抗黑色素瘤分化相关蛋白-5抗体阳性炎性肌病临床特征分析  

Clinical characteristics of idiopathic inflammatory myopathy patients with positive anti-melanoma differentiation-associated gene 5 antibody

作　　者：武雅楠 王颖嫒[1] 祁福敏 王若明 王高亚 徐泳 张娜[1] 侯候[1] 孙文闻[1] 李昕[1] 魏蔚[1] Wu Ya'nan;Wang Ying'ai;Qi Fumin;Wang Ruoming;Wang Gaoya;Xu Yong;Zhang Na;Hou Hou;Sun Wenwen;Li Xin;Wei Wei(Departments of Rheumatology and Immunology,Tianjin Medical University General Hospital,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院风湿免疫科,天津300052

出　　处：《中华风湿病学杂志》2022年第2期105-114,I0003,共11页Chinese Journal of Rheumatology

摘　　要：目的探讨抗黑色素瘤分化相关蛋白-5(MDA5)抗体阳性的特发性炎性肌病(IIM)患者临床特征及预后情况。方法收集2015年1月至2020年9月于天津医科大学总医院风湿免疫科住院治疗的IIM患者共194例,其中抗MDA5抗体阳性组29例,抗MDA5抗体阴性组165例,回顾性分析其临床资料。正态分布的计量资料采用t检验,非正态分布的计量资料采用Mann-Whitney U秩和检验,计数资料采用χ^(2)检验。危险因素分析采用二元Logistic回归,生存分析采用Kaplan-Meier法和Cox回归分析。结果抗MDA5抗体阳性患者DM特异性皮疹发生率高且皮疹为最常见首发症状,肌肉症状轻,易出现发热、关节炎、口腔溃疡、体质量下降,患者全部合并肺间质病变(ILD)。与抗MDA5抗体阴性组相比,抗MDA5抗体阳性组患者白细胞计数[7.59(5.61,9.89)×10^(9)/L和4.07(3.17,5.50)×10^(9)/L,Z=-5.05,P<0.001]、血小板计数[249.00(200.00,302.00)×10^(9)/L和205.00(178.00,244.00)×10^(9)/L,Z=-2.59,P=0.010]、淋巴细胞计数(LY)[1.34(0.85,1.94)×10^(9)/L和0.64(0.40,0.83)×10^(9)/L,Z=-5.78,P<0.001]、肌酸激酶(CK)[558.00(72.00,2959.00)U/L和64.00(35.00,149.50)U/L,Z=-3.97,P<0.001]、CK-MB[38.00(17.00,127.00)U/L和16.00(14.00,25.00)U/L,Z=-3.84,P<0.001]、肌红蛋白(MYO)[243.65(60.50,829.83)ng/ml和34.55(21.00,104.23)ng/ml,Z=-3.98,P<0.001]、肌钙蛋白T(TnT)[0.09(0.03,0.44)ng/ml和0.02(0.01,0.04)ng/ml,Z=-4.17,P<0.001]、白蛋白[34.00(30.00,38.00)g/L和31.00(26.50,36.00)g/L,Z=-2.68,P=0.007]、CD4+T细胞[498.00(276.00,752.00)/μl和259.50(179.00,498.25)/μl,Z=-2.79,P=0.005]、动脉血二氧化碳分压(PaCO2)[39.00(36.13,42.00)mmHg和35.35(31.30,38.88)mmHg,Z=-3.75,P<0.001]、血氧分压(PaO2)[82.00(71.90,90.20)mmHg和73.25(64.30,84.05)mmHg,Z=-2.08,P=0.037]、动脉血氧饱和度(SaO2)[96.50%(95.05%,97.30%)和95.80%(93.70%,96.55%),Z=-2.11,P=0.035]、肺一氧化碳弥散量(DLco)[(63±21)%和(52±14)%,t=0.96,P=0.006]显著减低,尿蛋白定量(24 h)[260.50(172.25,401.25)g和331.00(252.75Objective To investigate the clinical characteristics and prognosis of idiopathic inflammatory myopathy(IIM)patients with positive anti-melanoma differentiation-associated gene 5(MDA5)antibody.Methods A total of 194 hospitalized IIM patients who were tested for myositis-specific autoantibodies(MSAs)in the Departments of Rheumatology and Immunology of Tianjin Medical University General Hospital from January 2015 to September 2020 were collected,including 29 cases with positive anti-MDA5 antibody and 165 cases with negative anti-MDA5 antibody.Their clinical data were analyzed retrospectively.T test was used for measurement data with normal distribution.Measurement data with non-normal distribution were tested by Mann-Whitney U rank sum test.χ^(2) test was used for counting data.Risk factors were analyzed by binary Logistic regression,survival analysis by Kaplan-Meier method and Cox regression analysis.Results IIM patients with positive anti-MDA5 antibody had a high incidence of dermatomyositis specific skin rash,and the skin rash was the most common presenting symptom.In the positive anti-MDA5 antibody group,muscle symptoms were mild;and the patients were prone to have fever,arthritis,oral ulcer and weight loss.All patients were complicated with interstitial lung disease(ILD).In patients with negative anti-MDA5 antibody,white blood cell(WBC)count[7.59(5.61,9.89)×10^(9)/L vs 4.07(3.17,5.50×10^(9)/L,Z=-5.05,P<0.001],platelet(PLT)[249.00(200.00,302.00)×10^(9)/L vs 205.00(178.00,244.00)×10^(9)/L,Z=-2.59,P=0.010],lymphocyte(LY)[1.34(0.85,1.94)×10^(9)/L vs 0.64(0.40,0.83)×10^(9)/L,Z=-5.78,P<0.001),serum creatine kinase(CK)[558.00(72.00,2959.00)U/L vs 64.00(35.00,149.50)U/L,Z=-3.97,P<0.001],creatine kinase isoenzymes(CK-MB)[38.00(17.00,127.00)U/L vs 16.00(14.00,25.00)U/L,Z=-3.84,P<0.001],myoglobin(MYO)[243.65(60.50,829.83)ng/ml vs 34.55(21.00,104.23)ng/ml,Z=-3.98,P<0.001],troponin T(TnT)[0.09(0.03,0.44)ng/ml vs 0.02(0.01,0.04)ng/ml,Z=-4.17,P<0.001],albumin(ALB)[34.00(30.00,38.00)g/L vs 31.00(26.50,36.00)g/L,Z=-2.68

关 键 词：皮肌炎 抗黑色素瘤分化相关基因-5抗体 肺疾病 间质性 

分 类 号：R593.26[医药卫生—内科学]