Patients with pulmonary involvement in lymphangiomyomatosis usually experience progressive decline in lung function. Some patients respond to progesterone or anti-estrogen therapy. Sirolimus therapy has been used recently and it appears to improve the quality of life and slow down pulmonary insufficiency. The definitive treatment is heart-lung transplantation; although recurrences have been noted in the transplanted lung. The 10-yr mortality is reported to be between 30% and 70%.