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Updated: Jul 7, 2026
07:36
Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
Published on: May 1, 2015
Lymphangioleiomyomatosis: a clinical update
1Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, MSB 6053, 231 Albert Sabin Way, Cincinnati, OH 45267-0564, USA. Frank.McCormack@uc.edu
Chest
|February 7, 2008
Summary
Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting women, characterized by progressive shortness of breath and lung function decline. Diagnosis often requires HRCT scans, and management focuses on symptom control and potential lung transplantation.
Area of Science:
- Pulmonology
- Genetics
- Radiology
Background:
- Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease primarily affecting women.
- It is associated with tuberous sclerosis genes, renal angiomyolipomas, and lymphatic spread.
- Clinical manifestations include progressive dyspnea, recurrent pneumothorax, and chylous effusions.
Purpose of the Study:
- To provide a comprehensive overview of Lymphangioleiomyomatosis (LAM).
- To discuss diagnostic criteria, clinical course, and current management strategies.
- To highlight emerging research and treatment targets.
Main Methods:
- Diagnosis relies on high-resolution CT (HRCT) scans and, in some cases, lung biopsy.
- Management strategies include bronchodilators, pleurodesis for pneumothorax, and embolization for large angiomyolipomas.
- Lung transplantation is a viable option for advanced disease.
Main Results:
- Lung function declines significantly faster in LAM patients compared to healthy individuals.
- HRCT scans are crucial for diagnosis, though biopsy may be needed.
- Angiomyolipomas over 4 cm pose a bleeding risk requiring evaluation for embolization.
Conclusions:
- Early HRCT screening is recommended for women with unexplained pneumothorax or tuberous sclerosis.
- Current treatments are empiric, focusing on estrogen antagonism and symptom management.
- Multicenter clinical trials targeting molecular pathways are ongoing.
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