男性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预后分析  

Clinical characteristics and prognosis of male dermatomyositis patients with positive anti-melanoma differentiation associated gene 5 antibody

作　　者：时一添 袁风红 刘婷 谈文峰[2] 李菊 吴敏 达展云[5] 魏华 周磊 殷松楼 武剑[9] 陆燕[10] 苏定雷 刘志纯 刘琳 马龙新 徐晓[15] 臧银善 刘惠杰 任天丽[18] Shi Yitian;Yuan Fenghong;Liu Ting;Tan Wenfeng;Li Ju;Wu Min;Da Zhanyun;Wei Hua;Zhou Lei;Yin Songlou;Wu Jian;Lu Yan;Su Dinglei;Liu Zhichun;Liu Lin;Ma Longxin;Xu Xiaoyan;Zang Yinshan;Liu Huijie;Ren Tianli(Department of Rheumatology&Immunology,The Affiliated Wuxi People′s Hospital of Nanjing Medical University,Wuxi 214000,China;Department of Rheumatology,The First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China;Department of Rheumatology and Immunology,Huai’an First People’s Hospital,Huai′an 223300,China;Department of Rheumatology and Immunology,The First People′s Hospital of Changzhou,Changzhou 213003,China;Department of Rheumatology and Immunology,Affiliated Hospital of Nantong University,Nantong 226006,China;Department of Rheumatology and Immunology,Northern Jiangsu People′s Hospital,Yangzhou 225001,China;Department of Rheumatology and Immunology,Changzhou No.2 People′s Hospital,Changzhou 213004,China;Department of Rheumatology and Immunology,The Affiliated Hospital of Xuzhou Medical University,Xuzhou 221004,China;Department of Rheumatology and Immunology,The First Affiliated Hospital of Soochow University,Suzhou 215006,China;Department of Rheumatology and Immunology,Jiangsu Province Hospital of Chinese Medicine,Nanjing 214004,China;Department of Rheumatology and Immunology,Nanjing First Hospital,Nanjing 210006,China;Department of Rheumatology and Immunology,The Second Affiliated Hospital of Soochow University,Suzhou 215004,China;Department of Rheumatology and Immunology,Xuzhou Central Hospital,Xuzhou 221009,China;Department of Rheumatology and Immunology,Yancheng No.1 People’s Hospital,Yancheng 224006,China;Department of Rheumatology and Immunology,Zhongda Hospital Southeast University,Nanjing 210009,China;Department of Rheumatology and Immunology,The Affiliated Suqian First People′s Hospital of Nanjing Medical University,Suqian 223812,China;Department of Rheumatology and Immunology,the First People′s Hospital of Lianyungang,Lianyungang 222002,China;Department of Rheumat

机构地区：[1]南京医科大学附属无锡人民医院风湿免疫科,南京214023 [2]南京医科大学附属第一人民医院风湿免疫科,南京210029 [3]淮安第一人民医院风湿免疫科,淮安223300 [4]常州第一人民医院风湿免疫科,常州213003 [5]南通大学附属一院风湿免疫科,南通226006 [6]苏北人民医院风湿免疫科,扬州225001 [7]常州第二人民医院风湿免疫科,常州213004 [8]徐州医科大学附属医院风湿免疫科,徐州221004 [9]苏州大学附属第一人民医院风湿免疫科,苏州215006 [10]江苏省中医院风湿免疫科,南京214004 [11]南京第一人民医院风湿免疫科,南京210006 [12]苏州大学附属第二人民医院风湿免疫科,苏州215004 [13]徐州中心医院风湿免疫科,徐州221009 [14]盐城第一人民医院风湿免疫科,盐城224006 [15]东南大学中大医院风湿免疫科,南京210009 [16]南京医科大学附属宿迁第一人民医院风湿免疫科,宿迁223812 [17]连云港第一人民医院风湿免疫科,连云港222002 [18]无锡市第二人民医院风湿免疫科,无锡214001

