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Pleuroparenchymal Fibroelastosis: A Case Report
Oumayma Haloui1, Fatima El Allam1, Ouiame Nabou1
1Department of Respiratory Diseases, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Abstract:
Pleuroparenchymal fibroelastosis (PPFE) is a rare, idiopathic interstitial pneumonia. wherein the first symptom might be dyspnea or a dry cough. The condition can also be manifested with chest pain secondary to pneumothorax. While the definitive diagnosis is based on a histological evaluation (which is not often performed), a computed tomography scan shows findings, such as apical fibrosis and pleural thickening of the apical lobes, which help assess the diagnosis. We describe a case of PPFE diagnosed radiologically in a 69-year-old man. This case highlights that PPFE is a pathology that can go unnoticed for a long time, and patients might neglect the revealing symptoms such as coughing.
Insights
Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. Radiological diagnosis is key, as symptoms like coughing may be overlooked, delaying treatment for this interstitial pneumonia.
Area of Science:
- Pulmonology
- Radiology
Background:
- Pleuroparenchymal fibroelastosis (PPFE) is an uncommon idiopathic interstitial pneumonia.
- Symptoms include dyspnea, dry cough, and chest pain potentially due to pneumothorax.
Observation:
- A case of PPFE diagnosed via radiological findings in a 69-year-old male is presented.
- Radiological hallmarks include apical fibrosis and pleural thickening of the apical lobes.
Findings:
- Computed tomography (CT) scans are crucial for assessing PPFE, complementing histological evaluation.
- PPFE can remain undiagnosed for extended periods, with patients potentially ignoring early symptoms like persistent coughing.
Implications:
- This case underscores the importance of considering PPFE in differential diagnoses, especially with characteristic radiological findings.
- Early recognition and diagnosis, even if radiological, are vital for managing PPFE and improving patient outcomes.
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