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Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry

Mehta, P; Machado, PM; Gupta, L; (2021) Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry. Rheumatology International , 41 (6) pp. 1021-1036. 10.1007/s00296-021-04819-1. Green open access

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Abstract

Anti-Melanoma Differentiation-Associated gene 5 (MDA-5) Dermatomyositis (MDA5, DM) is a recently identified subtype of myositis characteristically associated with Rapidly Progressive Interstitial Lung Disease (RP-ILD) and unique cutaneous features. We reviewed PubMed, SCOPUS and Web of Science databases and selected 87 relevant articles after screening 1485 search results, aiming to gain a better understanding of the pathophysiology, clinical features, diagnosis, and treatment approaches of anti-MDA-5 DM described in the literature. The etiopathogenesis is speculatively linked to an unidentified viral trigger on the background of genetic predisposition culminating in an acquired type I interferonopathy. The clinical phenotype is highly varied in different ethnicities, with new clinical features having been recently described, expanding the spectrum of cases that should raise the suspicion of anti-MDA-5 DM. Unfortunately, the diagnosis is frequently missed despite excessive mortality, calling for wider awareness of suspect symptoms. RP ILD is the major determinant of survival, treatment being largely based on observational studies with recent insights into aggressive combined immunosuppression at the outset.

Type: Article
Title: Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry
Open access status: An open access version is available from UCL Discovery
DOI: 10.1007/s00296-021-04819-1
Publisher version: http://doi.org/10.1007/s00296-021-04819-1
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Science & Technology, Life Sciences & Biomedicine, Rheumatology, Dermatomyositis, Myositis, Phenotype, Interstitial lung disease, Genetic predisposition, Autoantibodies, Immunosuppression
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10128435
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