以肺部症状为首发表现的MDA5抗体阳性皮肌炎的临床特征与预后分析

Clinical features and prognosis of anti⁃MDA5 positive dermatomyositis with pulmonary symptom as the first manifestation

目的:分析以肺部症状为首发表现的黑色素瘤分化相关基因蛋白5(melanoma differentiation⁃associated gene 5,MDA5)抗体阳性皮肌炎(dermatomyositis,DM)患者的临床特征与预后。方法:回顾性选取2017年8月—2021年10月南京医科大学第一附属医院收治的MDA5抗体阳性DM患者57例为研究对象,根据临床首发表现分为肺部首发组(n=15)和非肺部首发组(n=42),比较两组一般临床资料、实验室检查结果、影像学表现和预后情况。结果:肺部首发组患者血清铁蛋白(serum ferritin,SF)、C反应蛋白(C⁃reactive protein,CRP)水平明显高于非肺部首发组,肌酸激酶(creatine kinase,CK)水平明显低于非肺部首发组(P<0.05)。肺部首发组患者的胸部CT征象以斑片状影为主,其次为磨玻璃影、网格影、实变影,小叶间隔增厚、结节影、蜂窝肺和胸腔积液等征象较少。多因素Logistic回归分析结果显示,SF、CK水平是MDA5抗体阳性DM患者以肺部症状为首发表现的独立危险因素(P<0.05)。两组患者发病后6个月内的累积生存率比较,差异有统计学意义(P<0.05)。结论:以肺部症状为首发表现的MDA5抗体阳性DM患者治疗方案以糖皮质激素+免疫抑制剂为主,患者预后较差,其临床表现及影像学特征具有多样性;SF、CK水平与MDA5抗体阳性DM患者以肺部症状为首发表现相关,或可成为实验室监测指标之一。

Objective:To analyze the clinical features and prognosis of patients with anti⁃melanoma differentiation⁃associated gene 5(MDA5)positive dermatomyositis(DM)with pulmonary symptom as the first manifestation.Methods:The curren study retrospectively reviewed 57 patients with anti⁃MDA5 positive DM who were admitted to the First Affiliated Hospital of Nanjing Medical University between August 2017 and October 2021.The patients were divided into the pulmonary first symptom group(n=15)and the non⁃pulmonary first symptom group(n=42)according to the first clinical manifestation.The general clinical data,laboratory test results,imaging findings and prognosis were compared between the two groups.Results:There were statistically significant differences in therapeutic regimens between the two groups(P<0.05).The levels of serum ferritin(SF)and C⁃reactive protein(CRP)in the pulmonary first symptom group were significantly higher than those in the non⁃pulmonary first symptom group,and the level of creatine kinase(CK)was significantly lower than that in the non⁃pulmonary first symptom group(P<0.05).The main chest CT sign of patients in the pulmonary first symptom group was patchy shadow,followed by ground⁃glass opacity,thread net,and consolidation.Few interlobular septal thickening,nodular opacity,honeycomb lung,and pleural effusion were observed.Multivariate logistic regression analysis showed that the levels of SF and CK were independent risk factors for anti⁃MDA5 positive DM with pulmonary symptoms as the first manifestations(P<0.05).The cumulative survival rate within six months after the onset was significantly different in the two groups(P<0.05).Conclusion:Glucocorticoids combined with immunosuppressive agents is the main therapeutic regimen for anti⁃MDA5 positive DM patients with pulmonary symptoms as the first manifestations.The patients have poor prognosis,and their clinical manifestations and imaging findings are diverse.The levels of SF and CK are related to anti⁃MDA5 positive DM with pulmonary symptom as the first manifestation,which indicates that they can be used as laboratory monitoring indicators.