期刊
INTERNAL MEDICINE
卷 58, 期 6, 页码 837-841出版社
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.1516-18
关键词
acute interstitial pneumonia; anti-melanoma differentiation-associated gene 5; clinically amyopathic dermatomyositis
资金
- JSPS KAKENHI [JP17K09617, JP15K08790]
- Non-Profit Organization Aimed to Support Community Medicine Research in Nagasaki
Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody is associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM). We herein report three Japanese cases of anti-MDA5 antibody-positive RP-ILD without signs of CADM or DM. High-resolution computed tomography revealed patchy or subpleural distribution of consolidations and/or ground-glass opacities accompanied by traction bronchiectasis. All patients succumbed to respiratory failure within two months. Anti-MDA5 antibody-positive RP-ILD without signs of CADM or DM should be included in the differential diagnosis of acute/subacute ILD. Measurement of anti-MDA5 antibody and an intensive immunosuppressive regimen might rescue these patients from RP-ILD.
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