Dyspnea in a young woman with chest radiography showing large lung volumes

Lymphangioleiomyomatosis is a rare pulmonary disorder that almost exclusively affects women of childbearing age. Histopathological findings of LAM are very similar to tuberous sclerosis, raising the possibility that LAM is a forme fruste of TS. Chest radiographic findings of LAM include large lung volumes with bilaterally symmetric linear and/or reticular abnormalities or direct visualization of the pulmonary cysts. HRCT in patients with LAM shows numerous thin-walled, regular cysts without a zonal predominance. Pleural effusions may occur in patients with LAM, and pneumothorax may be the presenting feature of the disease in a number of patients. The diagnosis of LAM may be established with transbronchial or surgical lung biopsy, although a tissue diagnosis may be avoided in patients with sufficiently characteristic clinical, laboratory, and HRCT findings.