CTICasesAnti-MDA5 and PL-7 Positive Amyopathic Dermatomyositis Presenting as Acute Lung Injury
Imaging findings
Initial chest radiographs show significantly reduced lung volumes and symmetric, bilateral consolidations. Subsequent CT scans demonstrate the organizing and early fibrotic phases of diffuse alveolar damage, characterized by dependent consolidations, bronchiectasis, and diffuse ground-glass opacities in a patient requiring ECMO.
Key takeaways
Autoimmune connective tissue diseases can present as rapidly progressive, acute respiratory failure or idiopathic ARDS, even in patients with no prior history of rheumatologic disease. Rapidly progressive interstitial lung disease or diffuse alveolar damage can be associated with anti-MDA5 and antisynthetase antibodies (like PL-7) in patients with amyopathic dermatomyositis, where skin and muscle findings may be entirely absent.
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