文章摘要
曾艳平,柳胤,解燕春,梁静静,卢祖能.抗HMGCR/SRP抗体阳性的特发性炎性肌病特点分析[J].神经损伤功能重建,2019,14(7):333-335
抗HMGCR/SRP抗体阳性的特发性炎性肌病特点分析
Characteristics of Idiopathic Inflammatory Myopathy with Anti-HMGCR or Anti-SRPAntibodies
  
DOI:
中文关键词: 特发性炎性肌病  免疫坏死性肌病  肌炎自身抗体  肌肉病理
英文关键词: idiopathic inflammatory myopathy  necrotizing myopathy  myositis antibody  muscle pathology
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作者单位
曾艳平,柳胤,解燕春,梁静静,卢祖能 武汉大学人民医院 神经内科 
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中文摘要:
      目的:探讨抗HMGCR/SRP 抗体阳性的特发性炎性肌病(IIM)的临床和病理特点。方法:收集 HMGCR/SRP 抗体阳性的IIM 患者5 例,对其危险因素、临床表现、实验室检查、肌电图、肌肉MRI、肌肉病 理、肌炎自身抗体及药物治疗进行分析。结果:抗HMGCR/SRP抗体阳性的IIM 患者临床变异较大,但大多 有肌肉无力;血清肌酸激酶均较高;5 例患者肌电图均表现为典型的肌源性损害。肌肉MRI主要表现为肌肉 水肿。3 例为典型坏死性肌病表现,1 例镜下偶见肌细胞坏死,1 例为多发性肌炎表现。HMGCR抗体阳性患 者其中1 例为正服用他汀药物,另一患者服用抗精神病药物;SRP 抗体阳性患者1 例为自身免疫性疾病患 者,1 例长期服用他汀,另1 例病因未明确。激素治疗效果不一,2 例加用丙种球蛋白治疗,1 例加用免疫抑制 剂治疗。结论:抗HMGCR/SRP抗体阳性的IIM 临床表现各异,肌电图仅能定位肌肉损害,主要依靠肌肉活 检,肌炎自身抗体检查更有助于诊断和具体分型,疗效各型不一。
英文摘要:
      To explore clinical and pathological characteristics of idiopathic inflammatory myopathy with anti-HMGCR or anti-SRP antibodies. Methods: Total 5 patients positive for anti-HMGCR/anti-SRP antibody with idiopathic inflammatory myopathy were enrolled in this study. Risk factors, clinical manifestations, laboratory findings, electromyography, muscle MRI, muscle pathology, myositis autoantibody, and drug treatment information were collected and analyzed. Results: Idiopathic inflammatory myopathy patients that were anti-HMGCR/anti-SRP antibody-positive displayed a large variation in clinical symptoms with most patients showing muscle weakness. Serum creatine kinase level was elevated in all patients. Electromyographical studies showed typical myogenic impairments in all patients. Muscle MRI was mainly characterized by muscle edema. Pathological manifestations of muscle biopsy showed necrotizing myopathy in 3 patients that were anti-HMGCR/anti-SRP antibody-positive, occasionally seen muscle fiber necrosis in 1 patient, and polymyositis in 1 patient. Of the 2 patients that were anti-HMGCR antibody-positive, 1 was taking statin, and the other was taking antipsychotics. Of the 3 patients that were anti-SRP antibody-positive, 1 patient had autoimmune disease, 1 patient had been taking statin long-term, and the other patient had no clear cause of disease. Results of hormone therapy varied; 2 patients received gamma globulin therapy, and 1 patient received immunization inhibitor treatment. Conclusion: The clinical manifestations of idiopathic inflammatory myopathy with anti-HMGCR/anti-SRP antibody are different. EMG can only be used to assess muscle damage, and muscle biopsy and myositis autoantibodies antibody are more helpful for diagnosis and determination of myopathy type. The curative effect varies from type to type.
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