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Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting

Anti-Melanoma Differentiation-Associated gene 5 (Anti-MDA-5) dermatomyositis is a rare subtype of inflammatory myopathy characterized by unique skin lesions, rapidly progressive interstitial lung disease, and skeletal muscle inflammation. It has a high mortality rate in the absence of early treatmen...

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Autores principales: Risal, Ujjwol, Pandey, Suravi, Pangeni, Raju, Pandey, Rakshya, Bhattarai, Dharmagat, Adhikari, Sudeep, Basnyat, Buddha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: F1000 Research Limited 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10220425/
https://www.ncbi.nlm.nih.gov/pubmed/37251661
http://dx.doi.org/10.12688/wellcomeopenres.18728.1
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author Risal, Ujjwol
Pandey, Suravi
Pangeni, Raju
Pandey, Rakshya
Bhattarai, Dharmagat
Adhikari, Sudeep
Basnyat, Buddha
author_facet Risal, Ujjwol
Pandey, Suravi
Pangeni, Raju
Pandey, Rakshya
Bhattarai, Dharmagat
Adhikari, Sudeep
Basnyat, Buddha
author_sort Risal, Ujjwol
collection PubMed
description Anti-Melanoma Differentiation-Associated gene 5 (Anti-MDA-5) dermatomyositis is a rare subtype of inflammatory myopathy characterized by unique skin lesions, rapidly progressive interstitial lung disease, and skeletal muscle inflammation. It has a high mortality rate in the absence of early treatment. However, diagnosis of this entity is challenging in a country like Nepal because of various constraints such as lack of expert rheumatologists and resource limitations. Here we describe a case of one patient who had presented to us with generalized weakness, cough and shortness of breath who was finally diagnosed as anti-MDA-5 dermatomyositis. He responded to combination of immunosuppressives and is currently doing well. This case highlights the diagnostic and therapeutic challenges in managing such cases in a resource-limited setting.
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spelling pubmed-102204252023-05-28 Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting Risal, Ujjwol Pandey, Suravi Pangeni, Raju Pandey, Rakshya Bhattarai, Dharmagat Adhikari, Sudeep Basnyat, Buddha Wellcome Open Res Case Report Anti-Melanoma Differentiation-Associated gene 5 (Anti-MDA-5) dermatomyositis is a rare subtype of inflammatory myopathy characterized by unique skin lesions, rapidly progressive interstitial lung disease, and skeletal muscle inflammation. It has a high mortality rate in the absence of early treatment. However, diagnosis of this entity is challenging in a country like Nepal because of various constraints such as lack of expert rheumatologists and resource limitations. Here we describe a case of one patient who had presented to us with generalized weakness, cough and shortness of breath who was finally diagnosed as anti-MDA-5 dermatomyositis. He responded to combination of immunosuppressives and is currently doing well. This case highlights the diagnostic and therapeutic challenges in managing such cases in a resource-limited setting. F1000 Research Limited 2023-01-05 /pmc/articles/PMC10220425/ /pubmed/37251661 http://dx.doi.org/10.12688/wellcomeopenres.18728.1 Text en Copyright: © 2023 Risal U et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Risal, Ujjwol
Pandey, Suravi
Pangeni, Raju
Pandey, Rakshya
Bhattarai, Dharmagat
Adhikari, Sudeep
Basnyat, Buddha
Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting
title Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting
title_full Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting
title_fullStr Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting
title_full_unstemmed Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting
title_short Case Report: Anti-MDA-5 dermatomyositis in a resource-limited setting
title_sort case report: anti-mda-5 dermatomyositis in a resource-limited setting
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10220425/
https://www.ncbi.nlm.nih.gov/pubmed/37251661
http://dx.doi.org/10.12688/wellcomeopenres.18728.1
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