| 程路,高文佳,徐艳.抗合成酶抗体综合征合并抗 3-羟基 -3-甲基戊二酰辅酶还原酶蛋白抗体阳性坏死性肌病 1例[J].安徽医药,2024,28(11):2269-2272. |
| 抗合成酶抗体综合征合并抗 3-羟基 -3-甲基戊二酰辅酶还原酶蛋白抗体阳性坏死性肌病 1例 |
| A case of anti-synthetase syndrome combined with anti-HMGCR antibody positive necrotizing myopathy |
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| DOI：10.3969/j.issn.1009-6469.2024.11.032 |
| 中文关键词: 肌病 抗合成酶抗体综合征 抗 3-羟基 -3-甲基戊二酰辅酶还原酶蛋白抗体 抗 Ro52抗体 皮肌炎 间质性肺病 |
| 英文关键词: Myopathy Anti-synthase syndrome Anti-HMGCR antibody Anti-Ro52 antibody Dermatomyositis Interstitial lung disease |
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| 中文摘要: |
| 目的总结抗合成酶抗体综合征(ASS)合并免疫介导坏死性肌病的疾病特点及治疗经验。方法回顾性分析 2022年 1月南京医科大学附属宿迁第一人民医院收治的 1例 ASS合并抗 3-羟基 -3-甲基戊二酰辅酶还原酶蛋白(HMGCR)抗体阳性坏死性肌病病人，总结此类病人的临床特点。结果病人主诉咳嗽 1周，查肌炎抗体谱:抗组氨酰 tRNA合成酶( Jo-1)抗体( +++)、抗 SSA/Ro52抗体( +++)、抗 HMGCR抗体( +++)，诊断:抗合成酶抗体综合征、抗 HMGCR抗体阳性坏死性肌病、间质性肺病、低蛋白血症。该病人通过糖皮质激素、免疫抑制剂及丙种球蛋白等治疗后症状缓解。结论不同类型的特发性炎性肌病( IIM)临床表现差异较大，各亚型之间也存在重叠表现，其预后及治疗各不相同。如有两种及以上肌炎的合并，则进展快，预后差，需早期完善肌炎抗体谱检查，明确分型，精准治疗。 |
| 英文摘要: |
| Objective To summarize the characteristics and treatment experience of anti-synthase syndrome(ASS) complicated with immune-mediated necrotizing myopathy.Methods A retrospective analysis of a patient with ASS combined with anti-3-hydroxy-3-methylglutaryl coenzyme reductase protein (HMGCR) antibody-positive necrotizing myopathy admitted to the Affiliated Suqian First People'sHospital of Nanjing Medical University in January 2022 was performed to summarize the clinical features of such patients.Results The patient complained of coughing for one week and checked myositis antibody profile:anti-Jo-1 antibody (+++ ), anti-SSA/Ro52 antibody (+++),anti-HMGCR antibody (+++),diagnosis: anti-synthetase syndrome,anti-HMGCR antibody positive necrotizing myopathy,interstitiallung disease, hypoproteinemia.The patient's symptoms resolved after treatment with glucocorticoids,immunosuppressants and gammaglobulin.Conclusions The clinical manifestations of different types of idiopathic inflammatory myopathy (IIM) vary greatly,and there isoverlap among subtypes.The prognosis and treatment are different.If there are two or more types of myositis combined,the progress is rapid and the prognosis is poor.It is necessary to improve the antibody spectrum of myositis early,clear typing,and precise treatment. |
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