Jun 28, 2024 · Pleuroparenchymal fibroelastosis (PPFE) is a rare, benign entity. About half of the cases are thought to be idiopathic, with the other half secondary to underlying diseases or conditions (e.g. transplantation). Idiopathic cases belong to …

Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia that is often underdiagnosed on computed tomography scans. The disease process involves a combination of fibrosis involving the visceral pleura and fibroelastic …

Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in 2013, its name refers to a combination of fibrosis ...

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare condition that is classified as an idiopathic interstitial pneumonia (1). It involves upper lobe fibrosis of the pleura and subpleural lung parenchyma.

Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in ...

Pleuroparenchymal fibroelastosis (PPFE) is a distinct pattern of pulmonary fibrosis which often runs a rapidly progressive course with a poor prognosis, and it is likely to be introduced as a separate entity in the new classification scheme of idiopathic interstitial pneumonias.

Jan 1, 2025 · On high-resolution computed tomography (HRCT), PPFE is typified by dense subpleural consolidation with traction bronchiectasis, architectural distortion, and upper-lobe volume loss.

Aug 26, 2015 · Due to rapid clinical and radiological deterioration evidenced by high resolution computed tomography (HRCT), the suspicion of PPFE was raised leading to pathologists to perform elastic fiber stain (procedure not routinely done) on the surgical lung biopsy (SLB).

Apr 20, 2021 · Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia that is often underdiagnosed on computed tomography scans. The disease process involves a combination of fibrosis involving the visceral pleura and fibroelastic changes within the subpleural lung parenchyma.

Aug 26, 2022 · HRCT diagnostic criteria include upper lobe pleural thickening and subpleural fibrosis with less marked or absent lower lobe involvement [4,5,6,7]. HRCT appears to be the only imaging modality that can procure a definitive diagnosis. Prognosis and outcome. PPFE may progres gradually over 10 years –20 years .