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Scleroderma-associated interstitial lung disease (NSIP/LIP)

Imaging findings

CT scan in a young patient with severe scleroderma demonstrates progressive, bilateral subpleural cystic spaces (bubbly lung / pseudo-honeycombing) at the bases and anteriorly, without significant volume loss or traction bronchiectasis initially. Follow-up CT one year later shows collapse of these cysts, increased surrounding consolidation, and volume loss.

Key takeaways

Connective tissue diseases like scleroderma can present with subpleural cystic spaces that mimic honeycombing but lack the typical fibrosis and volume loss of UIP early on. These spaces can represent LIP or NSIP and may eventually collapse and scar down over time.

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