Lung histopathological pattern in a survivor with rapidly progressive interstitial lung disease and anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis. (2016)

• Record Type: Journal Article
• Authors: Suzuki, Atsushi; Kondoh, Yasuhiro; Taniguchi, Hiroyuki; Tabata, Kazuhiko; Kimura, Tomoki; Kataoka, Kensuke; Ono, Kenzo; Hashisako, Mikiko; Fukuoka, Junya
• Is Part Of: Respiratory medicine case reports. Volume 19(2016)
• Page Start: 5
• Page End: 8
• Publication Date: 2016
• Subjects: CADM -- RP-ILD -- MDA5 -- Lung histopathological pattern -- DAD; MDA5 melanoma differentiation-associated gene 5 -- CADM clinically amyopathic dermatomyositis -- RP-ILD rapidly progressive interstitial lung disease -- DAD diffuse alveolar damage -- CT computed tomography -- FVC forced vital capacity -- DLco diffusing capacity of the lung for carbon monoxide -- 6MWD six-min walk distance -- MMRC modified medical research council dyspnea scale -- NSIP nonspecific interstitial pneumonia -- IVCY intravenous cyclophosphamide; Respiratory organs -- Diseases -- Periodicals; Chest -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory System -- Periodicals; Chest -- Diseases; Respiratory organs -- Diseases; Electronic journals; Periodicals; 616.2
• DOI: 10.1016/j.rmcr.2016.05.008
• Languages: English
• ISSNs: 2213-0071

Abstract

Abstract: Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are specific indicators of patients with dermatomyositis, particularly clinically amyopathic dermatomyositis (CADM). CADM is occasionally accompanied by fatal, treatment-resistant, rapidly-progressive interstitial lung disease (RP-ILD). All previous reports showed that histopathological findings in RP-ILD with anti-MDA5 antibody-positive CADM indicated diffuse alveolar damage (DAD). This is the first report describing a non-DAD pattern in RP-ILD with anti-MDA5 antibody-positive CADM, which was improved by immunosuppressive therapy. This case may be a milder clinical phenotype than a typical DAD pattern in RP-ILD with anti-MDA5 antibody-positive CADM.