CTICasesPulmonary Amyloidosis in Sjogren Syndrome
Imaging findings
Serial CT chest scans over 10 years show stable, multiple bilateral thin-walled peribronchovascular cysts (consistent with LIP), associated with progressive, dense, calcifying/ossifying pulmonary nodules.
Key takeaways
Pulmonary amyloidosis is strongly associated with Sjögren syndrome and lymphoid interstitial pneumonia (LIP). The deposition of light-chain amyloid proteins prompts a foreign-body reaction and progressive calcification, often located in close proximity to LIP cysts. The cysts may develop due to valvular airway obstruction or elastolytic enzyme release by macrophages.
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