Clinical Features of Anti-MDA5 Antibody-positive Rapidly Progressive Interstitial Lung Disease without Signs of Dermatomyositis

Noriho Sakamoto; Hiroshi Ishimoto; Shota Nakashima; Hirokazu Yura; Takuto Miyamura; Daisuke Okuno; Atsuko Hara; Takeshi Kitazaki; Tomoyuki Kakugawa; Yuji Ishimatsu; Minoru Satoh; Hiroshi Mukae

doi:10.2169/internalmedicine.1516-18

CASE REPORTS

Clinical Features of Anti-MDA5 Antibody-positive Rapidly Progressive Interstitial Lung Disease without Signs of Dermatomyositis

Noriho Sakamoto, Hiroshi Ishimoto, Shota Nakashima, Hirokazu Yura, Takuto Miyamura, Daisuke Okuno, Atsuko Hara, Takeshi Kitazaki, Tomoyuki Kakugawa, Yuji Ishimatsu, Minoru Satoh, Hiroshi Mukae

Author information

Noriho Sakamoto
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Hiroshi Ishimoto
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Shota Nakashima
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Hirokazu Yura
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Takuto Miyamura
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Daisuke Okuno
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Atsuko Hara
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Takeshi Kitazaki
Department of Respiratory Medicine, The Japanese Red Cross Nagasaki Genbaku Hospital, Japan
Tomoyuki Kakugawa
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Yuji Ishimatsu
Department of Cardiopulmonary Rehabilitation Science, Unit of Rehabilitation Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan
Minoru Satoh
Department of Clinical Nursing, School of Health Sciences, University of Occupational and Environmental Health, Japan
Hiroshi Mukae
Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan

Keywords: acute interstitial pneumonia, anti-melanoma differentiation-associated gene 5, clinically amyopathic dermatomyositis

JOURNAL OPEN ACCESS

2019 Volume 58 Issue 6 Pages 837-841

DOI https://doi.org/10.2169/internalmedicine.1516-18

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Abstract

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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