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.
作者
张倩
李男
苏欣
田露
加慧
夏书月
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)
出处
《国际呼吸杂志》
2024年第5期592-597,共6页
International Journal of Respiration
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
Lung disease,interstitial
Anti-melanoma differentiation associated gene 5 antibody
Amyopathic dermatomyositis
Rapidly progressive interstitial lung disease
Triple therapy
