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

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• 作者：Atsushi Suzuki^a ; ^{atsushi-salyu@hotmail.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Yasuhiro Kondoh^a ; ^{konyasu2003@yahoo.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Hiroyuki Taniguchi^a ; ^{taniguchi@tosei.or.jp" class="auth_mail" title="E-mail the corresponding author} ; Kazuhiko Tabata^b ; ^{t-kaz@nagasaki-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Tomoki Kimura^a ; ^{tomoki_kimura@tosei.or.jp" class="auth_mail" title="E-mail the corresponding author} ; Kensuke Kataoka^a ; ^{kataoka@tosei.or.jp" class="auth_mail" title="E-mail the corresponding author} ; Kenzo Ono^c ; ^{byori@tosei.or.jp" class="auth_mail" title="E-mail the corresponding author} ; Mikiko Hashisako^b ; ^{mikiko-h@nagasaki-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Junya Fukuoka^b ; ^{fukuokaj@nagasaki-u.ac.jp" class="auth_mail" title="E-mail the corresponding author}
• 关键词：CADM ; RP-ILD ; MDA5 ; Lung histopathological pattern ; DAD
• 刊名：Respiratory Medicine Case Reports
• 出版年：2016
• 出版时间：2016
• 年：2016
• 卷：19
• 期：Complete
• 页码：5-8
• 全文大小：1726 K

文摘

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.