CTICasesPleuroparenchymal Fibroelastosis
Imaging findings
Serial chest CT scans over a five-year period in a patient with scleroderma demonstrate progressive development of bilateral, upper-lobe-predominant pleural thickening and subpleural consolidative reticulation with marked volume loss, resulting in superior displacement of the hila and fissures. A dilated esophagus is noted.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by prominent pleural and subpleural parenchymal intra-alveolar fibrosis and elastosis, showing upper-lobe predominance. While most cases are idiopathic, PPFE can occur secondary to connective tissue diseases like scleroderma, bone marrow transplantation, or chemotherapy. CT shows progressive upper lobe volume loss and subpleural consolidation.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Aortic Valve ThrombusVascular
- Thoracotomy-Induced Keloid FormationIatrogenic
- Lupus-Associated Pulmonary FibrosisAutoimmune
- Tracheobronchial Stent MigrationIatrogenic
- Malignant Pericardial EffusionNeoplastic
- Enterococcal EndocarditisInfection
- Sternal Wire DehiscenceIatrogenic
- LymphangioleiomyomatosisNeoplastic
See all cases from January 30, 2018 →