首页|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

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

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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x2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),x2=4.18,P=0.041]、V字征比例高于女性组[50.0%(35/70)与 30.7%(54/176),x2=8.09,P=0.004],男性组 CK[112(18,981)U/ml 与 57(13.6,1 433)U/ml,Z=-3.50,P<0.001]及血清铁蛋白(SF)水平[1 500(166,32 716)ng/ml 与 569(17.8,14 839)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),x2=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),x2=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),x2=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(224,1 464)U/L 与 352(170,1 213)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[2 000(681,7 676)ng/ml 与 1 125(166,32 716)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),x2=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、LDH水平及RP-ILD是男性抗MDA5抗体阳性患者的预后不良的相关因素.
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.Results ① The male group had a higher proportion of rash at the sun exposure area[67.1%(47/70)vs 52.8%(93/176),x2=4.18,P=0.041]and V-sign[50.0%(35/70)vs 30.7%(54/176),x2=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(13.6,1 433)U/L,Z=-3.50,P<0.001]and ferritin[1 500(166,32 716)ng/ml vs 569(18,14 839)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),x2=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),x2=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),x2=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(24,1 464)U/L vs 352(170,1 213)U/L,Z=-3.38,P=0.001],C-reactive protein(CRP)[11.6(2.9,61.7)mg/Lvs 4.95(0.6,86.4)mg/L,Z=-1.96,P=0.050],and ferritin levels[2 000(681,7 676)vs 1 125(166,32 716)ng/ml,Z=-3.18,P=0.001]were higher than those in the survival group,and RP-ILD[95.8%(23/24)vs 22.2%(10/45),x2=33.99,P<0.001]occurred at a significantly higher rate.(3)Cox regression analysis indicated that the course of disease LDH level,and RP-ILD were related factors for the prognosis of male anti-MDA5 antibodies[HR(95%CI)=0.203(0.077,0.534),P=0.001;HR(95%CI)=1.002(1.001,1.004),P=0.003;HR(95%CI)=95.674(10.872,841.904),P<0.001].Conclusion The clinical manifestations of male anti-MDA5 antibody-positive patients are different from those of female.The incidence of ILD is low,but the proportion of PR-ILD is high.The course of disease,serum LDH level,and RP-ILD are prognostic factors of male anti-MDA5 antibody-positive patients.

DermatomyositisManPrognosisAnti-MDA5 antibody

时一添、袁风红、刘婷、谈文峰、李菊、吴敏、达展云、魏华、周磊、殷松楼、武剑、陆燕、苏定雷、刘志纯、刘琳、马龙新、徐晓龑、臧银善、刘惠杰、任天丽

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南京医科大学附属无锡人民医院风湿免疫科,南京 214023

南京医科大学附属第一人民医院风湿免疫科,南京 210029

淮安第一人民医院风湿免疫科,淮安 223300

常州第一人民医院风湿免疫科,常州 213003

南通大学附属一院风湿免疫科,南通 226006

苏北人民医院风湿免疫科,扬州 225001

常州第二人民医院风湿免疫科,常州 213004

徐州医科大学附属医院风湿免疫科,徐州 221004

苏州大学附属第一人民医院风湿免疫科,苏州 215006

江苏省中医院风湿免疫科,南京 214004

南京第一人民医院风湿免疫科,南京 210006

苏州大学附属第二人民医院风湿免疫科,苏州 215004

徐州中心医院风湿免疫科,徐州 221009

盐城第一人民医院风湿免疫科,盐城 224006

东南大学中大医院风湿免疫科,南京 210009

南京医科大学附属宿迁第一人民医院风湿免疫科,宿迁 223812

连云港第一人民医院风湿免疫科,连云港 222002

无锡市第二人民医院风湿免疫科,无锡 214001

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皮肌炎 男人 预后 抗MDA5抗体

2024

中华风湿病学杂志
中华医学会

中华风湿病学杂志

CSTPCD
影响因子:0.651
ISSN:1007-7480
年,卷(期):2024.28(1)
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