CTICasesLight-Chain Deposition Disease (LCDD) in Sjogren's Syndrome
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Imaging findings
Chest CT shows bilateral, multiple perivascular cysts, some of which are associated with small, non-calcified soft tissue nodules. There is a small right apical pneumothorax. Pathology from a previous lung biopsy confirms light-chain deposition disease along the amyloid spectrum, demonstrating follicular architecture, aggregates of B cells, and lymphocytes.
Key takeaways
Light-chain deposition disease (LCDD) is a rare monoclonal immunoglobulin deposition disease that can manifest as cystic lung disease, often closely related to amyloidosis and lymphocytic interstitial pneumonia (LIP). On imaging, it classically presents with perivascular cysts and nodules. It can be isolated, associated with multiple myeloma, or linked to connective tissue diseases like Sjogren's syndrome (suggested here by positive SS-A/SS-B serology and follicular bronchiolitis pathology).
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