出　　处：《中华风湿病学杂志》2024年第1期44-49,I0009,共7页Chinese Journal of Rheumatology

摘　　要：目的探讨男性抗黑色素瘤分化相关基因5(MDA5)抗体阳性皮肌炎患者临床特征及预后不良因素。方法回顾性分析江苏省肌炎协作组2017—2020年间住院246例抗MDA5抗体阳性的皮肌炎患者的临床资料。计数资料组间比较采取χ^(2)检验;定量资料采用M(Q1,Q3)表示,组间比较采用秩和检验;单因素生存分析采用Kaplan-Meier法和Log-rank检验;多因素生存分析采用Cox比例风险模型;以P<0.05为差异有统计学意义。结果①男性抗MDA5抗体阳性患者向阳疹[67.1%(47/70)与52.8%(93/176),χ^(2)=4.18,P=0.041]、V字征比例高于女性组[50.0%(35/70)与30.7%(54/176),χ^(2)=8.09,P=0.004],男性组CK[112(18,981)U/ml与57(13.6,1433)U/ml,Z=-3.50,P<0.001]及血清铁蛋白(SF)水平[1500(166,32716)ng/ml与569(17.8,14839)ng/ml,Z=-5.85,P<0.001]高于女性组;男性组ESR[31.0(4.0,101.5)与43.4(5.0,126.5)mm/1 h,Z=-2.22,P=0.026]、间质性肺疾病(ILD)[40.0%(28/70)与59.7%(105/176),χ^(2)=7.82,P=0.020]患者的比例低于女性组,但快速进展型间质性肺疾病(PR-ILD)比例[47.1%(33/70)和31.3%(55/176),χ^(2)=5.51,P=0.019]高于女性组。②男性抗MDA5抗体阳性患者,死亡组较存活组病程更短[1.0(1.0,3.0)个月与2.5(0.5,84)个月,Z=-3.07,P=0.002],关节炎发生率更低[16.7%(4/24)与42.2%(19/45),χ^(2)=4.60,P=0.032];死亡组AST[64(22.1,565)U/L与51(14,601)U/L,Z=-2.42,P=0.016]、LDH[485(224,1464)U/L与352(170,1213)U/L,Z=-3.38,P=0.001]、CRP[11.6(2.9,61.7)mg/L与5.0(0.6,86.4)mg/L,Z=-1.96,P=0.050]、血清SF水平[2000(681,7676)ng/ml与1125(166,32716)ng/ml,Z=-3.18,P=0.001)高于存活组,RP-ILD发生率显著增高[95.8%(23/24)与22.2%(10/45),χ^(2)=33.99,P<0.001];③Cox回归分析提示病程、LDH水平、RP-ILD[HR值(95%CI)=0.203(0.077,0.534),P=0.001;HR值(95%CI)=1.00(1.001,1.004),P=0.003;HR值(95%CI)=95.67(10.872,841.904),P<0.001]是男性抗MDA5抗体阳性患者预后相关因素。结论男性抗MDA5抗体阳性患者临床表现与女性比有一定差异,ILD发生率低,但PR-ILD占比高,病程Objective To investigate the clinical features and prognosis of male with anti-melanoma differentiation-associated gene 5(MDA5)autoantibody.Methods The clinical data of 246 patients with DM and anti-MDA5 autoantibodies hospitalized by Jiangsu Myositis Cooperation Group from 2017 to 2020 were collected and retrospectively analyzed.Chi-square test was performed to compared between counting data groups;Quantitative data were expressed by M(Q1,Q3),and rank sum test was used for comparison between groups;Single factor survival analysis was performed by Kaplan-Meier method and Log rank test;Cox regression analysis were used for multivariate survival analysis.Results①The male group had a higher proportion of rash at the sun exposure area[67.1%(47/70)vs 52.8%(93/176),χ^(2)=4.18,P=0.041]and V-sign[50.0%(35/70)vs 30.7%(54/176),χ^(2)=8.09,P=0.004]than the female group.The male group had higher levels of creatine kinase[112(18,981)U/L vs 57(13.6,1433)U/L,Z=-3.50,P<0.001]and ferritin[1500(166,32716)ng/ml vs 569(18,14839)ng/ml,Z=-5.85,P<0.001]than the female group.The proportion of ILD[40.0%(28/70)vs 59.7%(105/176),χ^(2)=7.82,P=0.020]patients and the red blood cell sedimentation rate[31.0(4.0,101.5)mm/1 h vs 43.4(5.0,126.5)mm/1 h,Z=-2.22,P=0.026]in the male group was lower than that of the female group,but the proportion of rapidly progressive interstitial lung disease(PR-ILD)[47.1%(33/70)vs 31.3%(55/176),χ^(2)=5.51,P=0.019]was higher than that of the female group.②In male patients with positive anti-MDA5 antibodies,the death group had a shorter course of disease[1.0(1.0,3.0)month vs 2.5(0.5,84)month,Z=-3.07,P=0.002],the incidence of arthritis[16.7%(4/24)vs 42.2%(19/45),χ^(2)=4.60,P=0.032]were low than those in survival group,while aspartate aminotransferase(AST)[64(22.1,565)U/L vs 51(14,601)U/L,Z=-2.42,P=0.016],lactate dehydrogenase(LDH)[485(24,1464)U/L vs 352(170,1213)U/L,Z=-3.38,P=0.001],C-reactive protein(CRP)[11.6(2.9,61.7)mg/L vs 4.95(0.6,86.4)mg/L,Z=-1.96,P=0.050],and ferritin levels[2000(681,7676)vs 1125(166,3

关 键 词：皮肌炎 男人 预后 抗MDA5抗体 

分 类 号：R593.26[医药卫生—内科学]