From: Efficacy and safety of low-dose Sirolimus in Lymphangioleiomyomatosis
Characteristic | Total | Low-dose | Conventional-dose |
---|---|---|---|
Number of patients | 39 | 20 | 19 |
Age, years | 34.8 ± 8.3 | 34.0 ± 8.7 | 35.6 ± 8.1 |
Female | 39 (100.0) | 20 (100.0) | 19 (100.0) |
Smoking | |||
Never | 36 (92.3) | 19 (95.0) | 17 (89.5) |
Former | 3 (7.7) | 1 (5.0) | 2 (10.5) |
Current | 0 (0.0) | 0 (0.0) | 0 (0.0) |
TSC | 6 (15.4) | 6 (30.0)* | 0 (0.0) |
Postmenopausal state | 2 (5.1) | 1 (5.0) | 1 (5.3) |
Previous treatment | |||
Medroxyprogesterone | 14 (35.9) | 7 (35.0) | 7 (36.8) |
LHRH | 4 (10.3) | 1 (5.0) | 3 (15.8) |
Bilateral oophorectomy | 1 (2.6) | 1 (5.0) | 0 (0.0) |
History of extrapulmonary manifestation | |||
Pneumothorax | 24 (61.5) | 15 (75.0) | 9 (47.4) |
Angiomyolipoma | 14 (35.9) | 9 (45.0) | 5 (26.3) |
Lymphangioleiomyoma | 5 (12.9) | 1 (5.0) | 4 (21.1) |
Chylothorax | 3 (7.7) | 2 (10.0) | 1 (5.3) |
Lung function | |||
FEV1, % predicted | 67.7 ± 26.4 | 62.9 ± 27.4 | 72.3 ± 2.3 |
FVC, % predicted | 86.2 ± 14.9 | 83.3 ± 13.8 | 88.9 ± 15.7 |
DLco, % predicted | 51.7 ± 21.8 | 52.6 ± 23.1 | 50.9 ± 21.2 |
FEV1/FVC ratio | 66.2 ± 20.8 | 64.1 ± 2 4.1 | 68.2 ± 17.6 |
6MWT | |||
Distance, meter | 456.9 ± 103.6 | 458.4 ± 103.5 | 455.1 ± 106.9 |
Initial SaO2, % | 97.6 ± 1.7 | 97.7 ± 1.7 | 97.6 ± 1.7 |
Lowest SaO2, % | 93.7 ± 4.7 | 94.5 ± 4.9 | 92.8 ± 4.5 |
Sirolimus level, ng/mL | 5.5 ± 2.8 | 3.5 ± 1.3* | 7.7 ± 2.3 |