抗MDA5抗体阳性皮肌炎伴快速进展型间质性肺病2例并文献复习  

Anti-MDA5 antibody-positive dermatomyositis combined with rapidly progressive interstitial lung disease:two cases and a literature review

作　　者：张倩 李男[1] 苏欣 田露 加慧 夏书月[1] Zhang Qian;Li Nan;Su Xin;Tian Lu;Jia Hui;Xia Shuyue(Department of Respiratory and Critical Care Medicine,Affiliated Central Hospital of Shenyang Medical College,Shenyang 110020,China)

机构地区：[1]沈阳医学院附属中心医院呼吸与危重症医学科,沈阳110020

出　　处：《国际呼吸杂志》2024年第5期592-597,共6页International Journal of Respiration

摘　　要：本文通过回顾性分析2例抗黑色素瘤分化相关基因5(MDA5)抗体阳性皮肌炎合并快速进展型间质性肺病(RP-ILD)病历资料,结合相关文献报道,探讨了抗MDA5抗体阳性皮肌炎伴RP-ILD的早期识别方法、有效治疗方案和病情监测手段,为临床工作提供有价值的相关信息。2例患者均以皮肤损害为首发表现,继而出现活动后气短、乏力和RP-ILD表现,辅助检查均存在相关抗体阳性,同时合并癌胚抗原升高,无明显肌痛等肌病表现,分析得出抗MDA5抗体阳性皮肌炎患者的临床特征为易伴发RP-ILD,疾病进展迅速,预后差,死亡率高,提示临床医生接诊RP-ILD同时合并特异性皮疹患者应尽早完善抗MDA5抗体筛查,早诊早治,以期改善该类患者预后。This article retrospectively analyzed the medical records of two cases of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis(anti-MDA5+DM)complicated with rapidly progressive interstitial lung disease(RP-ILD),and then discussed the early identification methods,effective treatment options and disease monitoring methods of anti-MDA5+DM combined with RP-ILD,so as to provide valuable information for clinical work.Two patients presented skin lesions as the first manifestation,followed by the development of post-activity shortness of breath,fatigue,and manifestations of RP-ILD.Auxiliary examinations showed positivity for relevant antibodies.Considering the increase in carcinoembryonic antigen,lack of obvious muscle pain or other muscle disease manifestations,we summarized that clinical characteristics of anti-MDA5+DM included the prone to RP-ILD,rapid disease progression,poor prognosis,and high mortality.We recommended an early screening of anti-MDA5 antibody in RP-ILD patients combined with specific rash.Early diagnosis and treatment greatly favor their prognosis.

关 键 词：肺疾病 间质性 抗黑色素瘤分化相关基因5抗体 无肌病性皮肌炎 快速进展型间质性肺病 三联治疗 

分 类 号：R593.26[医药卫生—内科学] R563[医药卫生—临床医学]