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
; 19: 5-8, 2016.
Article
em En
| MEDLINE
| ID: mdl-27354955
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.
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
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MEDLINE
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En
Ano de publicação:
2016
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Article