CTICasesRapidly Progressive Fibrotic NSIP from Anti-MDA5-Antibody-Positive Dermatomyositis
Imaging findings
A young patient presents with constitutional symptoms and a chest CT pattern of ground-glass opacity with subpleural sparing and no honeycombing, consistent with fibrotic NSIP. A follow-up CT less than four months later shows dramatic interval progression to advanced fibrotic NSIP.
Key takeaways
Rapidly progressive fibrotic NSIP developing over just a few months in a young patient should raise suspicion for anti-MDA5-antibody-positive dermatomyositis-associated interstitial lung disease, a presumed autoimmune process associated with this specific antibody that can necessitate lung transplant evaluation within months of presentation; prior biopsy in this pattern favored NSIP with focal fibroblastic foci over usual interstitial pneumonia.
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