摘要
特发性炎症性肌病(IIM)是一组病因未完全阐明的自身免疫性肌病。肺部受累比例大约为35%~40%,合并出现的间质性肺疾病是导致本病发病率、病死率最高的重要因素。肌炎相关抗体谱对临床诊断以及判断预后具有重要意义。其中抗MDA5抗体阳性皮肌炎患者的肌肉受累程度大多较轻,但是肺部并发的快速进展肺间质病变风险较高,通常导致难以纠正的呼吸衰竭,病死率高。通过对2015年至2017年治疗的9例抗MDA5抗体阳性特发性炎症性肌病合并肺间质疾病(IIM-ILD)患者临床资料进行分析。其中3例病情急剧进展,发展为呼吸衰竭而死亡。中医证候特点方面,此类患者临床表现复杂,虚证、实证、虚实夹杂均可见。起始西药治疗方案均给予激素联合免疫抑制剂强化方案,并序贯治疗。同时密切随访患者,若存在急性加重,及时积极干预。
Idiopathic inflammatory myopathy(IIM)is a group of autoimmune myopathy that is not fully elucidated.The proportion of pulmonary involvement is about 35-40%,and IIM-ILD is the risk factor of high mortality.The antibody spectrum of myositis is of great significance in clinical diagnosis and judging prognosis.The degree of muscle involvement is mostly mild in patients with MDA5 antibody positive dermatomyositis.However,the rapid development of pulmonary disease has a high risk of interstitial lung disease,which usually leads to respiratory failure with high mortality and high mortality.Clinical data of 9 cases of IIM-ILD who were administered in our department from 2015 to 2017 were analyzed.3 of them developed rapid progress and died of respiratory failure.In the aspects of TCM syndrome,the clinical manifestations were complex,and the deficiency syndrome,excess syndrome and combination of both were included.The initial western medicine treatments regimen were the combination of corticoid and immunosuppressive agents.At the same time,close follow up is required in case of acute exacerbation.
作者
张宗学
王玉光
ZHANG Zong-xue;WANG Yu-guang(Beijing Chinese Medicine Hospital Affiliated to Capital Medical University,Beijing 100010)
出处
《世界中西医结合杂志》
2019年第9期1191-1193,1197,共4页
World Journal of Integrated Traditional and Western Medicine
基金
北京市医院管理局“登峰”计划专项(DFL20150902)
关键词
抗MDA5抗体
特发性炎症性肌病
肺间质疾病
Anti-Mda5 Antibody
Idiopathic Inflammatory Myopathy
Interstitial Lung Disease
