Table 1 Comparison of baseline characteristics and treatment between the non-survivors and survivors among patients with LAM
From: Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis
Characteristic | Total | Non-survivors | Survivors | P-value |
|---|---|---|---|---|
Number of patients | 104 | 9 | 95 | |
Age, years | 40.3 ± 10.7 | 45.8 ± 13.0 | 39.8 ± 9.9 | 0.178 |
Female | 104 (100) | 9 (100) | 95 (100) | > 0.999 |
Ever-smoker | 10 (9.6) | 1 (11.1) | 9 (9.5) | > 0.999 |
TSC | 13 (12.5) | 1 (11.1) | 12 (12.6) | > 0.999 |
Pneumothorax | 44 (42.3) | 4 (44.4) | 40 (42.1) | 0.944 |
Chylothorax | 2 (1.9) | 0 (0.0) | 2 (2.1) | > 0.999 |
Extrapulmonary manifestations | 48 (46.2) | 4 (44.4) | 44 (46.3) | > 0.999 |
Angiomyolipoma | 32 (30.5) | 3 (33.3) | 29 (30.5) | > 0.999 |
Lymphangioleiomyoma | 18 (17.3) | 1 (11.1) | 17 (17.9) | > 0.999 |
Lung function, % predicted | ||||
FEV1 | 77.3 ± 22.2 | 55.4 ± 24.7 | 79.3 ± 19.6 | 0.005 |
FVC | 88.5 ± 16.8 | 88.1 ± 21.3 | 88.6 ± 13.8 | 0.939 |
DLCO | 63.5 ± 249 | 40.1 ± 19.7 | 65.7 ± 23.4 | 0.003 |
TLC | 97.8 ± 16.5 | 104.9 ± 18.0 | 97.3 ± 12.9 | 0.102 |
FEV1/FVC | 87.3 ± 19.1 | 64.8 ± 25.2 | 89.4 ± 17.1 | 0.003 |
RV | 102.3 ± 35.5 | 123.9 ± 24.2 | 99.5 ± 28.8 | 0.010 |
FEF25–75% | 67.3 ± 29.9 | 29.6 ± 21.8 | 71.0 ± 39.5 | 0.448 |
6MWT | ||||
Distance, m | 467.5 ± 114.6 | 320.5 ± 168.9 | 481.0 ± 86.5 | 0.004 |
Lowest SpO2, % | 94.4 ± 5.8 | 87.4 ± 12.2 | 95.0 ± 4.4 | 0.048 |
Treatment | ||||
Medroxyprogesterone | 31 (29.8) | 4 (44.4) | 27 (28.4) | 0.446 |
LHRH | 2 (1.9) | 0 (0.0) | 2 (2.1) | > 0.999 |
Bilateral oophorectomy | 5 (4.8) | 0 (0.0) | 5 (5.3) | > 0.999 |
Sirolimus therapy | 62 (59.6) | 4 (44.4) | 58 (61.1) | 0.480 |
Dose per day, mg | 1.6 ± 0.6 | 1.4 ± 0.5 | 1.6 ± 0.6 | 0.490 |
TDM, ng/mL | 5.9 ± 3.0 | 4.3 ± 1.8 | 6.0 ± 3.1 | 0.361 |
Duration, years | 4.8 (1.5–6.2) | 1.5 (0.3–3.4) | 5.0 (1–6.7) | 0.054 |