What's the treatment for lymphangioleiomyomatosis?
Lymphangioleiomyomatosis is generally progressive, with a life expectancy between 25-50 years for patients undergoing treatment. Therapeutic treatments have focused on reducing the production or effects of estrogen. It has been thought that
the hormone estrogen might be involved in the abnormal muscle cell growth that characterizes the disease, just as it is in the growth of smooth muscle in the uterus in a woman's childbearing years. Although there is no firm evidence that there is a relationship between estrogen and LAM, the treatment of ther disease has focused on reducing the production or effects of estrogen. The response to treatment has been highly individual, and no therapy has been found to be effective for all LAM patients. Oxygen therapy may be necessary if the disease continues to worsen and lung function is impaired.
Oxygen therapy may become necessary if the disease continues to worsen and lung function is impaired. Management of recurrent pneumothoraces or pleural effusions may require surgical intervention; in addition, patients with AML can develop complications (eg, hemorrhage), requiring intervention. For LAM patients with severe disease, lung transplantation is an established therapy. One year survival following transplant is approximately 70 percent, and 3-year survival is approximately 50 percent. |
