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Lupus-Related Cystic Lung Disease Mimicking Fibrosis

Lupus-Related Cystic Lung Disease Mimicking Fibrosis▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

Confluent, thin-walled to moderately thick-walled cystic changes, predominantly in the lung bases. These changes evolved from a prior appearance of consolidation consistent with an organizing pneumonia pattern. Biopsy revealed a non-specific interstitial pneumonitis pattern with fibrosis, but no follicular bronchiolitis or usual interstitial pneumonia (UIP). Subpleural sparing was noted.

Key takeaways

This case demonstrates that lupus patients can develop cystic lung disease that appears as lung destruction or evolution of fibrosing organizing pneumonia or non-specific interstitial pneumonitis (NSIP), rather than classic honeycombing of UIP. The presence of subpleural sparing is a key feature differentiating it from UIP.

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