Lymphangioleiomyomatosis

Pulmonary lymphangioleiomyomatosis is an uncommon chronic debilitating disorder of unknown etiology afflicting women of childbearing age, characterized histologically by proliferation of atypical smooth muscle cells in the lung. The clinical features of lymphangioleiomyomatosis can be typical, with airflow limitation, diffuse pulmonary infiltrates on chest radiograph, and numerous lung cysts on computed tomogram of the chest. Diagnosis has been made by open lung biopsy often in connection with pneumothorax. Recently, histologic diagnosis was confirmed by transbronchial biopsy results. Hormonal manipulation therapy had beneficial effects on chylothorax or chylous ascites, whereas pulmonary parenchymal changes appeared to be stationary or progressive. Several prognostic factors were reported among pulmonary function data and histologic findings of open lung biopsy specimens.