Related Experiment Videos
Pulmonary lymphangiomyomatosis. A review
The American Journal of Pathology
|May 1, 1975
Summary
Pulmonary lymphangiomyomatosis (PLAM) is a rare lung disease affecting women of reproductive age, causing progressive breathlessness and potentially fatal respiratory insufficiency. Further research is needed to understand its relationship with tuberous sclerosis.
Area of Science:
- Pulmonary Medicine
- Pathology
- Radiology
Background:
- Pulmonary lymphangiomyomatosis (PLAM) is a rare, progressive lung disease exclusively affecting women of reproductive age.
- Symptoms include progressive dyspnea, pneumothorax, chylous effusions, and hemoptysis, often leading to respiratory insufficiency within a decade.
Purpose of the Study:
- To document and discuss anatomic and clinical observations of 28 cases of PLAM.
- To investigate the potential relationship between PLAM and tuberous sclerosis.
Main Methods:
- Retrospective analysis of 28 clinical cases of PLAM.
- Review of radiographic, functional, and pathological findings.
- Comparison with known cases of tuberous sclerosis.
Main Results:
- PLAM presents with characteristic radiographic findings of diffuse lung lesions, bullous changes, and honeycombing.
- Smooth muscle proliferation obstructs airways, venules, and lymphatics, leading to complications like pneumothorax and chylothorax.
- Renal angiomyolipomas and epithelial adenomatoid proliferations were noted, suggesting a possible link to tuberous sclerosis.
Conclusions:
- PLAM is a distinct clinicopathologic entity with a poor prognosis.
- The frequent co-occurrence of PLAM with angiomyolipomas and epithelial changes warrants further investigation into its relationship with tuberous sclerosis, possibly as a forme fruste.