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. 2020 Apr;50(4):484-487.
doi: 10.1111/imj.14789.

Anti-MDA5-associated dermatomyositis

Rajiv Gupta  1 , Sunil Kumar  1 , Peter Gow  1 , Leon Hsien-Cheng Chang  2 , Linda Yen  1
Affiliations

Anti-MDA5-associated dermatomyositis

Rajiv Gupta et al. Intern Med J. 2020 Apr.

Abstract

Anti-MDA5-associated dermatomyositis (MDA5-associated DM) is an uncommon presentation of idiopathic inflammatory myositis, typically amyopathic, associated with rapidly progressive, treatment refractory interstitial lung disease and poor prognosis, particularly in patients with concomitant rapidly progressive interstitial lung disease (RP-ILD). We report two cases of MDA5-associated DM with fatal outcome in one of the patients, despite 'aggressive triple therapy' for RP-ILD.

Keywords: MDA5-associated interstitial lung disease; anti-MDA5-associated dermatomyositis; cyclosporine; inflammatory myopathy; intravenous immunoglobulins.

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References

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