SHM Converge Scientific Abstract ePoster Gallery-Breath on Borrowed Time: A Tale of Mda5-Positive Dermatomyositis

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This case report describes a 62-year-old man with anti-MDA5 dermatomyositis (MDA5-DM), a rare idiopathic inflammatory myopathy notable for its strong association with rapidly progressive interstitial lung disease (RP-ILD). He presented after two months of diffuse rash, progressive weakness, fever, weight loss, dyspnea, and recurrent “pneumonia” that did not improve with outpatient antibiotics.<br /><br />On exam, he was febrile, hypoxic, tachycardic, and hypotensive, with bibasilar crackles, proximal muscle weakness, Gottron’s papules, and an eschar-like rash. Laboratory testing showed mildly elevated inflammatory markers and ferritin, but many routine autoimmune tests were negative. Infectious workup was also negative. Further testing revealed a positive anti-MDA5 antibody. Imaging demonstrated severe pulmonary involvement, including consolidation, pneumothorax, and pneumomediastinum. MRI of the thigh showed feathery intramuscular edema consistent with active inflammatory myositis.<br /><br />He was treated aggressively with IV steroids, pulse-dose methylprednisolone, plasma exchange, cyclophosphamide, IVIG, and tacrolimus. Despite this, his respiratory status worsened from nasal cannula to high-flow oxygen, BiPAP, intubation, and eventually ECMO. Because of refractory RP-ILD, he was transferred for lung transplant evaluation.<br /><br />The report emphasizes that MDA5-DM should be considered in patients with nonspecific symptoms such as rash, weakness, and recurrent pneumonia, even when standard autoimmune tests are negative. Early recognition and prompt immunosuppression are critical, as delayed diagnosis can lead to severe lung disease, transplant need, and high mortality.

Asset Subtitle

Chetana Guthikonda

Meta Tag

Author List Chetana Guthikonda, Christopher Chan

Category Clinical Vignettes

Concept Anti-MDA5 Dermatomyositis

Concept Rapidly Progressive Interstitial Lung Disease

Concept Refractory Interstitial Lung Disease

Concept Multimodal Immunosuppressive Treatment

Concept Rash

Distinguished Finalist

Presenter Organization Wellstar Kennestone Regional Medical Center

Presenting Author Chetana Guthikonda

Track Adult

Keywords

anti-MDA5 dermatomyositis

rapidly progressive interstitial lung disease

idiopathic inflammatory myopathy

Gottron's papules

pneumomediastinum

pneumothorax

immunosuppression

cyclophosphamide

tacrolimus

lung transplant evaluation

Anti-MDA5 Dermatomyositis

Rapidly Progressive Interstitial Lung Disease

Refractory Interstitial Lung Disease

Multimodal Immunosuppressive Treatment

Rash