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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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Detalles Bibliográficos
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
Descripción
Sumario: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.