CTICasesMDA-5 Amyopathic Dermatomyositis
Imaging findings
CT chest in a 30-something male shows bilateral, peripheral organizing pneumonia that rapidly progressed over a month to diffuse alveolar damage, severe fibrotic changes, pneumomediastinum, and death. MRI of the hands showed severe periarticular soft tissue swelling and osteomyelitis.
Key takeaways
Anti-MDA-5 antibody-associated dermatomyositis is a rare subtype characterized by amyopathic presentation (no muscle weakness), rapidly progressive interstitial lung disease (RPILD) with high mortality, and painful, ulcerating skin/digital lesions. CT typically shows rapidly progressive organizing pneumonia/DAD with spontaneous air leaks.
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