Pulmonary Lymphangiomyomatosis Responsive to Progesterone

PULMONARY lymphangiomyomatosis occurs almost exclusively in women of reproductive age. 1 The principal manifestation is a proliferation of immature smooth muscle throughout the peribronchial, perivascular, and perilymphatic regions of the lung. This disorder is usually part of a more inclusive syndrome causing similar lesions in extrapulmonary lymphatics and lymph nodes. Similar pulmonary lesions are a component of tuberous sclerosis, but the relation between the isolated pulmonary manifestations and the generalized disease requires further clarification. 2 3 4 5 6 The most frequent problem reported by patients with lymphangiomyomatosis is progressive dyspnea. Pneumothorax, chylous effusions, and hemoptysis are frequent complications. The roentgenographic appearance is characteristic although not . . .