Table 1.
Baseline Characteristics of the Cohort (n = 25)
| Variable | Data |
|---|---|
| Age, y | 51 (47-58) |
| Race | |
| White | 23 (92) |
| Asian | 2 (8) |
| Ethnicity | |
| Not Hispanic or Latino | 25 (100) |
| Lymphangioleiomyomatosis subtype | |
| Sporadic lymphangioleiomyomatosis | 22 (88) |
| TSC lymphangioleiomyomatosis | 3 (12) |
| Menopausal status | |
| Premenopausal | 13 (52) |
| Postmenopausal | 12 (48) |
| Prior pneumothorax (n = 20) | 10 (50) |
| Dyspnea on exertion | 25 (100) |
| Supplemental oxygen use | 11 (44) |
| FEV1 | |
| L | 1.72 (1.10-2.36) |
| % Predicted | 64.5 (42-80) |
| FVC | . . . |
| L | 2.99 (2.70-3.96) |
| % Predicted | 96 (73.5-107.5) |
| Dlco | |
| mL/mm Hg/min | 14.68 (7.07-17.3) |
| % Predicted | 37 (26-64) |
| Serum VEGF-D, pg/mL | 617 (486-787) |
| Sirolimus dose, mg/d | 2 (1-2) |
| Sirolimus trough, ng/mL | 6.3 (4.2-7.7) |
| SGRQ score | 42 (39-46.5) |
| SOBQ score | 29 (19-36.5) |
| ATAQ-LAM score | 72 (51-95.5) |
| EQVAS score | 80 (65-85) |
Data are presented as No. (%) or median (interquartile range). ATAQ-LAM = A Tool to Assess Quality of Life in LAM [lymphangioleiomyomatosis]; Dlco = diffusion capacity of the lungs for carbon monoxide; EQVAS = EuroQol visual analog scale; SGRQ = St. George’s Respiratory Questionnaire; SOBQ = University of California, San Diego, Shortness of Breath Questionnaire; TSC = tuberous sclerosis complex; VEGF-D = vascular endothelial growth factor D.