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Related Concept Videos

Pneumothorax-I01:26

Pneumothorax-I

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A pneumothorax is a condition where air builds up in the space between the lung and the chest wall, causing the lung to collapse. This condition arises when air enters the space between the parietal and visceral pleura, disrupting the negative pressure essential for lung inflation. This can lead to a partial or complete collapse of the lung.
Pneumothorax can be even further classified as spontaneous, traumatic, and tension pneumothorax.
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Pneumothorax-II01:27

Pneumothorax-II

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Pneumothorax is a medical condition defined by the buildup of air in the pleural space between the lungs and the chest wall. This accumulation of air can lead to partial or complete lung collapse, resulting in a range of clinical manifestations. Understanding the clinical presentation and effective management strategies is crucial for healthcare professionals in providing timely and appropriate care to individuals with pneumothorax.
Clinical Manifestations:
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Related Experiment Video

Updated: Feb 13, 2026

International Expert Consensus and Recommendations for Neonatal Pneumothorax Ultrasound Diagnosis and Ultrasound-guided Thoracentesis Procedure
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Pleuroparenchymal fibroelastosis presenting with pneumothorax.

Yen-Yu Lin1, Wen-Hu Hsu2, Mei-Han Wu3

  • 1Division of Molecular Pathology, Pathology and Laboratory Medicine Department, Taipei Veterans General Hospital, Taipei, Taiwan.

SAGE Open Medical Case Reports
|March 20, 2018
PubMed
Summary

This study describes pleuroparenchymal fibroelastosis, a rare interstitial lung disease characterized by pleural and subpleural fibrosis. Early diagnosis and understanding of this progressive condition are crucial for patient management.

Keywords:
Interstitial lung diseasepleurapneumothorax

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Area of Science:

  • Pulmonology
  • Pathology
  • Radiology

Background:

  • Interstitial lung diseases (ILDs) encompass a heterogeneous group of disorders affecting the lung parenchyma.
  • Pleuroparenchymal fibroelastosis (PPFE) is a rare form of ILD with distinct pathological and clinical features.

Observation:

  • A 47-year-old woman presented with spontaneous pneumothorax and bilateral lung opacities.
  • Imaging revealed consolidations and reticular opacities predominantly in pleural and subpleural regions.
  • Histopathology confirmed significant fibrosis of the visceral pleura and subpleural areas, particularly in the upper lobes.

Findings:

  • The case presented is consistent with pleuroparenchymal fibroelastosis, distinguished by pleural involvement and upper lobe predominance.
  • Pathological findings included alveolar septal elastosis and intra-alveolar fibrosis.
  • PPFE differs from usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP) due to its specific pleural and upper lobe characteristics and limited inflammation.

Implications:

  • Pleuroparenchymal fibroelastosis is a distinct interstitial lung disease with a progressive course, impacting approximately half of patients within 10 years.
  • Currently, no curative treatments are available for PPFE.
  • Accurate diagnosis through integrated clinical, imaging, and pathological assessment is vital for managing this rare lung condition.