CTICasesCystic Lung Disease in Connective Tissue Disease Overlap Mimicking UIP
[...]. Cystic Lung Disease in Connective Tissue Disease Overlap Mimicking UIP. CTICases. Published June 10, 2017. https://cticases.com/c/94f67
Imaging findings
Chest CT shows severe cystic changes in the lower lobes, mimicking the honeycombing of usual interstitial pneumonia (UIP). However, there is a striking absence of reticulation, architectural distortion, or traction bronchiectasis in the remaining lung parenchyma, which is completely normal. The cysts are thin-walled and run along the airways, consistent with airway-derived cysts (bronchiolitis-associated) rather than true fibrotic honeycombing. Serology was positive for both anti-SSA (Sjögren's) and anti-Jo-1 (anti-synthetase syndrome).
Key takeaways
Progressive lower-lobe thin-walled cysts can closely mimic the honeycombing of UIP on CT. This 'pseudo-honeycombing' can occur in connective tissue diseases (such as Sjögren's syndrome or anti-synthetase syndrome) due to lymphoid follicular bronchiolitis or light chain deposition, where cysts are derived from dilated airways rather than a matrix of dense fibrosis. Identifying the lack of surrounding fibrosis/reticulation and checking autoimmune serology are critical, as these patients benefit from immunosuppressive therapy rather than antifibrotics.
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