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.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Necrobiotic Rheumatoid Nodules Causing Pneumothorax and PneumomediastinumAutoimmune
- Diaphragmatic Intramuscular HematomaTrauma
- Eisenmenger Syndrome (Secundum Atrial Septal Defect) with Pulmonary Arterial Atherosclerosis and In Situ ThrombosisCongenital
- Lung Abscess secondary to Perforated Esophageal CancerNeoplastic
- Right Phrenic Nerve Injury and Hemidiaphragm Paresis post Left Atrial CryoablationIatrogenic
- Bilobed Bronchogenic CystCongenital
- Watchman Left Atrial Appendage Occlusion DeviceArtifact
- Giant Benign Schwannoma of the ThoraxNeoplastic
See all cases from March 9, 2017 →