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Bizarre Cystic Lung Disease in Monoclonal Gammopathy (MGUS) and Cirrhosis with Hepatopulmonary Syndrome

Imaging findings

Chest CT shows bizarre, extensive, and confluent subpleural and perivascular pulmonary cysts primarily in the lower lobes. Additionally, there are dilated, beaded, and telangiectatic peripheral pulmonary vessels extending directly to the pleural surface. The patient also has findings of cirrhosis and a known monoclonal gammopathy.

Key takeaways

This case illustrates two separate rare pulmonary processes: cystic lung disease associated with a plasma cell dyscrasia (suggesting light chain deposition disease or localized amyloidosis) and classic vascular changes of hepatopulmonary syndrome secondary to liver cirrhosis. Extensive subpleural and perivascular cystic changes can sometimes mimic lymphocytic interstitial pneumonia (LIP), but their association with monoclonal gammopathy points to light chain deposition disease as a primary differential diagnosis.

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