抗黑色素瘤分化相关蛋白5抗体阳性皮肌炎合并纵隔气肿的临床分析

Analysis of clinical characteristics of dermatomyositis patients with positive anti-melanoma differentiation-associated protein 5 antibody complicated with pneumomediastinum

目的探讨抗黑色素瘤分化相关蛋白5(MDA5)抗体阳性皮肌炎(DM)合并纵隔气肿的临床特征。方法收集2017年3月至2019年12月宁波市第一医院抗MDA5抗体阳性DM合并或不合并纵隔气肿患者的临床资料,复习国内外文献并与之比较。计量资料采用t检验或Mann-Whitney U检验,计数资料采用χ^2检验或Fisher确切概率法,Logistic回归分析抗MDA5抗体阳性DM合并纵隔气肿的危险因素。结果该院共收治有12例抗MDA5抗体阳性DM不合并纵隔气肿者,1例抗MDA5抗体阳性DM合并纵隔气肿者,结合文献复习资料详细的16例抗MDA5抗体阳性DM合并纵隔气肿者,2组间比较,纵隔气肿组血清铁蛋白(SF)水平[991.6(548.5,2875.1)ng/ml与355(143.5,395)ng/ml,Z=-2.506,P=0.012]、快速进展性肺间质病变(RPILD)发生率(76.5%与16.7%,χ^2=10.076,P=0.002)高于非纵隔气肿组。进一步Logistic回归分析并未发现男性[OR=0.192,95%CI(0.009,4.125),P=0.291]、SF[OR=1.002,95%CI(0.998,1.006),P=0.279]、RPILD(OR=0.084,95%CI(0.003,2.178),P=0.136)、临床无肌病性皮肌炎(CADM)[OR=0.258,95%CI(0.009,7.419),P=0.429]是抗MDA5抗体阳性DM合并纵隔气肿的危险因素。结论抗MDA5抗体阳性DM患者中,SF水平高、肺间质病变进展迅速的患者易合并纵隔气肿。

Objective To investigate the clinical characteristics of dermatomyositis(DM)patients with positive anti-melanoma differentiation-associated protein 5(MDA5)antibody-complicated with pneumomediastinum.Methods Clinical data of patients with anti-MDA5 antibody-positive DM with or without pneumomediastinum from March 2017 to December 2019 in Ningbo First Hospital were collected and analyzed.The international literature were reviewed and compared.T-test or Mann-Whitney U test was used for measurement data,chi-square test or Fisher exact probability was used for count data.Logistic regression analysis was used to analyze the risk factors for anti-MDA5 antibody-positive DM with pneumomediastinum.Results Twelve DM patients with-positive anti-MDA5 antibody without pneumomediastinum,and 1 DM patient with positive anti-MDA5 antibody-complicated with pneumomediastinum.Pooling with literature review,16 DM patients with positive anti-MDA5 antibody-complicated with pneumomediastinum were compared.It was found that the serum ferritin(SF)level[991.6(548.5,2875.1)ng/ml vs 355(143.5,395)ng/ml,Z=-2.506,P=0.012]and the rate of rapid progressive pulmonary interstitial disease(RPILD)in the pneumo-mediastinum group[76.5%vs 16.7%,χ^2=10.076,P=0.002]were significantly higher than those in the non-pneumome-diastinum group.Further Logi-stic regression analysis did not show male gender[OR=0.192,95%CI(0.009,4.125),P=0.291];SF[OR=1.002,95%CI(0.998,1.006),P=0.279];RPILD[OR=0.084,95%CI(0.003,2.178),P=0.136];CADM[OR=0.258,95%CI(0.009,7.419),P=0.429]was risk factor for pneumomediastinum.Conclusion DM patients with positive anti-MDA5 antibody and high seral SF level and rapid progression of pulmonary interstitial disease are more likely to complicate with pneumomediastinum.