抗黑色素瘤分化相关基因5抗体阳性皮肌炎患者早期肺间质改变对其预后的影响及分析  

The impact of early inte rstitial pneumonia on the prognosis of patients with anti-melanoma differentiation-associated gene 5 antibody positive dermatomyositis

作　　者：石慧婧 于萍[1] 胡玉琴 杨文芳 李健[2] 崔刘福[1] 舒荣[1] 宋海澄[1] 高利常[3] 王洁蕊[1] Shi Huijing;Yu Ping;Hu Yuqin;Yang Wenfang;Li Jian;Cui Liufu;Shu Rong;Song Haicheng;Gao Lichang;Wang Jierui(Department of Rheumatology,Kailuan General Hospital,Tangshan 063000,China;CT Scan Room,Kailuan General Hospital,Tangshan 063000,China;Department of Laboratory,Kailuan General Hospital,Tangshan 063000,China)

机构地区：[1]开滦总医院风湿免疫科,唐山063000 [2]开滦总医院CT室,唐山063000 [3]开滦总医院检验科,唐山063000

出　　处：《中华风湿病学杂志》2021年第5期316-322,共7页Chinese Journal of Rheumatology

基　　金：河北省医学科学研究课题(20201284)。

摘　　要：目的采用前瞻性队列研究分析抗黑色素瘤分化相关基因5(MAD5)抗体阳性DM患者早期肺间质改变对其临床预后的影响及分析。方法选取2017年9月至2019年12月开滦总医院收治的DM的患者为研究对象,包括13例抗MAD5抗体阳性DM患者(7例幸存,6例死亡)和18例抗合成酶(ARS)抗体阳性DM患者,对2组患者的首次肺CT结果及临床表现进行比较,采用校正年龄和性别的Cox回归分析确定预后因素。结果与抗ARS抗体阳性患者比较,抗MAD5抗体阳性组急性/亚急性间质性肺炎(A/SIP)的发生率及病死率显著增高[发生率(69%和0%,χ^(2)=17.7,P<0.01),病死率(46%和0%,χ^(2)=10.3,P=0.002]。与ARS抗体阳性患者比较,抗MAD5抗体组γ-谷氨酰转移酶(GGT)和铁蛋白值显著增高[70.0(37.0,122.5)和21.0(16.5,33.5),Z=-3.37,P=0.001;977.0(502.5,1366.0)和307.1(72.3,546.9),Z=-3.44,P=0.001]。抗MAD5抗体阳性组中A/SIP组较缓慢渐进间质性肺炎(CIP)组累积生存率显著降低(100%和70%,P=0.001)。Cox回归分析提示肺CT评分与抗MDA5抗体阳性患者的疾病预后不相关,GGO总分与纤维化总分的[HR=1.08,95%CI(0.95,1.23),P=0.229;HR=0.97,95%CI(0.72,1.30),P=0.814]。结论抗MDA5抗体是早期诊断DM合并A/SIP的有力指标。早期肺CT评分并不能预测抗MDA5抗体阳性患者的疾病严重程度及预后。Objective To explore the association between chest high resolution CT(HRCT)scoring and prognostic factors of interstitial lung disease(ILD)in patients with anti-melanoma differentiation-associated gene 5(MDA5)antibody-positive dermatomyositis(DM).Methods The patients with DM admitted to Kailuan General Hospital between January 2017 and December 2019 were included into the study including 13 patients with positiveanti-MDA5 antibody(7 survivors,6 deaths)and 18 patients with anti-synthase(ARS)-antibody positive.All patients underwent chest HRCT prior to treatment.The consolidation,ground-glass opacity(GGO)and fibrosis were scored to assess HRCT findings.The clinical manifestations were compared between the two groups.Cox regression analysis adjusted for age and sex was used to determine the prognostic factors for anti-MDA5 antibody-related ILD.Results Compared with ARS patients,glutamyl transferase(GGT)and ferritin levels were significantly higher in MDA5-ILD patients[70.0(37.0,122.5)vs 21.0(16.5,33.5),Z=-3.37,P=0.001;977.0(502.5,1366.0)vs 307.1(72.3,546.9),Z=-3.44,P=0.001].The cumulative survival rate was significantly lower in patients with positive anti-MDA5 antibody than in those with positive anti-ARS antibody(100%vs 70%,P=0.001).The DM complicated with acute/subacute interstitial pneumonia(A/SIP)were found to significantly relate to death.There were no significant differences in chest HRCT scoringbetween the survivors and the deceased patients[HR=1.08,95%CI(0.95,1.23),P=0.229;HR=0.97,95%CI(0.72,1.30),P=0.814].Conclusion Anti-MDA5 antibody is an important index for early diagnosis of DM complicated with acute/subacute interstitial pneumonia(A/SIP).The chest HRCT scoreis is not associated with the prognosis of anti-MDA5 antibody-related ILD patients.

关 键 词：皮肌炎 间质性肺疾病 黑色素瘤分化相关基因5 铁蛋白 

分 类 号：R563[医药卫生—呼吸系统]