抗MDA5抗体阳性的皮肌炎患者的临床特征及对其预后的影响

Clinical Features and Prognosis of Anti-MDA5-Positive Dermatomyositis Patients

目的 分析并探究抗MDA5抗体阳性和抗MDA5阴性皮肌炎(DM)患者的临床特征及该抗体对患者预后的影响.方法 回顾性收集分析2013年01月01日—2019年10月01日就诊于苏州大学附属第一医院风湿科门诊及住院部的36例DM患者的临床资料,包括临床表现、肌酶谱、铁蛋白、肌电图、肌活检、肺高分辨CT、肺功能、肌炎抗体谱等.由此对比分析抗MDA5抗体阳性和阴性DM患者的临床特征以及该抗体与DM-ILD的病情相关性.结果 该研究中15例抗MDA5抗体阳性的患者均为无肌病性皮肌炎(CADM),抗MDA5抗体阳性患者病程中往往以DM特征性皮损为仅有表现或主要症状,也就是说抗MDA5阳性DM患者发生眶周红肿、Gottron疹、皮肤破溃等的概率较抗MDA5抗体阴性患者高,肺间质病变发生率较抗MDA5抗体阴性的患者高且进展较快,7例患者在3个月内出现快速进展的间质性肺病;而抗MDA5抗体阳性DM患者的血清肌酸激酶水平显著较抗MDA5抗体阴性DM患者低;快速进展的间质性肺病患者的血清铁蛋白水平高于其他间质性肺病患者,并且在患者胸部高分辨CT提示间质性肺炎出现及发生进展之前,其血清铁蛋白水平就已经出现异常升高.结论 1.抗MDA5抗体阳性的DM患者均为CADM;2.抗MDA5抗体通常与DM患者并发肺间质病变密切相关;3.血清铁蛋白水平或许可以成为抗MDA5抗体阳性的DM患者肺间质病变病情进展的一个实验室监测指标之一.抗MDA5抗体阳性的DM患者往往病情重,肺部进展快,预后差,临床上需高度重视并及时干预.

Objective To analyze and explore the clinical characteristics of patients with positive anti-MDA5 antibody and anti-MDA5 negative dermatomyositis(DM)and the effect of anti-MDA5 antibody on the prognosis of patients.Methods Clinical data of 36 patients with DM,including clinical manifestations,myozyme,ferritin,EMG,muscle biopsy,lung high-resolution CT,lung function,and myositis antibody,were retrospectively collected and analyzed from 2013-01-01 to 2019-10-01 in the outpatient department and inpatient department of rheumatology department of the First Affiliated Hospital of Soochow University.The clinical characteristics of anti-MDA5 positive and negative DM patients and the correlation between this antibody and DM-ILD were compared and analyzed.Results 15 cases MDA5 antibody positive patients were without myopathy dermatomyositis(CADM),often MDA5 antibody positive patients in the course of the disease resistance in DM characteristic lesion is only performance or main symptoms,that is to say MDA5 positive resistance in patients with DM peri-orbit redness,Gottron rash,skin burst probability of patients with MDA5 antibody negative resistance is high,the incidence of pulmonary interstitial lesions in patients with MDA5 antibody negative resistance high and progress faster,7 patients had a rapid progress in three months of interstitial lung disease.The serum creatine kinase level of DM patients with positive anti-MDA5 antibody was significantly lower than that of DM patients with negative anti-MDA5 antibody.Serum ferritin levels in patients with rapidly progressive interstitial lung disease were higher than those in patients with other interstitial lung diseases,and serum ferritin levels were abnormally elevated before the onset and progression of interstitial pneumonia was indicated by high-resolution chest CT.Conclusion 1.A11 DM patients with positive anti-MDA5 were CADM;2.Anti-MDA5 antibodies are usually closely related to pulmonary interstitial disease in patients with DM;3.Serum ferritin levels may be one of the laboratory monitoring indicators for the progression of pulmonary interstitial disease in patients with MDA5-positive DM.Patients with anti-MDA5-positive DM are often in severe condition,with rapid pulmonary progression and poor prognosis.Therefore,it is necessary to attach great importance to and timely intervene in clinical practice.