CTICasesFamilial Pleuroparenchymal Fibroelastosis
▶ Watch on YouTube — age-restricted, cannot embed here
Imaging findings
The chest radiograph shows extensive upper lung zone fibrosis, left greater than right, with confluent reticulation extending to the apical pleural surface. Surgical staples are noted in the apical pleura from a lung biopsy. CT images from 2007 and 2016 demonstrate progression of patchy, coarse, very coarse reticulation with relative sparing of the lower lung zones. Traction bronchiectasis is also present. The appearance, with its apical and peripheral predominance, does not resemble typical IPF.
Key takeaways
Familial pleuroparenchymal fibroelastosis (PPFE) is an unusual form of fibrosing interstitial lung disease characterized by predominant upper lung zone and pleural fibrosis, which can progress over time. It typically involves confluent reticulation and traction bronchiectasis, often sparing the lower lobes. While rare, familial forms exist, and PPFE can also be seen in contexts such as lung and stem cell transplantation.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Massive GoiterMetabolic
- Macklin Phenomenon (Pneumomediastinum)Trauma
- Paratracheal Hematoma (Post-Aortic Stent Placement)Iatrogenic
- Tracheobronchomalacia (Severe)Congenital
- Pulmonary Arterial Hypertension with Recurrent HemoptysisVascular
- Pulmonary Infarct with Pleural Effusion ("Orange Peel" Sign)Vascular
- Massive Paraesophageal VaricesVascular
- Ganglioneuroma (Cystic Posterior Mediastinum)Neoplastic
See all cases from May 18, 2016 →
Related ILD cases
- Langerhans Cell Histiocytosis
- Hypersensitivity Pneumonitis
- Acute Exacerbation of Idiopathic Pulmonary Fibrosis (UIP pattern) with Organizing Pneumonia pattern
- Hypersensitivity Pneumonitis (Farmer's Lung)
- Hypersensitivity Pneumonitis (from Down Comforter)
- Pleuroparenchymal Fibroelastosis as a Manifestation of Graft-versus-Host Disease