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Related Experiment Video

Updated: Sep 21, 2025

Refined Murine Model of Idiopathic Pulmonary Fibrosis

Refined Murine Model of Idiopathic Pulmonary Fibrosis

Published on: June 17, 2025

Idiopathic Pleuroparenchymal Fibroelastosis.

Kameron Tavakolian¹, Ndausung Udongwo¹, Steven Douedi¹

¹Department of Medicine, Jersey Shore University Medical Center, Neptune City, NJ, USA.

Journal of Medical Cases

|June 3, 2022

Summary

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare lung disease. This case report highlights diagnostic challenges and the lack of effective treatments for IPPFE, emphasizing the need for further research.

Keywords:

Dyspnea Hypoxia Idiopathic pleuroparenchymal fibroelastosis Interstitial lung disease Interstitial pneumonia Platythorax Pneumomediastinum Pneumothorax

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Area of Science:

Pulmonology
Radiology
Pathology

Background:

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare idiopathic interstitial pneumonia.
It affects the pleura and subpleural lung parenchyma, predominantly in the upper lobes.
Diagnosis relies on clinical, radiographic, and histopathological findings.

Observation:

An 86-year-old female presented with dyspnea and hypoxia.
She had a history of pneumomediastinum and unintentional weight loss.
Chest CT confirmed radiographic evidence of IPPFE.

Findings:

Standard treatments including antibiotics, diuretics, and steroids were ineffective.
The patient's condition deteriorated, and she was transitioned to hospice care.
IPPFE has a median survival of 2 years and often overlaps with other interstitial lung diseases.

Implications:

IPPFE is challenging to diagnose due to overlapping features with other interstitial lung diseases.
There are no established treatment guidelines for IPPFE.
Further research and case reporting are crucial for improved diagnosis and management strategies.