A report of two long-surviving cases of pulmonary lymphangioleiomyomatosis and the response to progesterone therapy.

Two cases of pulmonary lymphangioleiomyomatosis, with survival between 12 and 28 years are described. Both were treated with medroxyprogesterone acetate with a slight initial improvement in both lung function and exercise tolerance, and no further deterioration during the period of follow-up of 10-20 months. The results are discussed and other reports of hormonal treatment of this rare condition are reviewed.