Lymphangioleiomyomatosis--treatment with progesterone

Lymphangioleiomyomatosis is a rare devastating disease affecting women mostly of child-bearing age. It presents with spontaneous pneumothorax, chylous effusions, hemoptysis and progressive breathlessness. Most patients die from respiratory failure within 10 years. There are no controlled studies on the efficacy of various treatment regimens. We report our experience with progesterone therapy in three patients. Two failed to respond, one died about 11 years after presentation and another after 5 1/2 years. The third patient has survived 11 years after onset of disease.