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Lung transplantation for lymphangioleiomyomatosis
A Boehler1, R Speich, E W Russi
1Department of Internal Medicine, University Hospital of Zurich, Switzerland.
The New England Journal of Medicine
|October 24, 1996
Summary
Lung transplantation offers a valuable treatment option for lymphangioleiomyomatosis (LAM), a rare lung disease. Despite frequent complications, survival rates improve, and lung function is enhanced post-transplant for LAM patients.
Area of Science:
- Pulmonology
- Thoracic Surgery
- Transplant Immunology
Background:
- Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease affecting women of reproductive age and individuals with tuberous sclerosis.
- LAM leads to respiratory failure and is often fatal.
- Lung transplantation is an emerging therapeutic option for advanced LAM.
Purpose of the Study:
- To evaluate the efficacy and outcomes of lung transplantation in patients with end-stage lymphangioleiomyomatosis.
- To assess survival rates and functional improvements following lung transplantation for LAM.
Main Methods:
- Retrospective questionnaire study of 34 patients with end-stage LAM who underwent lung transplantation between 1983 and 1995.
- Data collected from 16 transplant centers.
- Survival analysis using the Kaplan-Meier method.
Main Results:
- Actuarial survival was 69% at one year and 58% at two years post-transplant.
- Forced expiratory volume in one second (FEV1) significantly improved from 24% to 48% of predicted values six months after transplantation.
- Early and late deaths were primarily due to acute lung injury, infections, and bronchiolitis obliterans. Complications included pleural adhesions, pneumothorax, and recurrent LAM.
Conclusions:
- Lung transplantation can be a beneficial therapy for patients suffering from end-stage lymphangioleiomyomatosis.
- Despite significant disease-related complications, transplantation offers a chance for improved lung function and survival.