摘要
目的 分析抗黑素瘤分化相关基因5(MDA5)抗体阳性皮肌炎(MDA5-DM)临床特征,为早期诊断和早期治疗提供依据.方法 入组2019年3月至2021年6月南京医科大学炎性肌病相关肺间质病变队列MDA5-DM患者272例,以76例抗合成酶抗体综合征(ASS)患者为对照,分析其临床特征及肺间质病变发生情况.对于正态分布且方差齐的两组独立样本采用t检验比较均数,非正态分布计量资料采用Mann-Whitney U检验比较,对二分类变量资料使用x2检验或Fisher确切概率法进行比较.结果 在272例MDA5-DM患者中,88.6％(241/272)患者发生间质性肺疾病(ILD),33.8％(92/272)患者出现快速进展性ILD(RP-ILD).MDA5-DM患者6个月全因病死率为16.9％(46/272),伴发RP-ILD患者6个月全因病死率高达47.8％(44/92).与ASS患者相比,MDA5-DM患者男性和关节炎比例、Gottron征、向阳疹、V字征、甲周红斑和皮肤溃疡比例明显高于ASS组(P＜0.05),ALT、AST和铁蛋白显著升高(P＜0.05).与非 RP-ILD 患者相比,RP-ILD 患者男性比例[35.9％(33/92)和 23.3％(42/180),x2=4.79,P=0.029]、LDH[387(276,547)U/L 和 310(245,400)U/L,Z=-3.67,P＜0.001]、ESR[45.5(29.25,63.25)mm/1 h 和 31.2(20,51)mm/1 h,Z=-3.71,P＜0.001]、CRP[10.9(4.1,25.2)mg/L 和 4.54(2.58,9.08)mg/L,Z=-4.97,P＜0.001]和铁蛋白水平[1 340(650,2 000)ng/ml 和 556(203,1 186)ng/ml,Z=-4.40,P＜0.001]、抗 Ro52 抗体和抗 MDA5 抗体双阳性的比例[87.0％(80/92)和 52.2％(94/180),x2=31.87,P＜0.001]显著增加.结论 MDA5-DM患者易出现RP-ILD,预后差.
Abstract
Objective To analyze the clinical features of MDA5 antibody positive dermatomyositis(MDA5-DM)and to provide evidence for early diagnosis and treatment.Methods From March 2019 to June 2021,272 patients with anti-MDA5-DM from the Nanjing Medical University myositis-associated interstitial lung disease cohort were enrolled,with 76 patients with anti-synthetase syndrome(ASS)as the control group.The clinical characteristics and the occurrence of interstitial lung disease were analyzed.T-test was used for normally distributed and variance-homogeneous independent samples,Mann-Whitney U test for non-normally distributed data,and chi-square test or Fisher's exact test for dichotomous variables.Results Among the 272 anti-MDA5-DM patients,88.6％(241/272)developed interstitial lung disease(ILD),and 33.8％(92/272)developed rapidly progressive ILD(RP-ILD).The six-month all-cause mortality rate of anti-MDA5-DM patients was 16.9％(46/272),and it was as high as 47.8％(44/92)for those with RP-ILD.Compared with ASS patients,anti-MDA5-DM patients had a significantly higher proportion of males,arthritis,Gottron's sign,heliotrope rash,V-sign,periungual erythema,and skin ulcers(P＜0.05).The levels of ALT,AST,and ferritin were significantly increased(P＜0.05).Compared with non-RP-ILD patients,RP-ILD patients had a significantly higher proportion of males[35.9％(33/92)vs.23.3％(42/180),x2=4.79,P=0.029],higher levels of LDH[387(276,547)U/L vs.310(245,400)U/L,Z=-3.67,P＜0.001],ESR[45.5(29.25,63.25)mm/1 h vs.31.2(20,51)mm/1 h,Z=-3.71,P＜0.001],CRP[10.9(4.1,25.2)mg/L vs.4.54(2.58,9.08)mg/L,Z=-4.97,P＜0.001],ferritin[1 340(650,2 000)ng/ml vs.556(203,1 186)ng/ml,Z=-4.40,P＜0.001],and a higher proportion of anti-Ro52 antibody and anti-MDA5 antibody co-positivity[87.0％(80/92)vs.52.2％(94/1 80),x2=31.87,P＜0.001].Conclusion Anti-MDA5-DM patients are prone to develop RP-ILD and have poor prognosis.
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