CTICasesScleroderma with Severe Bronchiectasis and Cystic Lung Disease (Elastolysis due to Protein Deposition)
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Imaging findings
Patient with limited scleroderma and Sjögren's syndrome showing progression of cystic disease and severe bronchiectasis between 2008 and 2016. Many proximal generations of segmental bronchi were open and dilated, often with attenuated or no observable bronchial wall between the dilated lumen and parenchyma. Patches of ground glass and tiny nodules were also present. The bronchiectasis was described as the "most florid" seen.
Key takeaways
This case illustrates marked progression of cystic lung disease and very florid airway disease in scleroderma. The presenter attributes this to protein deposition (likely light chains, given the Sjögren's link) leading to elastolysis, possibly mediated by macrophage-derived metalloproteinases, rather than solely Lymphocytic Interstitial Pneumonia (LIP). This mechanism may also apply to conditions like paraseptal emphysema. The absence of bronchial walls in some dilated bronchi was a remarkable finding.
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