR抗体阳性的免疫介导坏死性肌病的临床表现肌肉影像学及病理学特征分析
Anti-HMGCR antibody-positive immune-mediated necrotizing myopathy:A clinical manifestations,muscle imaging,and pathological characteristics analysis
摘要
Abstract
Objective To analyze the clinical manifestations,muscle imaging features,and pathological characteristics of patients with immune-mediated necrotizing myopathy(IMNM)who are positive for anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase(HMGCR)antibodies.Methods Clinical data were collected from four patients diagnosed with HMGCR-IMNM at the Department of Neurology,Anyang People's Hospital Affiliated to Henan Medical University between January 2024 and March 2025.Their clinical presentations,muscle imaging findings,and pathological features were analyzed and summarized.Results All four patients were female,and their onset age were all over 45 years old.All presented with varying degrees of proximal limb muscle weakness,one patient also experienced myalgia,and two had muscle atrophy.Serum creatine kinase(CK)levels were significantly elevated in all cases,and electromyography(EMG)indicated myopathic changes.Lower limb MRI revealed varying degrees of skeletal muscle edema and fatty infiltration,predominantly involving the posteromedial thigh muscles and the soleus muscle.Muscle pathology demonstrated varying degrees of muscle fiber atrophy,necrosis,and regeneration,with minimal or absent inflammatory cell infiltration.Scattered lipid accumulation in muscle fibers in two cases,and immunohistochemical staining identified CD68-positive lymphocytes in two cases.Conclusion Clinically,patients with HMGCR-IMNM predominantly present with proximal limb muscle weakness of variable severity.MRI reveals a predilection for lesion involvement in the posteromedial thigh muscles and the soleus muscles of the lower legs,with diffuse edema.Histopathologically,the cardinal muscle finding is myofiber necrosis and regeneration with varying degrees of severity.关键词
抗3-羟基-3-甲基戊二酰辅酶A还原酶/坏死性肌病/特发性炎性肌病/磁共振成像/肌肉病理/肌肉影像学/临床表现Key words
Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase/Necrotizing myopathy/Idiopathic inflammatory myopathy/Magnetic resonance imaging/Muscle pathology/Muscle imaging/Clinical manifestations分类
医药卫生引用本文复制引用
王祎纯,李海燕..抗HMGCR抗体阳性的免疫介导坏死性肌病的临床表现肌肉影像学及病理学特征分析[J].中国实用神经疾病杂志,2026,29(1):53-57,5.基金项目
河南省医学科技攻关计划项目(编号:LHGJ20240521) （编号:LHGJ20240521）
