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Pulmonary Apical Cap as a Potential Risk Factor for Pleuroparenchymal Fibroelastosis
Daniel-Costin Marinescu1, John English2, Tony Sedlic3
1Department of Medicine, Thoracic Service, University of British Columbia, Vancouver, BC, Canada; Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.
Chest
|June 8, 2021
Summary
Pleuroparenchymal fibroelastosis (PPFE) may develop from pulmonary apical cap (PAC) after lung injury. This case shows PAC evolving into asymmetric PPFE, suggesting shared pathways in lung injury responses.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Pleuroparenchymal fibroelastosis (PPFE) is a progressive, fatal interstitial lung disease affecting upper lobes.
- The etiology of PPFE is unknown, but its histopathology resembles the benign pulmonary apical cap (PAC).
Observation:
- A case study details a patient with PAC that transformed into asymmetric PPFE over six years.
- This evolution occurred following a unilateral surgical lung injury.
Findings:
- The histopathologic similarities suggest PAC and PPFE share common biologic pathways.
- PAC may increase susceptibility to PPFE development following inflammatory lung insults.
Implications:
- This case highlights the potential histopathologic evolution from PAC to PPFE.
- Understanding these shared pathways could inform future research into PPFE pathogenesis and treatment.
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