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Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient
BACKGROUND: Each myositis-specific autoantibody (MSA) tends to have a distinct clinical presentation. Coexistence of MSAs do not commonly occur. If they do, it is unknown if there is an overlap of clinical features or prognostic implications. There are a few reported cases of overlap between these a...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7429127/ https://www.ncbi.nlm.nih.gov/pubmed/32799827 http://dx.doi.org/10.1186/s12890-020-01256-x |
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author | Li, Zi Ying Gill, Evanpaul Mo, Fan Reyes, Candice |
author_facet | Li, Zi Ying Gill, Evanpaul Mo, Fan Reyes, Candice |
author_sort | Li, Zi Ying |
collection | PubMed |
description | BACKGROUND: Each myositis-specific autoantibody (MSA) tends to have a distinct clinical presentation. Coexistence of MSAs do not commonly occur. If they do, it is unknown if there is an overlap of clinical features or prognostic implications. There are a few reported cases of overlap between these antibodies, mostly reported in patients with Japanese descent. Our aim for this case report is to turn more attention and interest for future MSA profile studies in the Hispanic population, which may hopefully spur better therapies if we realize the prognostic implications of certain myositis subsets including double-positive autoantibody syndromes. CASE PRESENTATION: A 27-year-old Hispanic female was admitted to the medical intensive care unit due to acute hypoxemic respiratory failure secondary to acute respiratory distress syndrome (ARDS). She had failed conventional mechanical ventilation and was cannulated for venovenous extracorporeal membrane oxygenation (VV-ECMO) to manage her respiratory failure. She had erythematous scaly plaques on bilateral 3rd metacarpophalangeal joints on examination. Her autoimmune workup revealed positivity for both anti-PL-7(threonyl) and anti-melanoma differentiation-associated gene 5 (MDA5) autoantibodies. After extensive evaluation, it was concluded that she had rapidly progressive interstitial lung disease (RPILD) due to amyopathic dermatomyositis. Despite maximal medical management, she was ultimately transitioned to comfort care measures and expired. CONCLUSION: We would like to highlight the rarity of double antibody positive amyopathic dermatomyositis. This unique clinical presentation has only been reported in persons of Japanese descent. Our case is likely to be the first reported to occur in a person of Hispanic descent in the United States. The rarity of our case could stimulate further study of overlapping MSA to understand its varied presentations and prognoses including possible tendency toward a rapidly progressive ILD phenotype. Earlier detection of these clinical syndromes can lead to better outcomes for patients with RPILD. This case report could also herald an increased recognition and understanding of MSA profile in the Hispanic population in the USA. |
format | Online Article Text |
id | pubmed-7429127 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-74291272020-08-17 Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient Li, Zi Ying Gill, Evanpaul Mo, Fan Reyes, Candice BMC Pulm Med Case Report BACKGROUND: Each myositis-specific autoantibody (MSA) tends to have a distinct clinical presentation. Coexistence of MSAs do not commonly occur. If they do, it is unknown if there is an overlap of clinical features or prognostic implications. There are a few reported cases of overlap between these antibodies, mostly reported in patients with Japanese descent. Our aim for this case report is to turn more attention and interest for future MSA profile studies in the Hispanic population, which may hopefully spur better therapies if we realize the prognostic implications of certain myositis subsets including double-positive autoantibody syndromes. CASE PRESENTATION: A 27-year-old Hispanic female was admitted to the medical intensive care unit due to acute hypoxemic respiratory failure secondary to acute respiratory distress syndrome (ARDS). She had failed conventional mechanical ventilation and was cannulated for venovenous extracorporeal membrane oxygenation (VV-ECMO) to manage her respiratory failure. She had erythematous scaly plaques on bilateral 3rd metacarpophalangeal joints on examination. Her autoimmune workup revealed positivity for both anti-PL-7(threonyl) and anti-melanoma differentiation-associated gene 5 (MDA5) autoantibodies. After extensive evaluation, it was concluded that she had rapidly progressive interstitial lung disease (RPILD) due to amyopathic dermatomyositis. Despite maximal medical management, she was ultimately transitioned to comfort care measures and expired. CONCLUSION: We would like to highlight the rarity of double antibody positive amyopathic dermatomyositis. This unique clinical presentation has only been reported in persons of Japanese descent. Our case is likely to be the first reported to occur in a person of Hispanic descent in the United States. The rarity of our case could stimulate further study of overlapping MSA to understand its varied presentations and prognoses including possible tendency toward a rapidly progressive ILD phenotype. Earlier detection of these clinical syndromes can lead to better outcomes for patients with RPILD. This case report could also herald an increased recognition and understanding of MSA profile in the Hispanic population in the USA. BioMed Central 2020-08-15 /pmc/articles/PMC7429127/ /pubmed/32799827 http://dx.doi.org/10.1186/s12890-020-01256-x Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Li, Zi Ying Gill, Evanpaul Mo, Fan Reyes, Candice Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient |
title | Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient |
title_full | Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient |
title_fullStr | Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient |
title_full_unstemmed | Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient |
title_short | Double anti-PL-7 and anti-MDA-5 positive Amyopathic Dermatomyositis with rapidly progressive interstitial lung disease in a Hispanic patient |
title_sort | double anti-pl-7 and anti-mda-5 positive amyopathic dermatomyositis with rapidly progressive interstitial lung disease in a hispanic patient |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7429127/ https://www.ncbi.nlm.nih.gov/pubmed/32799827 http://dx.doi.org/10.1186/s12890-020-01256-x |
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