Related Experiment Video
Updated: Aug 8, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pleural disease in lymphangioleiomyomatosis
Khalid F Almoosa1, Francis X McCormack, Steven A Sahn
1Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati College of Medicine, Cincinnati, OH 45267-0564, USA. khalid.almoosa@uc.edu
Clinics in Chest Medicine
|May 24, 2006
Summary
Pleural disease, including pneumothorax and chylothorax, is common in lymphangioleiomyomatosis (LAM). Limited data exist on whether these complications worsen LAM prognosis or accelerate disease progression.
Area of Science:
- Pulmonary Medicine
- Rare Diseases
- Thoracic Surgery
Background:
- Pleural disease is a frequent complication of lymphangioleiomyomatosis (LAM).
- Secondary spontaneous pneumothorax is highly prevalent and recurrent in LAM patients.
- Pneumothorax often serves as the initial diagnostic indicator for LAM.
Purpose of the Study:
- To review the characteristics and management of pleural complications in LAM.
- To investigate the impact of pleural disease on functional decline and prognosis in LAM patients.
Main Methods:
- Literature review of studies on LAM and pleural disease.
- Analysis of incidence, recurrence, and treatment outcomes for pneumothorax and chylothorax in LAM.
- Evaluation of existing data on the prognostic significance of pleural complications in LAM.
Main Results:
- LAM is associated with the highest rates of secondary spontaneous pneumothorax incidence and recurrence among chronic lung disorders.
- Pneumothorax frequently precedes LAM diagnosis, and pleurodesis for recurrent cases has a high failure rate.
- Chylothorax in LAM results from lymphatic obstruction and often necessitates pleurodesis; however, its impact on prognosis remains poorly understood due to LAM's rarity.
Conclusions:
- Pleural complications, particularly pneumothorax and chylothorax, are significant clinical issues in LAM.
- Current treatment options for recurrent pneumothorax in LAM have limited efficacy.
- Further research is needed to determine if pleural disease affects the progression and prognosis of LAM.
Related Concept Videos
01:13Pleura of the Lungs
The lungs are nestled in a cavity, shielded by the pleura. The pleura, a form of serous membrane, wraps around each lung. This membrane arrangement consists of two layers: the visceral and parietal pleurae. The visceral pleura lines the surface of the lungIn contrast, the parietal pleura is the outer layer and contacts to the thoracic wall, the mediastinum, and the diaphragm. The hilum is the point of connection between the visceral and parietal layers. The space between the parietal and...
01:25Pleural Effusion I: Introduction
Pleural effusion is an abnormal fluid accumulation in the pleural cavity, a narrow space between the lungs and the chest wall. It is not a disease per se but rather a symptom or indication of an underlying disease. In normal circumstances, this space contains a small amount of fluid (5 to 15 mL), a lubricant facilitating the non-frictional movement of the pleural surfaces.
There are two main types of pleural effusion: transudative and exudative. They are differentiated using Light's criteria,...
There are two main types of pleural effusion: transudative and exudative. They are differentiated using Light's criteria,...
01:30Pleural Disorders: Types and Brief Description
The pleura is a vital part of the respiratory system. It's a double-layered membrane surrounding the lungs and lining the chest cavity. The two layers of the pleura are:
01:28Pleural Effusion II: Symptoms and Management
Pleural Effusion Overview
A pleural effusion is the abnormal collection of fluid between the parietal and visceral pleura layers of tissue that form the lining of the lungs and chest cavity. It can occur independently or due to surrounding parenchymal diseases, such as infection, malignancy, or inflammatory conditions.
Clinical Manifestations:
A pleural effusion is the abnormal collection of fluid between the parietal and visceral pleura layers of tissue that form the lining of the lungs and chest cavity. It can occur independently or due to surrounding parenchymal diseases, such as infection, malignancy, or inflammatory conditions.
Clinical Manifestations:
01:08Pneumothorax II: Pathophysiology
Pneumothorax means the presence of air in the pleural space — the thin potential gap between the visceral and parietal pleura. This condition disrupts the normal pressure balance that keeps the lungs inflated, leading to partial or complete collapse of the affected lung.Normal physiologyUnder normal conditions, the pleural space maintains a slightly negative intrapleural pressure, which keeps the lungs expanded against the chest wall. This negative pressure creates a delicate balance between...
01:27Pneumothorax-II
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:
Clinical Manifestations: