2023 年第 9 期 第 18 卷

抗黑色素瘤分化相关基因5抗体阳性皮肌炎患者临床特征及早期死亡危险因素分析

Clinical characteristics and risk factors for early mortality in anti-melanoma differentiation associated gene 5 antibody-positive dermatomyositis patients

作者：成蔚韦雨颂李芬唐琪谢希杜金烽

英文作者：Cheng Wei Wei Yusong Li Fen Tang Qi Xie Xi Du Jinfeng

单位：中南大学湘雅二医院风湿免疫科，长沙410000

英文单位：Department of Rheumatology and Immunology the Second Xiangya Hospital of Central South University Changsha 410000 China

关键词：皮肌炎；抗黑色素瘤分化相关基因5抗体；临床特征；早期死亡

英文关键词：Dermatomyositis;Anti-melanomadifferentiationassociatedgene5antibody;Clinicalcharacteristics;Earlymortality

• 摘要：

• 目的  探索抗黑色素瘤分化相关基因5（MDA5）抗体阳性皮肌炎患者临床特征及预后，分析患者早期死亡的危险因素。方法 回顾性收集2015年6月1日至2021年10月1日在中南大学湘雅二医院住院的抗MDA5抗体阳性皮肌炎患者的临床表现、实验室检查、免疫学抗体谱、辅助检查以及初始治疗方案相关资料。根据患者疾病确诊3个月内生存结局将其分为死亡组（25例）、生存组（91例）。比较2组临床特征及预后情况，对患者早期死亡（发病3个月）进行单因素及多因素Logistic回归分析。结果 本中心抗MDA5抗体阳性皮肌炎患者3个月死亡率为21.6%(25/116)。死亡组患者发病年龄高于生存组，病程短于生存组，发热、Ⅰ型呼吸衰竭比例大于生存组，氧合指数、用力肺活量占预计值百分比及一氧化碳弥散量占预计值百分比均低于生存组，淋巴细胞计数、白蛋白水平低于生存组，红细胞沉降率（ESR）、C反应蛋白（CRP）、铁蛋白、乳酸脱氢酶（LDH）水平高于生存组，采用血浆置换及糖皮质激素比例高于生存组,使用环磷酰胺比例低于生存组(均P＜0.05)。单因素Logistic分析结果显示,高龄、病程短、发热、淋巴细胞计数减少、炎性指标（ESR、CRP、铁蛋白）升高、低蛋白血症、Ⅰ型呼吸衰竭、氧合指数下降是抗MDA5抗体阳性皮肌炎患者早期死亡的危险因素(均P＜0.05)。多因素Logistic回归结果提示，高龄（比值比=1.097，P=0.041）、发热（比值比=6.279，P=0.020）、氧合指数下降（比值比=0.986，P=0.004）是抗MDA5抗体阳性皮肌炎患者3个月内死亡的独立危险因素。结论 高龄、病程短、发热、淋巴细胞计数减少、炎性指标升高、低蛋白血症、出现Ⅰ型呼吸衰竭、氧合指数下降可预警抗MDA5抗体阳性皮肌炎患者早期死亡风险。高龄、发热、氧合指数下降是抗MDA5抗体阳性皮肌炎患者早期死亡的独立危险因素。

• Objective To explore the clinical characteristics and prognosis of anti- melanoma differentiation associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) patients, and to analyze the risk factors for early mortality in these patients. Methods The clinical data, laboratory examinations, immunological antibody profiles, auxiliary examination and initial treatment regimens of anti-MDA5 antibody-positive DM patients admitted to the Second Xiangya Hospital of Central South University from June 1, 2015 to October 1, 2021 were retrospectively collected. Patients were divided into the death group (25 cases) and the survival group (91 cases) according to the outcome within 3 months after diagnosis. The clinical characteristics and prognosis were compared between the two groups, and univariate and multivariate Logistic regression analyses were performed to identify risk factors for early mortality (within 3 months of disease onset). Results The 3-month mortality rate of anti-MDA5 antibody-positive DM patients in this center was 21.6%(25/116). Compared with the survival group, patients in the death group were older, had a shorter disease duration, higher rates of fever and type Ⅰ espiratory failure, lower oxygenation index, forced vital capacity as a percentage of estimate, carbon monoxide dispersion as a percentage of estimate, lymphocyte count and albumin levels, higher levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ferritin, and lactic dehydrogenase, higher proportions of plasma exchange and glucocorticoid use, and lower proportion of cyclophosphamide use (all P＜0.05). Univariate Logistic analysis showed that, older age, shorter disease duration, fever, decreased lymphocyte count, elevated inflammatory markers (ESR, CRP, ferritin), hypoalbuminemia, type Ⅰ respiratory failure, decreased oxygenation index were identified as risk factors for early mortality in anti-MDA5 antibody-positive DM patients (all P＜0.05). Multivariate Logistic regression analysis showed that older age (odds ratio=1.097, P=0.041), fever (odds ratio=6.279, P=0.020), and decreased oxygenation index (odds ratio=0.986, P=0.004) were independent risk factors for 3-month mortality in anti-MDA5 antibody-positive DM patients. Conclusions  sOlder age, shorter disease duration, fever, decreased lymphocyte count, elevated inflammatory markers, hypoalbuminemia, type Ⅰ respiratory failure and decreased oxygenation index can predict the risk of early mortality in anti-MDA5 antibody-positive DM patients. Older age, fever, and decreased oxygenation index are independent risk factors for early mortality in anti-MDA5 antibody-positive DM patients.