Table 1.
Characteristic | Value |
Age, mean ± SEM, y | |
At presentation with LAM-related symptoms | 36.8 ± 8.6 |
At diagnosis of LAM symptoms | 40.9 ± 8.4 |
At enrollment in the NHLBI LAM protocol | 42.7 ± 8.1 |
At initiation of sirolimus therapy | 47.2 ± 10.0 |
At most recent follow-up visit | 49.7 ± 9.3 |
Interval from diagnosis to initiation of sirolimus therapy, mean ± SD, y | 6.3 ± 6.7 |
Race | |
White | 20 (87) |
African American | 1 (4) |
Asian | 1 (4) |
Unknown | 1 (4) |
Clinical features | |
Tuberous sclerosis complex | 4 (17) |
Postmenopausal | 9 (39) |
History of pneumothorax | 7 (30) |
History of dyspnea | 15 (65) |
Requirement for oxygen therapy | 9 (39) |
Chylous pleural effusions | 10 (43) |
Ascites | 6 (26) |
Lymphangioleiomyomas | 16 (70) |
Lymphadenopathy | 6 (26) |
Renal angiomyolipoma | 7 (30) |
Data are presented as No. (%) unless otherwise indicated. LAM = lymphangioleiomyomatosis; NHLBI = National Heart, Lung, and Blood Institute.