CTICasesAnti-MDA5 ILD (Rapidly Progressive Interstitial Lung Disease)
Imaging findings
Sequential CTs over three months in a patient with known myositis demonstrate rapidly progressive basal-predominant peripheral pulmonary fibrosis with developing traction bronchiectasis and increasing reticulation, consistent with an accelerating fibrotic process.
Key takeaways
Anti-MDA5 (melanoma differentiation-associated gene 5) antibody-positive dermatomyositis or clinically amyopathic myositis is associated with a rapidly progressive and often fatal interstitial lung disease (RP-ILD). It is one of the most important causes of rapidly progressive ILD in the myositis spectrum, with a mortality of up to 50%. Early recognition and aggressive immunosuppression are critical.
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