抗黑色素瘤分化相关基因-5抗体与皮肌炎合并肺间质病变的相关分析  

Analysis of Anti-MDA5 autoantibodies associated with interstitial lung disease in dermatomyositis

作　　者：邢晓婧[1] 李安琪 黄敏[1] 王睿[1] 李承新[1] Xiaojing Xing;Anqi Li;Min Huang(Department of Dermatology,First Medical Center of Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第一医学中心皮肤科,北京100853 [2]南开大学医学院

出　　处：《中华保健医学杂志》2020年第2期120-123,共4页Chinese Journal of Health Care and Medicine

基　　金：北京市自然科学基金项目(No.7184248)。

摘　　要：目的探究抗黑色素瘤分化相关基因-5(antimelanoma differentiation-associated gene 5,MDA5)抗体阳性的皮肌炎(dermatomyositis,DM)患者的临床特征,分析其发生肺间质病变(interstitial lung disease,ILD)的病程特点与预后情况。方法选取解放军总医院第一医学中心2017年1月~2019年4月收治的31例抗MDA5抗体阳性的DM患者,并对其临床资料进行回顾性分析。结果抗MDA5抗体阳性的DM患者皮疹发生率高,易出现皮肤破溃,肌炎发生率与此抗体阴性者无明显差异(P<0.05),未发现合并肿瘤的患者。所有患者均合并ILD,且ILD常在DM病程后期出现,83.87%的患者出现呼吸系统症状。经治疗后6例(19.35%)患者死亡,其中5例死于ILD及其并发症。经随访1例病程中出现急进性肺间质病变(Rapid Progressive Interstitial Lung Disease,RPILD)的患者,疗程中其血清中抗MDA5抗体水平持续降低,且病情较前好转。结论抗MDA5抗体阳性的DM患者常伴皮损,患癌风险低,合并ILD的概率高。ILD常在DM后期发病,具有死亡率高、预后差的特点,经治疗后抗MDA5抗体水平可降低。MDA5抗体可指导DM患者的治疗及预后判断。Objective To explore the clinical character of dermatomyositis(DM)patients with antimelanoma differentiationassociated gene 5(MDA5)antibodies.To analyse the feature of course and prognosis on interstitial lung disease(ILD).Methods The31 DM patients with anti-MDA5 antibodies were screened by immunoblotting test,and the clinical data of them were retrospectively analyzed.Results DM patients with anti-MDA5 antibodies had a high incidence of rash and were prone to skin ulcer.The incidence of myositis was not significantly different from patients without anti-MDA5 antibodies.There were no patients with tumor found.All the patients were combined with ILD,which always occurred in the late stage of DM,and 83.87%of them presented respiratory symptoms.There were 6 patients(19.35%)died after treatment,and 5 of them died from ILD or the complications of ILD.One DM patient with anti-MDA5 antibodies who was accompanied Rapid Progressive Interstitial Lung Disease(RPILD)in the course of disease was followed up.We found that the level of anti-MDA5 antibodies has been decreasing continuously with the state of illness better after treatment.Conclusion DM patients with anti-MDA5 antibodies are always associated with skin ulceration and have a low risk of cancer.They have a high probability of combining ILD,which happens latter but has the feature of high mortality and poor prognosis.The level of anti-MDA5 antibodies can be reduced after treatment.

关 键 词：皮肌炎 抗黑色素瘤分化相关基因-5抗体 肺间质病变 

分 类 号：R593.26[医药卫生—内科学]