3例抗MDA5抗体阳性皮肌炎合并快速进展型间质性肺病患者的临床特点附文献复习  

Clinical features of 3 patients with positive anti-MDA5 antibody dermatomyositis combined with rapidly progressive interstitial lung disease and literature review

作　　者：孙兰 胡强 宋本艳 张榆铃 曹冬胤 李明凤 孙燕玲 付劲锋 尹霞 SUN Lan;HU Qiang;SONG Ben-yan;ZHANG Yu-ling;CAO Dong-yin;LI Ming-feng;SUN Yan-ling;FU Jin-feng;YIN Xia(PCCM Department,The Affiliated Hospital of Panzhihua University,Panzhihua 617000,China;Department of Rheumatology and Immunology,The Affiliated Hospital of Panzhihua University,Panzhihua 617000,China;Pathology department,The Affiliated Hospital of Panzhihua University,Panzhihua 617000,China;Beidahuang Group General Hospital,Haerbin 150000,China)

机构地区：[1]攀枝花学院附属医院呼吸与危重症医学科,四川攀枝花617000 [2]攀枝花学院附属医院风湿免疫科,四川攀枝花617000 [3]攀枝花学院附属医院病理科,四川攀枝花617000 [4]北大荒集团总医院,黑龙江哈尔滨150000

出　　处：《实用医院临床杂志》2024年第2期21-28,共8页Practical Journal of Clinical Medicine

基　　金：四川省医疗卫生与健康促进会科研基金资助项目(编号:KY2022SJ0116);成都市科技局重大科技应用示范项目(编号:2022-YF09-00003-SN)。

摘　　要：目的探讨抗MDA5阳性皮肌炎合并快速进展型间质性肺病的临床特点、治疗及预后。方法回顾性分析2020年7月至2021年10月在攀枝花学院附属医院呼吸与危重症医学科住院的3例抗MDA5抗体阳性皮肌炎合并快速进展型间质性肺病患者的临床资料、治疗经过、预后特点,结合文献复习进行研讨。结果3例患者中男1例,女2例,年龄(55.7±2.1)岁,病程(3.8±0.2)月。临床主要表现为典型的皮疹,进行性呼吸困难;血清肌酶均增高,血清抗MDA5抗体、抗组氨酰合成酶抗体(Jo-1)、抗Ro-52抗体均阳性;皮疹部位皮肤肌肉活检均提示炎性改变;短期内影像学呈进行性加重的肺间质纤维化。3例患者均使用了激素冲击+丙种球蛋白+环磷酰胺治疗,1例使用了利妥昔单抗治疗。3例患者分别在住院治疗后27、37、48天死亡,2例死于急性呼吸衰竭,1例死于脓毒性休克。结论抗MDA5阳性皮肌炎合并快速进展型间质性肺病是一种病情进展快、疗效差、死亡率高的疾病。其临床主要表现是典型的皮疹和进行性呼吸困难,血清肌酶增高,抗MDA5抗体阳性(3例患者同时合并抗组氨酰合成酶抗体Jo-1、抗Ro-52抗体阳性)。影像学均短期内呈进行性加重的肺间质纤维化。以激素冲击、免疫调节、靶向阻断等为主的药物治疗,未能成功挽救患者的生命。治疗后免疫低下所导致的严重感染,也是死亡的重要原因。早期识别,早期干预,以及疾病后期的ECMO支持、肺移植,可能是降低死亡率的有效手段。Objective To explore the clinical features,treatment and prognosis of patients with anti-melanoma differentiationassociated gene 5(MDA5)positive dermatomyositis combined with rapidly progressive interstitial lung disease(RPILD).Methods Clinical data,treatment process,and prognostic characteristics of 3 patients with anti MDA5 antibody positive dermatomyositis combined with RPILD admitted to our hospital from July 2020 to October 2021 were retrospectively analyzed.The discussion was performed after literature review.Results Among the 3 patients,1 was male and 2 were females with an average age of(55.7±2.1)years old and an average disease course of(3.8±0.2)months.The main clinical manifestations were typical rash and progressive dyspnea.Serum muscle enzyme was increased.Serum anti-MDA5 antibody,anti-histaminoyl synthase antibody(Jo-1)and anti-Ro-52 antibody were all positive.The skin and muscle biopsies at the rash sites all indicated inflammatory changes.The imaging showed progressive worsening pulmonary interstitial fibrosis in the short term.All 3 patients were treated with hormone shock+immunoglobulin+cyclophosphamide,and 1 patient was treated with rituximab.The 3 patients died on the 27th,37th,and 48th day after hospitalization,respectively.Moreover,2 patients died of acute respiratory failure,and 1 patient died of septic shock.Conclusions Anti-MDA5 positive dermatomyositis combined with RPILD is a disease with rapid progression,poor efficacy and high mortality rate.The main clinical manifestations are typical rash and progressive dyspnea,elevated serum muscle enzymes,and positive anti-MDA5 antibodies.In addition,the 3 patients simultaneously had positive anti-Jo-1 antibodies and anti-Ro-52 antibodies.Imaging shows progressive exacerbation of pulmonary interstitial fibrosis in the short term.Drug treatments mainly focused on hormone shock,immune regulation and targeted blockade.However,the drug treatments have failed to save the lives of the patients.Severe infection caused by immunosuppression after treatme

关 键 词：抗MDA5抗体阳性 皮肌炎 快速进展型间质性肺病 

分 类 号：R563.13[医药卫生—呼吸系统]