[Pulmonary lymphangioleiomyomatosis: presentation and results of treatment].

Lymphangioleiomyomatosis is a rare lung disease of unknown aetiology that affects only women. Eight premenopausal women with LAM confirmed by lung biopsy specimens were observed in 1984-2001. The most common presenting feature was exertional dyspnea (6) followed by chylous pleural effusions and pneumothoraces. In two women severe airflow obstruction was observed at presentation. HRCT revealed characteristic cysts in all cases. All women were given hormonal therapy (tamoxifen, medroxyprogesterone). The best results of treatment were achieved in cases with chylothoraces.