SHM Converge Scientific Abstract ePoster Gallery-Spontaneous Intramuscular Hemorrhage in Anti-Mda5+ Dermatomyositis

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A 44-year-old man with a history of gout presented with 6 months of progressive bilateral hand swelling, muscle weakness, fatigue, rash, and shortness of breath, with worsening chest rash over the prior 2 weeks. On exam, he was hypoxic (84% on room air), required 10 L oxygen, had bilateral crackles, and showed an erythematous chest rash with heliotrope features. Workup revealed lymphopenia, markedly elevated ferritin (4606 ng/mL), negative ANA/ANCA/RF/anti-CCP, and a myomarker panel positive for anti-MDA5 antibody, consistent with anti-MDA5 dermatomyositis with rapidly progressive interstitial lung disease (ILD).<br /><br />CT chest showed bilateral ground-glass opacities, septal thickening, and mild bronchiectasis in an NSIP pattern, compatible with ILD associated with mixed connective tissue disease. He was treated with high-dose steroids, tacrolimus, and rituximab. However, one week into hospitalization he developed hemorrhagic shock. CT abdomen/pelvis demonstrated a large left retroperitoneal hematoma and diffuse left iliopsoas intramuscular hemorrhage with imaging features suggesting active bleeding.<br /><br />Despite treatment, he deteriorated and died from multiorgan failure. The case highlights anti-MDA5 dermatomyositis, a subtype often associated with rapidly progressive ILD, skin findings, and relatively limited muscle involvement. It also emphasizes spontaneous intramuscular hemorrhage as a rare but highly fatal complication, with reported mortality of 50–60%, particularly in patients with ILD, male sex, anti-MDA5 positivity, lymphopenia, hyperferritinemia, and exposure to high-dose steroids or heparin.

Asset Subtitle

Ethan Molitch-Hou

Meta Tag

Author List Ethan Molitch-Hou, Pooja Parekh

Category Clinical Vignettes

Concept Anti-MDA5 Dermatomyositis

Concept Rapidly Progressive Interstitial Lung Disease

Concept Spontaneous Intramuscular Hemorrhage

Concept Interstitial Lung Disease

Concept NSIP Pattern

Distinguished Non-Finalist

Presenter Organization Department of Medicine, University of Chicago Medicine

Presenting Author Ethan Molitch-Hou

Track Adult

Keywords

anti-MDA5 dermatomyositis

rapidly progressive interstitial lung disease

heliotrope rash

ground-glass opacities

NSIP pattern

hyperferritinemia

lymphopenia

retroperitoneal hematoma

iliopsoas hemorrhage

hemorrhagic shock

Anti-MDA5 Dermatomyositis

Rapidly Progressive Interstitial Lung Disease

Spontaneous Intramuscular Hemorrhage

Interstitial Lung Disease

NSIP Pattern