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第43卷第4期                           南京医科大学学报(自然科学版)
                  2023年4月                   Journal of Nanjing Medical University(Natural Sciences)     ·525 ·


               ·临床研究·

                以肺部症状为首发表现的 MDA5 抗体阳性皮肌炎的临床特征

                与预后分析



                李宗广 ,俞飞超 ,吴超杰 ,陶            媛 ,黄 茂 ,孙        伟 ,吉宁飞     1*
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                南京医科大学第一附属医院呼吸与危重症医学科,江苏                      南京   210029;无锡市锡山人民医院呼吸与危重症医学科,江苏
                1                                                         2
                无锡 214105
               [摘   要] 目的:分析以肺部症状为首发表现的黑色素瘤分化相关基因蛋白 5(melanoma differentiation⁃associated gene 5,
                MDA5)抗体阳性皮肌炎(dermatomyositis,DM)患者的临床特征与预后。方法:回顾性选取2017年8月—2021年10月南京医科
                大学第一附属医院收治的MDA5抗体阳性DM患者57例为研究对象,根据临床首发表现分为肺部首发组(n=15)和非肺部首发
                组(n=42),比较两组一般临床资料、实验室检查结果、影像学表现和预后情况。结果:肺部首发组患者血清铁蛋白(serum ferritin,
                SF)、C反应蛋白(C⁃reactive protein,CRP)水平明显高于非肺部首发组,肌酸激酶(creatine kinase,CK)水平明显低于非肺部首发
                组(P < 0.05)。肺部首发组患者的胸部CT征象以斑片状影为主,其次为磨玻璃影、网格影、实变影,小叶间隔增厚、结节影、蜂
                窝肺和胸腔积液等征象较少。多因素Logistic回归分析结果显示,SF、CK水平是MDA5抗体阳性DM患者以肺部症状为首发表
                现的独立危险因素(P < 0.05)。两组患者发病后6个月内的累积生存率比较,差异有统计学意义(P < 0.05)。结论:以肺部症
                状为首发表现的MDA5抗体阳性DM患者治疗方案以糖皮质激素+免疫抑制剂为主,患者预后较差,其临床表现及影像学特征
                具有多样性;SF、CK水平与MDA5抗体阳性DM患者以肺部症状为首发表现相关,或可成为实验室监测指标之一。
               [关键词] 皮肌炎;黑色素瘤分化相关基因蛋白5抗体;肺部症状;临床特征;预后
               [中图分类号] R593.26                  [文献标志码] A                       [文章编号] 1007⁃4368(2023)04⁃525⁃07
                doi:10.7655/NYDXBNS20230411



                Clinical features and prognosis of anti ⁃ MDA5 positive dermatomyositis with pulmonary
                symptom as the first manifestation

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                LI Zongguang ,YU Feichao ,WU Chaojie ,TAO Yuan ,HUANG Mao ,SUN Wei ,JI Ningfei 1*
                1 Department of Respiratory and Critical Care Medicine,the First Affiliated Hospital of Nanjing Medical University,
                Nanjing 210029;Department of Respiratory and Critical Care Medicine,Xishan People’s Hospital of Wuxi City,
                               2
                Wuxi 214105,China
               [Abstract] Objective:To analyze the clinical features and prognosis of patients with anti⁃melanoma differentiation⁃associated gene
                5(MDA5) positive dermatomyositis(DM) with pulmonary symptom as the first manifestation. Methods:The curren study
                retrospectively reviewed 57 patients with anti⁃MDA5 positive DM who were admitted to the First Affiliated Hospital of Nanjing Medical
                University between August 2017 and October 2021. The patients were divided into the pulmonary first symptom group(n=15)and the
                non ⁃ pulmonary first symptom group(n=42)according to the first clinical manifestation. The general clinical data,laboratory test
                results,imaging findings and prognosis were compared between the two groups. Results:There were statistically significant differences
                in therapeutic regimens between the two groups(P < 0.05). The levels of serum ferritin(SF)and C⁃reactive protein(CRP)in the
                pulmonary first symptom group were significantly higher than those in the non⁃pulmonary first symptom group,and the level of creatine
                kinase(CK)was significantly lower than that in the non⁃pulmonary first symptom group(P < 0.05). The main chest CT sign of patients
                in the pulmonary first symptom group was patchy shadow,followed by ground ⁃ glass opacity,thread net,and consolidation. Few



               [基金项目] 江苏省自然科学基金面上项目(BK20181497);无锡市卫生健康委科研课题(Z202113)
                ∗
                通信作者(Corresponding author),E⁃mail:sunweiwx@163.com,jiningfei@163.com
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