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Table 3 Paired comparisons of functional tests and serum VEGF-D levels between the baseline and annual time points during sirolimus treatment

From: Long-term efficacy and safety of sirolimus therapy in patients with lymphangioleiomyomatosis

Variable 1 year 2 years 3 years 4 years
Baseline After sirolimus P value Baseline After sirolimus P value Baseline After sirolimus P value Baseline After sirolimus P value
Pulmonary ventilation function N = 67 N = 36 N = 22 N = 11
 FEV1 (ml) 1590 ± 690 1640 ± 710 0.045 1640 ± 610 1610 ± 690 0.462 1470 ± 660 1520 ± 740 0.560 1440 ± 590 1310 ± 440 0.099
 FVC (ml) 2640 ± 760 2830 ± 700 0.004 2700 ± 620 2880 ± 560 0.044 2680 ± 660 2780 ± 630 0.398 2570 ± 560 2800 ± 380 0.149
 FEV1%pred 55.97 ± 23.75 59.28 ± 24.3 0.004 57.48 ± 21.97 58.28 ± 24.6 0.705 54.22 ± 23 57.02 ± 26.44 0.346 50.38 ± 21.09 49.19 ± 17.75 0.557
 FVC%pred 80.85 ± 22.37 87.87 ± 24.06 0.005 83.2 ± 17.94 89.68 ± 18.03 0.019 83.56 ± 22.27 92.08 ± 22.08 0.054 81.32 ± 18.72 90.84 ± 15.3 0.128
 FEV1/FVC 56.27 ± 17.42 56.33 ± 18.02 0.966 58.32 ± 20.66 53.72 ± 21.82 0.000 53.59 ± 22.1 52.94 ± 23.07 0.812 54.04 ± 19.9 45.25 ± 15.17 0.014
Gas exchange N = 48 N = 26 N = 19 N = 7
 DLCO%pred 40.62 ± 20.34 41.23 ± 20.42 0.378 46.94 ± 23.87 45.23 ± 22.26 0.197 32.86 ± 19.01 35.64 ± 16.91 0.104 34.46 ± 17.65 30.49 ± 12.71 0.109
Arterial blood gas N = 69 N = 39 N = 31 N = 17
 PaO2 (mmHg) 66.99 ± 12.58 71.21 ± 14.98 0.006 68.14 ± 12.61 75.68 ± 16.5 <  0.001 66.34 ± 10.68 70.63 ± 13.36 0.026 69.24 ± 9.662 74.09 ± 11.02 0.084
 P(A-a) O2 (mmHg) 41.29 ± 13.51 36.67 ± 16.07 0.013 40.6 ± 13.28 32.97 ± 17.23 <  0.001 42.63 ± 12.13 36.76 ± 13.89 0.007 37.89 ± 12.72 35.61 ± 11.6 0.504
6-min walk test N = 67 N = 34 N = 27 N = 12
 6MWD (m) 417 ± 122 461 ± 105 <  0.001 420 ± 120 472 ± 98 <  0.001 408 ± 105 459 ± 107 0.004 379 ± 145 436 ± 96 0.1572
SGRQ N = 63 N = 33 N = 27 N = 12
 Total score 46.11 ± 22.11 38.37 ± 22 <  0.001 42.85 ± 21.83 31.21 ± 21.79 <  0.001 48.04 ± 23.24 38.93 ± 21.94 0.0078 53.25 ± 24.51 49.08 ± 21.95 0.5019
Serum VEGF-D level N = 89 N = 50 N = 34 N = 15
 VEGF-D (pg/ml) 3594 ± 3156 2001 ± 1972 <  0.001 3711 ± 3714 2133 ± 2550 <  0.001 3280 ± 2228 1761 ± 1255 <  0.001 3445 ± 2453 1851 ± 1355 <  0.001