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Isolated Left Lower Lobe Pleuroparenchymal Fibroelastosis Presenting as Hemothorax
Michael J Fassler1, Francis J Podbielski1
1University of Illinois at Chicago College of Medicine, Chicago, Illinois.
The Annals of Thoracic Surgery
|January 12, 2020
Summary
Pleuroparenchymal fibroelastosis, a rare lung disease, can present with spontaneous hemothorax. This case highlights an unusual presentation of this restrictive lung disease, emphasizing chronic lung injury as a potential cause.
Area of Science:
- Pulmonology
- Rare Diseases
- Thoracic Surgery
Background:
- Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia characterized by fibrosis and elastosis in the upper lobes.
- It typically presents with restrictive lung function, dyspnea, and cough, often mimicking other lung diseases.
- Acute complications like spontaneous pneumothorax have been documented, but hemothorax is exceedingly rare.
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