Table 2. Pre- and post-doxycycline functional parameters, matrix metalloproteinase levels, VEGF-D levels, and Medical Outcomes Study 36-item Short-form Health Survey scores in the 13 lymphangioleiomyomatosis patients allocated to the doxycycline-responder groupa on the basis of their response to doxycycline (as determined by the variation in FEV1). b .
Variable | Doxy-R group | p | ||
Pre-doxycycline | Post-doxycycline | |||
Pulmonary function test | ||||
FVC, L | 3.0 ± 0.3 | 3.2 ± 0.3 | 0.023 | |
FVC, % of predicted | 90 ± 13 | 95 ± 12 | 0.026 | |
FEV1, L | 2.31 ± 0.40 | 2.40 ± 0.40 | 0.003 | |
FEV1, % of predicted | 84 ± 14 | 86 ± 14 | 0.002 | |
FEV1/FVC ratio | 0.77 ± 0.10 | 0.76 ± 0.10 | 0.173 | |
TLC, L | 4.6 ± 0.5 | 4.8 ± 0.6 | 0.310 | |
TLC, % of predicted | 98 ± 13 | 101 ± 15 | 0.327 | |
RV, L | 1.6 ± 0.5 | 1.6 ± 0.6 | 0.956 | |
RV, % of predicted | 117 ± 43 | 118 ± 47 | 0.940 | |
RV/TLC ratio | 0.34 ± 0.08 | 0.33 ± 0.08 | 0.640 | |
DLCO, mL/min/mmHg | 18.5 ± 6.0 | 18.0 ± 5.0 | 0.514 | |
DLCO, % of predicted | 72 ± 20 | 70 ± 18 | 0.448 | |
Six-minute walk test | ||||
Distance, m | 515 ± 66 | 523 ± 52 | 0.633 | |
Minimum SpO2, % c | 94 (93-95) | 94 (91-95) | 0.500 | |
Biomarkers c | ||||
Serum MMP-9, ng/mL | 837 (716-1,033) | 1,103 (773-1,237) | 0.328 | |
Urinary MMP-9, pg/mL | 8,905 (5,607-21,829) | 6,735 (674-8,983) | 0.050 | |
Serum MMP-2, pg/mL | 0 (0-182) | 0 (0-40) | 0.144 | |
VEGF-D, pg/mL | 413 (277-2,113) | 328 (257-2,008) | 0.374 | |
SF-36 domains c | ||||
Physical functioning | 75 (65-85) | 80 (75-95) | 0.281 | |
Role-physical | 50 (50-50) | 100 (50-100) | 0.020 | |
Bodily pain | 57.5 (55-90) | 70 (55-77.5) | 1.000 | |
General health | 80 (45-90) | 75 (70-85) | 0.412 | |
Vitality | 70 (45-90) | 70 (70-85) | 0.428 | |
Role-emotional | 33 (33-100) | 100 (33-100) | 0.158 | |
Mental health | 68 (40-84) | 76 (52-88) | 0.051 | |
Social functioning | 75.0 (50.0-88.5) | 87.5 (62.5-100) | 0.040 |
Doxy-R group: : doxycycline-responder group; minimum SpO2: minimum SpO2 sustained for 10 s;
: matrix metalloproteinase;
SF-36: : Medical Outcomes Study 36-item Short-form Health Survey.
Group of patients who responded to treatment with doxycycline, as evidenced by increased or stable FEV1 at doxycycline treatment month 12 in comparison with FEV1 at baseline.
Values expressed as mean ± SD, except where otherwise indicated.
Values expressed as median (interquartile range).