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  • 王文,高玲,徐艳,等.抗黑色素瘤分化相关基因5抗体阳性皮肌炎患者临床特征及生存分析[J].同济大学学报(医学版),2023,44(2):202-208.    [点击复制]
  • WANG Wen,GAO Ling,XU Yan,et al.Clinical characteristics and survival analysis of patients with anti-MDA5 positive dermatomyositis[J].同济大学学报(医学版),2023,44(2):202-208.   [点击复制]
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抗黑色素瘤分化相关基因5抗体阳性皮肌炎患者临床特征及生存分析
王文,高玲,徐艳,高文佳,臧银善
0
(南京医科大学附属宿迁第一人民医院风湿免疫科,江苏 宿迁223800)
摘要:
目的探讨抗黑色素瘤分化相关基因5抗体阳性皮肌炎(MDA5+DM)患者的临床特征及与生存时间的关系。方法回顾性分析2014年12月—2021年12月南京医科大学炎性肌病及结缔组织病相关间质性肺病专病联盟共249例抗MDA5阳性皮肌炎患者的临床资料,采用t检验、Mann-Whitney U检验、χ2检验分析死亡患者临床特征,应用Kaplan-Meier法计算累计生存率,Log-rank法比较生存率及COX模型法多因素分析。结果本组MDA5+DM患者死亡率为24.1%(60/249),死亡组男性比例、平均年龄高于非死亡组,实验室检查天冬氨酸氨基转移酶(glutamic oxaloacetic transaminase, AST)、乳酸脱氢酶(lactate dehydrogenase, LDH)、肌酸激酶(creatine kinase, CK)、C-反应蛋白(C-reactive protein, CRP)、血清铁蛋白(serum ferritin, SF)水平、抗Ro52抗体阳性率及高滴度抗MDA5抗体比例在死亡组中均明显升高,快速进展型间质性肺病(rapidly progressive interstitial lung disease, RPILD)发生率高于非死亡组,但关节炎发生率低于非死亡组,差异有统计学意义(P<0.05)。Log-rank单因素分析显示,抗MAD5抗体滴度、性别、年龄、LDH、CK、CRP、SF、抗Ro52抗体阳性、关节炎、皮疹、RPILD与MDA5+DM患者的生存情况相关(P<0.05);其中,年龄、CK、CRP、关节炎与MDA5+DM伴RPILD患者的生存期相关(P<0.05)。COX多因素分析提示,CK、CRP升高及抗Ro52抗体阳性是MDA5+DM预后的独立危险因素(P<0.05);CK、CRP升高是MDA5+DM伴RPILD亚组预后的独立危险因素(P<0.05),而关节炎是预后良好因素(HR=0.427,P<0.05)。结论MDA5+DM患者死亡率较高,伴RPILD的患者生存期更短,CK、CRP升高及抗Ro52抗体阳性可能增加死亡风险,而关节炎的存在可能是预后良好因素。
关键词:  皮肌炎  抗黑色素瘤分化相关基因5抗体  临床特征  生存分析
DOI:10.12289/j.issn.1008-0392.22312
投稿时间:2022-07-28
基金项目:
Clinical characteristics and survival analysis of patients with anti-MDA5 positive dermatomyositis
WANG Wen,GAO Ling,XU Yan,GAO Wenjia,ZANG Yinshan
(Department of Rheumatology and Immunology, the Affiliated Suqian First People’s Hospital of Nanjing Medical University, Suqian 223800, Jiangsu Province, China)
Abstract:
ObjectiveTo investigate the clinical features and survival of patients with anti-melanoma differentiation-associated gene 5 positive dermatomyositis(MDA5+DM). MethodsThe clinical data of 249 MDA5+DM patients, obtained from the Inflammatory Myopathy and Connective Tissue Disease-Associated Interstitial Lung Disease Alliance of Nanjing Medical University between December 2014 and December 2021, were retrospectively analyzed. Kaplan-Meier method was used to calculate the cumulative survival rate, univariate log-rank method and multivariate COX model was used to analyze the factors related to the survival of patients. ResultsThe fatality rate of MDA5+DM patients in this series was 24.1%(60/249). The proportion of males and average age in the death group were higher than those in the non-death group. The levels of AST, LDH, CK, CRP and SF, the positive rate of anti-Ro52 antibody and the proportion of high-titer anti-MDA5 antibody were significantly higher in the death group. The incidence of rapidly progressive interstitial lung disease(RPILD) was higher than that of non-death group, but the incidence of arthritis was lower than that of non-death group(P<0.05). Log-rank univariate analysis showed that anti-MAD5 antibody titer, sex, age, LDH, CK, CRP, SF, positive anti-Ro52 antibody, arthritis, rash, RPILD were significantly associated with survival of MDA5+DM patients(P<0.05); age, CK, CRP and arthritis were correlated with survival of univariate patients with RPILD(P<0.05). COX multivariate analysis showed that increased CK, CRP and positive anti-Ro52 antibody were independent risk factors for poor prognosis of MDA5+DM patients(P<0.05); the elevated CK and CRP were independent risk factors for poor prognosis of MDA5+DM combined with RPILD(P<0.05), while arthritis was a factor for favorable prognosis(HR=0.427, P<0.05). ConclusionMDA5+DM patients have high fatality, and patients with RPILD have shorter survival. Increased CK, CRP and positive anti-Ro52 antibody may increase the risk of poor diagnosis, while the presence of arthritis may be a factor of favorable prognosis.
Key words:  dermatomyositis  anti-melanoma differentiation-associated gene 5 antibody  clinical features  survival analysis

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