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MDA5 Dermatomyositis Associated ILD

Imaging findings

Serial chest CTs show rapidly progressive interstitial lung disease starting with a mixed organizing pneumonia/NSIP pattern (perilobular and peribronchovascular ground-glass opacities and consolidation) and progressing over 3 months to severe lung volume loss and fibrosis.

Key takeaways

Anti-MDA5 antibody-associated dermatomyositis is strongly linked to clinically amyopathic dermatomyositis and rapidly progressive interstitial lung disease (RPILD), which is often refractory to immunosuppressants and has a high mortality rate.

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