Lymphangioleiomyomatosis (LAM) is a rare lung disease of unkwnon etiology that frequently affects women in childbearing age. Clinically it manifests itself by and pneumothorax. Chylous pleural effusions and hemoptisis occur less frenquently. All these fenomena results from the abnormal smooth muscle proliferation in the lung parenchima. Lymph-nodes, small airways and blood vessels. More recently cytogenetic studies disclosed mutations of the TSC-2 gene in cells of renal angiomyolipoma and/or abdominal lymph nodes, pointing towards a possible origin of the hamartomatous lesions. Chest radiography may appear normal or yield reticulonodular infiltrates and signs of hyperinsuflation. At HRCT scans, multiple thin-walled cysts can be seen over the lung parenchyma. Abdominal imaging by either ultrasound or CT may show renal angiomyolipomas and retroperitoneal lymph node enlargement. Meningeomas may also be associated but their presence requires testing for tuberous sclerosis complex. Physiologically LAM is characterized by progressive airflow obstruction, air trapping and gastransfer impairment. Estrogenic suppression with either oophorectomy, deposit progestogens, tamoxifen and GnRh analogs, is still the main treatment. In addition to this therapy lung transplantations have increased patients median survival rates by more than ten years.
Lymphangioleiomyomatosis; Lung neoplasms
