title: Lung histopathological pattern in a survivor with rapidly progressive interstitial lung disease and anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis.
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.
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Suzuki
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kondoh
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Taniguchi
authors.affiliation: Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Tabata
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Kimura
authors.affiliation: Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Kensuke
authors.initials: K
authors.lastname: Kataoka
authors.affiliation: Department of Pathology, Tosei General Hospital, Seto, Aichi, Japan.
authors.forename: Kenzo
authors.initials: K
authors.lastname: Ono
authors.affiliation: Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Mikiko
authors.initials: M
authors.lastname: Hashisako
authors.affiliation: Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Junya
authors.initials: J
authors.lastname: Fukuoka
keywords: 6MWD, six-min walk distance; CADM; CADM, clinically amyopathic dermatomyositis; CT, computed tomography; DAD; DAD, diffuse alveolar damage; DLco, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; IVCY, intravenous cyclophosphamide; Lung histopathological pattern; MDA5; MDA5, melanoma differentiation-associated gene 5; MMRC, modified medical research council dyspnea scale; NSIP, nonspecific interstitial pneumonia; RP-ILD; RP-ILD, rapidly progressive interstitial lung disease