[Clinical analysis of pulmonary lymphangioleiomyomatosis]

To study the clinical features, treatment, and prognosis of pulmonary lymphangioleiomyomatosis (LAM).

A retrospective analysis of 16 cases of LAM was made, among which two were diagnosed and treated in the Chinese PLA General Hospital, and the clinical data of the other fourteen cases were studied by literature review.

Among the 16 patients, 15 were female (94%) and 1 was male (6%). The age of onset ranged 5 - 69 years, with an average age of onset of 35 +/- 15 years. Thirteen patients (81%) were women of childbearing age. The main clinical menifestations included dyspnea (15 cases, 94%), hemoptysis (10 cases, 62%), pneumothorax (7 cases, 44%), and chylothorax (7 cases, 44%). High-resolution computerized tomography conducted in 14 cases showed thin-walled air-filled cysts throughout both lungs. Seven patients took pulmonary function test with the results of obstructive ventilatory disturbance in 5 cases, mixed ventilatory disturbance in 2 cases, and reduction of carbon dioxide duffusing capacity in all cases. Arterial blood gas analysis was made among 11 patients and showed hypoxemia (blood oxygen < 80 mm Hg) in 8 cases and respiratory failure in 5 cases. All of the patients were misdiagnosed as other diseases at the first visit. The time between onset of symptoms and confirmation of diagnosis ranged 2 - 276 months (median 28 months). Six patients were treated with medroxyprogesterone for 6-50 months. Among them two patient' condition remained stable, two patients' condition was worsened, and two died. Six out of the 16 patients (38%) died of respiratory failure, pneumothorax, or lung infection 1.5 to 26 years after the onset.

The main clinical manifestations of LAM are dyspnea, hemoptysis, pneumothorax, and chylothorax. Medroxyprogesterone is of little curative effect on LAM. The main task in LAM research is to find out its etiology and effective treatment.