早期一线联合托法替布治疗重症抗黑色素瘤分化相关基因-5抗体阳性皮肌炎相关快速进展型间质性肺炎1例并文献复习  

Early first line combination of Tofacitinib for severe anti-melanoma differentiation associated gene-5 positive dermatomyositis with rapidly progressive interstitial lung disease:a case report and literature review

作　　者：谢姿 罗群[2] Xie Zi;Luo Qun(Department of Respiratory Medicine,Shenshan Medical Center,Memorial Hospital of Sun Yat-Sen University,Shanwei 516621,Guangdong,China;Department of Respiratory and Critical Care Medicine,Interstitial Pulmonary Disease Group,the First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510120,Guangdong,China)

机构地区：[1]中山大学孙逸仙纪念医院深汕中心医院呼吸内科,广东汕尾516621 [2]广州医科大学附属第一医院呼吸与危重症医学科间质性肺病组,广东广州510120

出　　处：《实用检验医师杂志》2023年第1期97-102,共6页Chinese Journal of Clinical Pathologist

摘　　要：目的 报告1例联合托法替布治疗有效的重症抗黑色素瘤分化相关基因-5抗体阳性皮肌炎相关快速进展型间质性肺炎(抗MDA5-Ab^(+)-DM-RP-ILD)病例,并进行文献复习,了解联合托法替布对重症抗MDA5-Ab^(+)-DM-RP-ILD的有效性。方法 采用自身前后对照研究方法,介绍2020年1月23日广州医科大学附属第一医院收治的1例重症抗MDA5-Ab^(+)-DM-RP-ILD患者的诊治经过及随访资料。以“快速进展型间质性肺炎、MDA5、皮肌炎、托法替布”为主题检索Medline数据库,评估联合托法替布治疗抗MDA5-Ab^(+)-DM-RP-ILD以及重症亚组患者的应用价值。结果 患者为49岁男性,因“皮疹、乏力1月余,呼吸困难1 d”入院,特征性体征为Gottron征、Gottron疹,高分辨率计算机断层扫描(HRCT)提示双肺多发磨玻璃影和实变。患者入院2 d接受小潮气量肺保护机械通气治疗,给予甲泼尼龙(2.22 mg·kg^(-1)·d^(-1))治疗后间质性肺炎缓解,因并发消化道大出血停药,半个月后间质性肺炎急性加重,予甲泼尼龙(0.56 mg·kg^(-1)·d^(-1))联合托法替布(10 mg/d)综合治疗,间质性肺炎逐渐缓解,成功脱机拔管,4个月后患者间质性肺炎明显缓解。分析9例联合托法替布治疗抗MDA5-Ab^(+)-DM-RP-ILD病例,间质性肺炎的缓解率为55.6%,病死率为37.5%,对照组病死率为84.6%;重症亚组的间质性肺炎缓解率为33.3%,病死率为50.0%。结论 联合托法替布治疗抗MDA5-Ab^(+)-DM-RP-ILD,患者病死率明显下降,但对重症患者的治疗有效率明显下降,早期一线联合托法替布以及早期肺保护策略可能有助于改善预后。Objective To report a case of severe anti-melanoma differentiation associated gene-5 antibody positive dermatomyositis with rapidly progressive interstitial lung disease(anti-MDA5-Ab^(+)-DM-RP-ILD),treated effectively with combination therapy of Tofacitinib,and review the literature to understand the effectiveness of combination therapy of Tofacitinib on severe anti-MDA5-Ab^(+)-DM-RP-ILD.Methods Using self controlled study method,the diagnosis,treatment and follow-up data of a patient with severe anti-MDA5-Ab^(+)-DM-RP-ILD admitted to the First Affiliated Hospital of Guangzhou Medical University on January 23,2020 were introduced.The Medline database was searched with the keywords or medical subject headings of"rapidly progressive interstitial lung disease,melanoma differentiation-associated gene 5,dermatomyositis and Tofacitinib"to evaluate the application value of combination therapy of Tofacitinib in the treatment of anti-MDA5-Ab^(+)-DM-RP-ILD and severe subgroups.Results The patient was a 49-year-old male admitted in hospital due to"rash,fatigue for over a month,dyspnea on exertion for 1 day".The characteristic signs were Gottron's sign and Gottron's papules.High resolution computed tomography(HRCT)showed multiple ground glass opacity and consolidation in bilateral lungs,with middle and lower lung predominance.The patient was intubated and low tidal volume mechanical ventilation was performed 2 days after admission,and interstitial pneumonia was relieved after receiving methylprednisolone(2.22 mg·kg^(-1)·d^(-1))prescription.Due to concurrent gastrointestinal bleeding,medication was stopped.As a result,the interstitial pneumonia worsened half a month later.Methylprednisolone(0.56 mg·kg^(-1)·d^(-1))combined with Tofacitinib(10 mg/d)was prescribed and the interstitial pneumonia gradually relieved.The patient was successfully weaning,after 4 months,interstitial pneumonia significantly improved.Analysis of 9 cases of anti-MDA5-Ab^(+)-DM-RP-ILD with combination therapy of Tofacitinib showed a interstitial pneum

关 键 词：快速进展型间质性肺炎 皮肌炎 人黑色素瘤分化相关基因-5 托法替布 机械通气 

分 类 号：R593.26[医药卫生—内科学] R563.13[医药卫生—临床医学]