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Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core
Cátia Esteves1, Francisco R Costa2, Margarida T Redondo3
1Department of Radiology, Centro Hospitalar de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal. catiamcesteves@gmail.com.
Objectives:
Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) with variable clinical and radiological features. Diagnosis is based on histology obtained by surgical lung biopsy, which is associated with significant mortality and morbidity. This study aims to briefly review PPFE and discuss the role of CT-guided transthoracic core lung biopsy (TTB) in its diagnosis.
Materials/Methods:
Four cases of PPFE diagnosed at our institution with TTB are reported and discussed.
Results:
Clinical, radiological and histological features are in agreement with the previous literature cases. TTB provided the diagnosis in all cases. Iatrogenic pneumothorax was the main complication in all patients. Placement of a chest tube was needed in three patients. An overlap between PPFE and other interstitial lung diseases (ILD) was documented.
Conclusion:
PPFE is an underdiagnosed IIP, so radiologist awareness of it needs to be widespread in patients with fibrosis with apical-caudal distribution. Coexistence of different lung diseases strengthens the idea of a predisposing factor. TTB proved to be a good diagnostic tool and can be considered the first choice for invasive assessment of these patients. PFFE has a variable course with no established therapeutic options; therefore a multidisciplinary team is crucial in the approach to patients with ILD.
Main Messages/Teaching Points:
• PPFE should be considered in the differential diagnosis of fibrosis with apical-caudal distribution. • CT-guided TTB can be considered the first choice for invasive assessment of PPFE. • Site of biopsy has to be chosen carefully in order not to miss PPFE. • Coexistence of different lung diseases strengthens the idea of a predisposing factor. • A multidisciplinary team is crucial in the approach to patients with ILD.
Insights
Pleuroparenchymal fibroelastosis (PPFE) is an underdiagnosed lung disease. CT-guided transthoracic core lung biopsy (TTB) is a valuable tool for diagnosing PPFE, offering an alternative to surgical biopsy.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) with diverse clinical and radiological presentations.
- Traditional diagnosis relies on surgical lung biopsy, carrying significant risks.
- This review highlights the diagnostic utility of CT-guided transthoracic core lung biopsy (TTB) for PPFE.
Purpose of the Study:
- To review Pleuroparenchymal fibroelastosis (PPFE).
- To discuss the role of CT-guided transthoracic core lung biopsy (TTB) in the diagnosis of PPFE.
- To emphasize the importance of radiologist awareness and multidisciplinary approaches for ILD.
Main Methods:
- Case series reporting four patients diagnosed with PPFE via TTB.
- Review of clinical, radiological, and histological findings.
- Analysis of TTB-related complications and diagnostic yield.
Main Results:
- TTB successfully diagnosed PPFE in all reported cases.
- Iatrogenic pneumothorax was the primary complication, requiring chest tube placement in three patients.
- Observed overlap between PPFE and other interstitial lung diseases (ILDs).
Conclusions:
- PPFE is an underdiagnosed IIP; radiologists must consider it in apical-caudal fibrotic patterns.
- CT-guided TTB is a viable first-choice diagnostic tool for PPFE assessment.
- A multidisciplinary team approach is essential for managing patients with ILD, including PPFE.
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