HIS评分在抗黑色素瘤分化相关基因5抗体阳性皮肌炎合并间质性肺疾病预后评估中的应用价值

Role of HIS score in predicting the prognosis of anti-melanoma differentiation associated gene 5 antibody-positive dermatomyositis patients with interstitial lung disease

目的探讨HIS(hyperinflammatory syndrome)评分在评估抗黑色素瘤分化相关基因5(MDA5)抗体阳性DM合并间质性肺病(ILD)患者预后中的作用。方法回顾性分析来源于江苏省人民医院风湿免疫科2018年1月至2021年4月43例抗MDA5抗体阳性皮肌炎(MDA5+DM)合并ILD的住院患者HIS评分, 以228例其他CTD相关的ILD作为对照。采用Mann-WhitneyU检验、χ^(2)检验、Fisher确切概率法、受试者工作特征曲线(ROC)等进行统计分析。结果入组MDA5+DM 43例, 228例对照组中, 抗合成酶抗体综合征(ASS)33例(14.5%), RA 44例(19.3%), SS 65例(28.5%), SSC 43例(18.9%), SLE 43例(18.9%)。MDA5+DM合并ILD组HIS评分[2(1, 3)]高于ASS-ILD组[1(0, 2), Z=-2.06, P<0.05], RA-ILD组[1(0, 2), Z=-2.87, P<0.01)], SS-ILD组[0(0, 1), Z=-5.78, P<0.01], SSC-ILD组[1(0, 1), Z=-3.84, P<0.01]和SLE-ILD组[1(0, 2), Z=-3.81, P<0.01]。在MDA5+DM合并ILD患者中, HIS评分低、中、高评分组3个月病死率分别为0(0/15)、38.1%(8/21)和85.7%(6/7), HIS评分预测MDA5+DM患者3个月病死率ROC曲线下面积(AUC)(95%CI)=0.857[(0.747, 0.967), P<0.001]。结论 MDA5+DM合并ILD患者HIS评分明显增高, 基线HIS评分水平与患者3个月的病死率相关。

Objective To investigate the role of HIS(hyperinflammatory syndrome)score in predicting the prognosis of anti-melanoma differentiation associated gene 5(MDA5)antibody-positive dermatomyositis(DM)patients with interstitial lung disease(ILD).Methods A total of 43 patients with anti-MDA5 antibody-positive dermatomyositis and 228 connective tissue disease(CTD)patients with ILD hospitalized in the First Affiliated Hospital of Nanjing Medical University from January 2018 to April 2021 were enrolled into this study.All patients were complicated with ILD and their HIS score were assessed.Non-parametric Mann-Whitney U test,Chi-squared test,Fisher exact probability and receiver operating characteristic(ROC)curve were used for data analysis.Results The HIS score of 43 patients with anti-MDA5 antibody-positive dermat-omyositis were collected.Among 228 CTD-ILD patients in the control groups,the primary disease consisted of 33(14.5%)anti-synthetase antibody syndrome(ASS),44(19.3%)rheumatoid arthritis(RA),65(28.5%)Sjögren's syndromes(SS),43(18.9%)systemic sclerosis(SSc)and 43(18.9%)systemic lupus erythematosus(SLE).The HIS score of anti-MDA5-positive DM-ILD patients[2(1,3)]was higher than those in ASS patients[1(0,2),Z=-2.06,P<0.05]and significantly higher than those in RA-ILD[1(0,2),Z=-2.87,P<0.01],SS-ILD[0(0,1),Z=-5.78,P<0.01],SSC-ILD[1(0,1),Z=-3.84,P<0.01]and SLE-ILD[1(0,2),Z=-3.81,P<0.01].Comparing HIS score of anti-MDA5-positive DM-ILD patients,the 3-months mortality rate in the low,medium and high groups was 0(0/15),38.1%(8/21)and 85.7%(6/7).The area under ROC curve(95%CI)was 0.857[(0.747,0.967),P<0.001]for HIS score in predicting 3-months mortality probability of anti-MDA5-positive DM-ILD.Conclusion HIS score of anti-MDA5-positive DM-ILD is higher than that in CTD-ILD patients and the baseline level is related to the 3-months mortality rate.