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Updated: Mar 16, 2026
Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Two cases of pleuroparenchymal fibroelastosis diagnosed with transbronchial lung biopsy
Hisako Kushima1, Kouko Hidaka2, Hiroshi Ishii1
1Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka, 814-0180, Japan.
Abstract:
Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare subset of idiopathic interstitial pneumonias (IIPs). Here we present two patients with PPFE in which the histology was confirmed with transbronchial lung biopsy (TBLB). The 25-year-old and 64-year-old men were both slender with a long history of pulmonary upper lobe fibrosis and a marked restrictive impairment. Although the imaging findings supported the diagnosis of PPFE, surgical lung biopsy (SLB) seemed to be needed to identify fibroelastosis for the definite diagnosis. However, we selected TBLB instead of SLB because of their general condition and the risk such as prolonged pneumothorax after TBLB. TBLB specimens in both patients showed aggregates of elastic fibers in the submucosa that were essential clues for the histological diagnosis of PPFE. TBLB may be an alternative tool for the histological diagnosis of PPFE, although a multidisciplinary discussion is necessary for the final diagnosis of PPFE as a clinicopathological entity.
Insights
Transbronchial lung biopsy (TBLB) can diagnose idiopathic pleuroparenchymal fibroelastosis (PPFE). This study shows TBLB identified key elastic fiber aggregates, offering an alternative to surgical lung biopsy for PPFE diagnosis.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP).
- Diagnosis typically requires surgical lung biopsy (SLB), which carries significant risks.
- Imaging findings can suggest PPFE, but histological confirmation is crucial.
Observation:
- Two male patients, aged 25 and 64, presented with upper lobe fibrosis and restrictive impairment.
- Transbronchial lung biopsy (TBLB) was performed instead of SLB due to patient condition and risks.
- TBLB specimens revealed submucosal elastic fiber aggregates, characteristic of PPFE.
Findings:
- Histological confirmation of PPFE was achieved using TBLB in both cases.
- The presence of elastic fiber aggregates in TBLB specimens was a key diagnostic finding.
- TBLB demonstrated potential as a less invasive diagnostic tool for PPFE.
Implications:
- TBLB may serve as a viable alternative to SLB for diagnosing PPFE.
- This approach could reduce procedural risks for patients with PPFE.
- Multidisciplinary discussion remains essential for definitive PPFE diagnosis.
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