中华风湿病学杂志2024，Vol.28Issue(1) ：44-49,后插8.DOI:10.3760/cma.j.cn141217-20211110-00457

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

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
中华风湿病学杂志2024，Vol.28Issue(1) ：44-49,后插8.DOI:10.3760/cma.j.cn141217-20211110-00457
✕
  • NETL
  • NSTL
  • 万方数据

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

时一添 1袁风红 1刘婷 1谈文峰 2李菊 3吴敏 4达展云 5魏华 6周磊 7殷松楼 8武剑 9陆燕 10苏定雷 11刘志纯 12刘琳 13马龙新 14徐晓龑 15臧银善 16刘惠杰 17任天丽18
扫码查看

作者信息

  • 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
  • 折叠

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.

Abstract

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.

关键词

皮肌炎/男人/预后/抗MDA5抗体

Key words

Dermatomyositis/Man/Prognosis/Anti-MDA5 antibody

引用本文复制引用

出版年

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
参考文献量20
段落导航相关论文