Pulmonary lymphangioleiomyomatosis: case reports

We reviewed the radiologic, physiologic, and pathologic features of biopsy-proved pulmonary lymphangioleiomyomatosis (LAM) in 2 patients. Chest radiographs showed diffuse increased interstitial lung markings with normal or slightly increased lung volume. The prominent computed tomographic (CT) feature was thin-walled cysts throughout the lungs. Pulmonary function test showed a mixed obstructive and restrictive pattern in one and a mainly restrictive pattern in another, which was somewhat different from that of the reported cases. Proliferation of smooth muscle cells in the lymphatics, alveolar septae, vessels, and bronchioles was the pathologic finding