CTICasesAnti-MDA5 Antibody-Associated Interstitial Lung Disease
Imaging findings
A patient with skin lesions, Gottron papules, and mechanic's hands has lower-lung-zone opacities with a morphology suggesting combined cellular NSIP and organizing pneumonia. Serologic testing was negative for the antisynthetase antibody but positive only for anti-MDA5. Over the interval from April to December, despite prednisone and mycophenolate, the disease progressed with new upper lung involvement and extensive fibrosing NSIP.
Key takeaways
A dermatomyositis-like clinical picture (Gottron papules, mechanic's hands) with an NSIP/organizing pneumonia pattern should prompt myositis-specific antibody testing; anti-MDA5 amyopathic disease is a particularly ominous subtype. MDA5-associated interstitial lung disease can be rapidly progressive and severe, sometimes causing acute respiratory failure, and progression despite steroids and mycophenolate signals a poor course requiring escalation of immunosuppression.
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