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

Atsushi Suzuki; Yasuhiro Kondoh; Hiroyuki Taniguchi; Kazuhiko Tabata; Tomoki Kimura; Kensuke Kataoka; Kenzo Ono; Mikiko Hashisako; Junya Fukuoka

doi:10.1016/j.rmcr.2016.05.008

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

Respir Med Case Rep. 2016 May 28:19:5-8. doi: 10.1016/j.rmcr.2016.05.008. eCollection 2016.

Authors

Atsushi Suzuki¹, Yasuhiro Kondoh¹, Hiroyuki Taniguchi¹, Kazuhiko Tabata², Tomoki Kimura¹, Kensuke Kataoka¹, Kenzo Ono³, Mikiko Hashisako², Junya Fukuoka²

Affiliations

¹ Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
² Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
³ Department of Pathology, Tosei General Hospital, Seto, Aichi, Japan.

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.

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.

Publication types

Case Reports