抗MDA5抗体阳性皮肌炎继发间质性肺疾病患者的临床特征和预后分析  

Clinical characteristics and prognosis of anti-MDA5 antibody positive dermatomyositis patients with secondary interstitial lung disease

作　　者：马宗伯 赵铖[2] 覃芳[2] 陈战瑞[2] 潘冬梅[2] 曾雯 廖晓玲 雷玲[2] MA Zongbo;ZHAO Cheng;QIN Fang;CHEN Zhanrui;PAN Dongmei;ZENG Wen;LIAO Xiaoling;LEI Ling(Department of Rheumatology and Immunology,the First People’s Hospital of Nanning,Nanning 530022,China;Department of Rheumatology and Immunology,the First Affiliated Hospital of Guangxi Medical University,Nanning 530021,China)

机构地区：[1]南宁市第一人民医院风湿免疫科,南宁530022 [2]广西医科大学第一附属医院风湿免疫科,南宁530021

出　　处：《广西医科大学学报》2024年第12期1628-1636,共9页Journal of Guangxi Medical University

基　　金：国家自然科学基金资助项目(No.82060300);广西自然科学基金资助项目(No.2023GXNSFDA026061);广西医疗卫生适宜技术开发与推广应用项目(No.S2022075)。

摘　　要：目的:分析抗黑色素瘤分化相关基因5(MDA5)阳性皮肌炎(DM)继发间质性肺疾病(ILD)患者的临床特征及预后影响因素。方法:回顾性分析2018年1月至2022年12月广西医科大学第一附属医院收治的97例DM-ILD患者的临床资料,分为抗MDA5阳性组与抗MDA5阴性组,比较两组临床特点、生存率,分析预后的影响因素。结果:与抗MDA5阴性组相比,抗MDA5阳性组病程短,出现胸闷、快速进展(RP)-ILD、肺部感染、皮肤溃疡的比例增高,肺动脉高压(PAH)比例下降,白细胞、血小板、肌酸激酶水平降低,血清铁蛋白水平升高(均P<0.05)。与抗MDA5阴性组相比,抗MDA5阳性组联用2种免疫抑制剂、使用丙种球蛋白及首次住院使用抗生素的比例增高(均P<0.05)。中位随访17.5月,抗MDA5阳性组总死亡率45.5%,6个月、12个月、36个月存活率分别为61.43%、58.57%、55.71%。Cox回归分析显示,RP-ILD、Velcro啰音、癌胚抗原(CEA)升高均为抗MDA5阳性ILD预后的危险因素(均P<0.05)。结论:抗MDA5阳性ILD患者病程较短,易出现皮肤溃疡、肺部感染,PAH发生率低,免疫抑制剂联用、抗菌素使用比例高,RP-ILD、Velcro啰音、CEA升高是预后的危险因素。Objective:To analyze the clinical characteristics and prognostic factors of anti-melanoma differentiation-associated gene 5(MDA5)-positive dermatomyositis(DM)patients with secondary interstitial lung disease(ILD).Methods:A retrospective collection was conducted on 97 patients with DM-ILD admitted to the First Affiliated Hospital of Guangxi Medical University from January 2018 to December 2022.They were di‐vided into anti-MDA5 positive group and anti-MDA5 negative group.The clinical characteristics and survival rate of the two groups were compared,and the influencing factors of prognosis were analyzed.Results:Com‐pared with the anti-MDA5 negative group,the disease duration of the anti-MDA5 positive group was shorter,the proportion of chest tightness,rapid progress(RP)-ILD,pulmonary infection and skin ulcers was increased,the proportion of pulmonary arterial hypertension(PAH)was decreased,the levels of white blood cells,platelets and creatine kinase were decreased,and the serum ferritin levels were increased(all P<0.05).Compared with the anti-MDA5 negative group,the proportions of combination of two immunosuppressants,use of gamma globulin and the first use of antibiotics during hospitalization were higher in the anti-MDA5 positive group(all P<0.05).With a median follow-up of 17.5 months,the overall mortality rate of anti-MDA5 positive group was 45.5%,and the 6-month,12-month and 36-month survival rates were 61.43%,58.57%and 55.71%,respectively.Cox regres‐sion analysis showed that the risk factors for the prognosis of anti-MDA5 positive ILD were RP-ILD,Velcro rales and elevated carcinoembryonic antigen(CEA)(all P<0.05).Conclusion:Patients with anti-MDA5 positive ILD have a shorter disease duration.They are prone to skin ulcers and lung infections,have a low incidence of PAH,and have a high proportion of immunosuppressive agents combination and antibiotic use.The occurrence of RP-ILD,Velcro rales,and elevated CEA are risk factors for prognosis.

关 键 词：抗黑色素瘤分化相关基因5 皮肌炎 间质性肺疾病 临床特征 预后 

分 类 号：R593.26[医药卫生—内科学]