Successful treatment of rapidly progressive interstitial lung disease complicated by a refractory pneumomediastinum in a patient with anti-MDA5 antibody-positive dermatomyositis  

作　　者：Lili Jiang Hong Chen Lihua Duan 

机构地区：[1]Department of Rheumatology and Clinical Immunology,Jiangxi Provincial People's Hospital,The First Affiliated Hospital of Nanchang Medical College,Nanchang,Jiangxi,China

出　　处：《Rheumatology & Autoimmunity》2023年第4期240-243,共4页风湿病与自身免疫（英文）

基　　金：Natural Science Foundation of Jiangxi Province,Grant/Award Number:20202ACBL206011;Jiangxi Provincial Clinical Research Center for Rheumatic and Immunologic Diseases,Grant/Award Number:20192BCD42005;Jiangxi Province Medical Leading Discipline Construction Project。

摘　　要：Introduction:Anti-melanoma differentiation-associated gene 5-positive dermatomyositis(anti-MDA5+DM)is a distinct subtype of DM,which is characterized by typical cutaneous features,minimal or no muscle involvement and notable interstitial lung disease,which typically progresses rapidly and has a high mortality.Spontaneous pneumomediastinum(PNM),a relatively unusual but serious complication of anti-MDA5+DM,further increases mortality.Currently,there is no generally accepted treatment regimen for anti-MDA5+DM-associated PNM.Case Description:A 53-year-old man with anti-MDA5+DM presented with rapidly progressive interstitial lung disease that progressed to diffuse subcutaneous emphysema and PNM despite aggressive immunosuppressive therapies.He responded well to combined anti-infection treatments,moderate immunotherapy,and continuous oxygen therapy.Conclusion:Comprehensive screening for potential infections,as well as close monitoring of the patient's immune status is essential for individualizing treatment and maximizing prognosis.

关 键 词：anti-melanoma differentiation-associated protein 5 antibody DERMATOMYOSITIS interstitial lung diseases PNEUMOMEDIASTINUM 

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