抗黑素瘤分化相关基因5抗体...体状态与临床特点相关性分析_王方明.pdf

9创伤与急危重病医学 2023 年 1 月 第 11 卷 第 1 期Trauma and Critical Care Medicine Jan.2023 Vol.11，No.1 论 著 抗黑素瘤分化相关基因 5 抗体阳性重症皮肌炎患者 抗转录中介因子 1 抗体状态与 临床特点相关性分析王方明，刘敏黎，汤 茜，宋冬明，孙 建江南大学附属医院风湿免疫科，江苏无锡214000摘要 目的 探讨抗黑素瘤分化相关基因 5（MDA5）抗体阳性重症皮肌炎患者抗转录中介因子 1（TIF1）抗体状态与临床特点的相关性。方法 选取自 2018 年 1 月至 2022 年 1 月收治的重症皮肌炎患者 62 例为研究对象。检测 62 例重症皮肌炎患者的抗 MDA5 抗体和抗 TIF1 抗体状态。分析抗 MDA5 抗体阳性重症皮肌炎患者抗 TIF1 抗体状态与临床特点的关系。Log-rank 检验分析抗 MDA5 抗体水平与抗 TIF1 抗体状态对预后的影响。以抗 MDA5 抗体浓度的中位数为阈值，划分为弱抗 MDA5 抗体水平和强抗 MDA5 抗体水平，分析不同抗 MDA5 抗体水平的重症皮肌炎患者中抗 TIF1 抗体状态与临床症状的关系。结果 抗 TIF1 抗体阳性重症皮肌炎患者的铁蛋白水平、人肌酸激酶水平、人乳酸脱氢酶水平、C 反应蛋白水平、红细胞沉降率均高于抗 TIF1 抗体阴性重症皮肌炎患者，差异有统计学意义（P0.05）；但在强抗 MDA5 抗体水平的重症皮肌炎患者中，抗 TIF1 抗体阳性患者显著多于抗 TIF1 抗体阴性患者，差异有统计学意义（P0.05）。Log-rank 检验结果显示，存活率从低到高依次为强抗 MDA5 抗体阳性+抗 TIF1 抗体阳性 60.0%（15/25）、强抗 MDA5 抗体阳性+抗TIF1 抗体阴性 66.7%（4/6）、弱抗 MDA5 抗体阳性+抗 TIF1 抗体阳性 87.5%（14/16）、弱抗 MDA5 抗体阳性+抗 TIF1 抗体阴性 93.3%（14/15），差异有统计学意义（P0.05）。重症皮肌炎患者临床表现中，抗 MDA5 抗体阳性伴抗TIF1 抗体阳性患者皮肤溃疡、声嘶、快速进展性间质性肺病（RP-ILD）所占比例明显高于抗 MDA5 抗体阳性伴抗 TIF1抗体阴性患者，差异有统计学意义（P0.05）。结论 抗TIF1抗体在抗MDA5抗体阳性的重症皮肌炎患者中的阳性率较高，且抗 MDA5 抗体阳性伴抗 TIF1 抗体阳性与皮肤溃疡、声嘶、RP-ILD 相关。结合抗 MDA5 抗体水平和抗 TIF1 抗体状态有助于评估患者预后。关键词 抗黑素瘤分化相关基因 5 抗体；抗转录中介因子 1 抗体；重症；皮肌炎 中图分类号：R593 DOI：10.16048j.issn.2095-5561.2023.01.02 文章编号：2095-5561（2023）01-0009-06Analysis of clinical characteristics of patients with severe dermatomyositis with positive anti-melanoma differentiation-associated gene 5 antibody and anti-transcriptional intermediary factor 1 gamma antibodyWANG Fang-ming，LIU Min-li，TANG Qian，SONG Dong-ming，SUN Jian（Department of Rheumatology and Immunology，Affiliated Hospital of Jiangnan University，Wuxi 214000，China）Abstract：Objective To investigate the correlation between anti-transcriptional intermediary factor 1 gamma（TIF1）antibody status and clinical features in patients with anti-melanoma differentiation-associated gene 5（MDA5）antibody-positive severe dermatomyositis.Methods Sixty-two patients with severe dermatomyositis who were admitted from January 2018 to January 2022 were selected for the study.The anti-MDA5 antibody and anti-TIF1 antibody status of 62 patients with severe dermatomyositis were tested.The relationship between anti-MDA5 antibody positive patients with severe dermatomyositis and the analysis of clinical characteristics was analyzed.Log-rank test was used to analyze the effect of anti-MDA5 antibody level and anti-TIF1 antibody status on prognosis.The median of anti-MDA5 antibody concentration was used as the threshold to classify the weak anti-MDA5 antibody level and the strong anti-MDA5 antibody level，and the relationship between anti-TIF1 antibody status and clinical symptoms in patients with severe dermatomyositis with different anti-MDA5 antibody levels was analyzed.Results Ferritin 基金项目：无锡市卫生健康委员会项目（Q20198）；江南大学附属医院临床研究与转化医学研究项目（LCYJ202213）第一作者：王方明（1984-），女，山东潍坊人，主治医师，硕士通信作者：孙 建，E-mail：10创伤与急危重病医学 2023 年 1 月 第 11 卷 第 1 期Trauma and Critical Care Medicine Jan.2023 Vol.11，No.1levels，creatine kinase levels，lactatedehydrogenase levels，C-reactiveprotein levels，anderythrocyte sedimentation rate were higher in patients with anti-TIF1 antibody-positive severe dermatomyositis than in patients with anti-TIF1 antibody-negative severe dermatomyositis，and the differences were statistically significant（P0.05）.However，in patients with severe dermatomyositis with strong anti-MDA5 antibody levels，there were significantly more anti-TIF1 antibody-positive patients than anti-TIF1 antibody-negative patients，and the difference was statistically significant（P0.05）.Log-rank test showed that the survival rate from low to high was strong anti-MDA5 antibody positive+anti-TIF1 antibody positive60.0%（15/25），strong anti-MDA5 antibody positive+anti-TIF1 antibody negative66.67%（4/6），weak anti-MDA5 antibody positive+anti-TIF1 antibody positive87.5%（14/16），weak anti-MDA5 antibody positive+anti-TIF1 antibody negative 93.3%（14/15），with statistically significant differences（P0.05）.Among the clinical manifestations of patients with severe dermatomyositis，the proportion skin ulcers，hoarseness，and rapidly progressive interstitial lung disease（RP-ILD）in patients with positive anti-MDA5 antibody and anti-TIF1 antibody were significantly higher than those in positive anti-MDA5 antibodies with negative anti-TIF1 antibodies，the differences were statistically significant（P0.05）.Conclusion Anti-TIF1 antibody positivity was higher in patients with anti-MDA5 antibody-positive severe dermatomyositis，and anti-MDA5 antibody positivity with anti-TIF1 antibody positivity was associated with skin ulcers，hoarseness，and RP-ILD.The combination of anti-MDA5 antibody levels and anti-TIF1 antibody status helps to assess patient prognosis.Key words：Anti-melanoma differentiation-associated gene5 antibody；Anti-transcriptional intermediary factor 1 gamma antibody；Severe disease；Dermatomyositis抗黑素瘤分化相关因子5（melanoma differentiation-associated gene5，MDA5）抗体皮肌炎是一种罕见的系统性自身免疫疾病1。抗 MDA5 抗体最初在皮肌炎的一个特定亚群中被确认，这种亚群被命名为临床淀粉病性皮肌炎（combination of clinically amyopathic dermatomyositis，CADM），其 与 快 速 进展性间质性肺病（ra