CTICasesRapidly Progressive Interstitial Lung Disease in Amyopathic Dermatomyositis
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Imaging findings
The patient presented with shortness of breath. Initial CT showed subtle lesions at the lung bases, including a particular blob that was biopsied and found to be non-infectious. A few weeks later, there was rapid progression with widespread ground glass opacities throughout the lungs. Intermediate CT scans showed findings suggesting organizing pneumonia, which can blend into NSIP.
Key takeaways
At autopsy, the lung disease was found to be an NSIP pattern. Serologic workup revealed anti-Ro 52 and anti-MDA5 antibodies, both associated with dermatomyositis. The patient did not have muscle findings, presenting with amyopathic dermatomyositis where interstitial lung disease was the leading symptom, along with eventual subtle skin findings. Anti-MDA5 antibody is associated with rapidly progressive amyopathic dermatomyositis, often with ILD leading the clinical picture. This presentation can include an acute lung injury pattern, often a combination of NSIP, acute lung injury, and organizing pneumonia.
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