Related Experiment Video
Updated: Dec 30, 2025
Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Pleuroparenchymal fibroelastosis in an 8-year old treated for neuroblastoma
Karyn Gerstle1, Kevin Tanager1, Satyanarayan Hegde1
1Pediatrics, University of Chicago, Chicago, Illinois.
Insights
Pleuroparenchymal fibroelastosis (PPFE) is a rare adult lung disease. This case study details the youngest patient with PPFE, highlighting diagnostic challenges and fatal progression.
Area of Science:
- Pulmonology
- Pediatric Pathology
Background:
- Pleuroparenchymal fibroelastosis (PPFE) is a rare, progressive lung disease typically affecting adults.
- It presents with pleural and subpleural fibrosis, leading to respiratory symptoms and potential mortality.
Observation:
- This report describes the youngest documented patient with PPFE, whose condition emerged years after neuroblastoma treatment.
- Initial symptoms mimicked asthma, delaying diagnosis and management.
Findings:
- The patient developed multiple comorbidities during the delayed diagnosis period.
- Progressive lung disease occurred before lung transplantation could be considered, resulting in a fatal outcome.
Implications:
- This case underscores the importance of considering PPFE in pediatric patients, even with atypical presentations.
- Early recognition and diagnosis of PPFE are critical for timely intervention and improved patient outcomes.
Abstract:
Pleuroparenchymal fibroelastosis (PPFE), which is primarily diagnosed in adults, is a progressive lung pathology associated with significant morbidity and mortality. PPFE is characterized by pleural and subpleural parenchymal disease causing dyspnea, cough, and recurrent pneumothoraces. PPFE can be precipitated by autoimmune disorders, recurrent respiratory infections, chemotherapy, and transplant. We describe the youngest recorded patient to develop PPFE, whose symptoms began several years after treatment for neuroblastoma. Her symptoms were initially mistaken for worsening asthma, and multiple comorbidities developed during the prolonged time to recognition of PPFE and she progressed to fatal lung disease before potentially curative lung transplantation could occur.
