Pleuroparenchymal fibroelastosis in an 8‐year old treated for neuroblastoma

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...

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Published in:Pediatric pulmonology 2020-03, Vol.55 (3), p.E1-E4
Main Authors: Gerstle, Karyn, Tanager, Kevin, Hegde, Satyanarayan, Chung, Jonathan H., Husain, Aliya N., Applebaum, Mark A., Dyamenahalli, Umesh
Format: Article
Language:English
Subjects:
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Child, Preschool
Cough - etiology
Dyspnea - etiology
Female
Fibrosis
Humans
interstitial lung disease
Lung Diseases, Interstitial - diagnosis
Lung Diseases, Interstitial - pathology
Neuroblastoma
Neuroblastoma - diagnostic imaging
Neuroblastoma - drug therapy
Neuroblastoma - radiotherapy
Parenchymal Tissue - diagnostic imaging
Parenchymal Tissue - pathology
Pleura - diagnostic imaging
Pleura - pathology
pulmonary hypertension
Transplants & implants
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Summary: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.
ISSN:8755-6863
1099-0496
DOI:10.1002/ppul.24638