CTICasesAnti-MDA5 Dermatomyositis Interstitial Lung Disease
Imaging findings
CT chest shows rapidly progressive, bilateral, peripheral consolidative and ground-glass opacities in an organizing pneumonia pattern, with a lower-lobe predominance. The patient had a skin rash, markedly elevated CPK levels, and positive anti-MDA5 autoantibodies.
Key takeaways
Anti-MDA5 antibody-positive dermatomyositis is strongly associated with rapidly progressive interstitial lung disease (RPILD) and acute respiratory failure, which can mimic acute interstitial pneumonia (AIP) or ARDS (or COVID-19). Aggressive immunosuppressive therapy (combination steroids and calcineurin inhibitors) is required due to the high mortality rate.
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