Pneumomediastinum in MDA5+ Dermatomyositis: A Case Series

MDA5+ DM, or anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (DM), is a rare autoimmune illness that primarily affects women of Asian origin. The typical presentation of MDA5+ DM includes a variety of cutaneous lesions accompanied by either no muscular weakness (amyopathic) or hypomyopathic features. In patients with MDA5+ DM, rapid progression of interstitial lung disease is a frequent manifestation associated with poor prognosis. Pneumomediastinum, spontaneous intramuscular haemorrhage, and macrophage activation syndrome form a spectrum of rare manifestations associated with MDA5+ DM. Since these issues are uncommon but fatal, it's important to explore the approaches for diagnosis, treatments, and possible mechanisms, that are useful for prompt treatments and management of the patient.

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Publication Details

Journal
Mediterranean Journal of Rheumatology
Published
2024-12-01
DOI
https://doi.org/10.31138/mjr.060124.pim
Citations
1
Primary Topic
Inflammatory Myopathies and Dermatomyositis
Type
article
Field-Weighted Citation Impact
0.39
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Pneumomediastinum in MDA5+ Dermatomyositis: A Case Series

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Pneumomediastinum in MDA5+ Dermatomyositis: A Case Series

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article en
1 citations

Abstract

MDA5+ DM, or anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (DM), is a rare autoimmune illness that primarily affects women of Asian origin. The typical presentation of MDA5+ DM includes a variety of cutaneous lesions accompanied by either no muscular weakness (amyopathic) or hypomyopathic features. In patients with MDA5+ DM, rapid progression of interstitial lung disease is a frequent manifestation associated with poor prognosis. Pneumomediastinum, spontaneous intramuscular haemorrhage, and macrophage activation syndrome form a spectrum of rare manifestations associated with MDA5+ DM. Since these issues are uncommon but fatal, it's important to explore the approaches for diagnosis, treatments, and possible mechanisms, that are useful for prompt treatments and management of the patient.

Mediterranean Journal of RheumatologyVol. 35(4)
JSS Academy of Higher Education and Research (IN), King George's Medical University (IN), JSS Medical College and Hospital (IN)
Zero hunger
Openalex Percentile: Top 30%
Inflammatory Myopathies and Dermatomyositis
0.39
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