Table 1.
Clinical Characteristics of Patients With LAM, From Visits When They Were Not Taking Sirolimus, Divided According to Categories of Serum CA-125 Levels
| Characteristic | All Normal CA-125 Levels | At Least One Above-Normal CA-125 Level | At Least Two Above-Normal CA-125 Levels | All Above-Normal CA-125 Levels |
|---|---|---|---|---|
| Visits | 8.6 ± 0.4 (1-22) [181] | 9.0 ± 0.6 (1-20) [60] | 8.9 ± 0.8 (3-20) [40] | 6.5 ± 1.1 (1-17) [17] |
| Initial age, y | 45.3 ± 0.7 (21-76) [181] | 41.0 ± 1.1 (25-63) [60] | 42.8 ± 1.2 (25-63) [40] | 39.6 ± 2.0 (25-57) [17] |
| Initial FEV1 (percent predicted) | 80.2 ± 1.9 (20-133) [175] | 70.9 ± 3.5 (19-116) [56] | 67.4 ± 4.2 (19-115) [36] | 68.1 ± 5.8 (35-115) [15] |
| Initial Dlco (percent predicted) | 77.7 ± 1.9 (25-138) [174] | 64.8 ± 3.3 (22-116) [56] | 61.7 ± 4.1 (23-116) [36] | 59.4 ± 6.0 (30-107) [15] |
| Final age, y | 51.4 ± 0.7 (24-79) [181] | 46.5 ± 1.3 (26-71) [60] | 48.2 ± 1.5 (26-71) [40] | 43.3 ± 2.5 (26-62) [17] |
| Final FEV1 (percent predicted) | 69.9 ± 2.2 (13-134) [161] | 62.5 ± 3.5 (22-134) [57] | 64.1 ± 4.1 (22-109) [37] | 63.3 ± 6.2 (22-109) [17] |
| Final Dlco (percent predicted) | 66.1 ± 2.1 (6-120) [152] | 53.8 ± 3.3 (21-121) [56] | 54.4 ± 3.9 (24-111) [37] | 50.7 ± 5.1 (26-101) [17] |
| Lymphatic involvementa,b | ||||
| No | 111 (61.3) | 16 (26.7) | 7 (17.5) | 1 (5.9) |
| Yes | 70 (38.7) | 44 (73.3) | 33 (82.5) | 16 (94.1) |
| Pleural effusionb,c | ||||
| No | 171 (95.5)c | 30 (50.0) | 16 (40.0) | 2 (11.8) |
| Yes | 8 (4.5) | 30 (50.0) | 24 (60.0) | 15 (88.2) |
| TSC | ||||
| No | 146 (80.7) | 47 (78.3) | 34 (85.0) | 14 (82.4) |
| Yes | 35 (19.3) | 13 (21.7) | 6 (15.0) | 3 (17.6) |
| AMLb | ||||
| No | 90 (49.7) | 30 (50.0) | 22 (55.0) | 9 (52.9) |
| Yes | 91 (50.3) | 30 (50.0) | 18 (45.0) | 8 (47.1) |
Data are presented as average ± SEM (range) [No.] or No. (%). In a univariate analysis, with cancer antigen 125 (CA-125) as a continuous variable, higher serum CA-125 levels were significantly associated with lower percent predicted FEV1 (P < .001) and diffusing capacity for carbon monoxide (Dlco) (P < .001), younger age (P < .044), premenopause (P = .001), lymphatic involvement (P < .001), and pleural effusions (P < .001). Tuberous sclerosis complex (TSC) and the presence of angiomyolipomas (AMLs) were not significantly associated with serum CA-125 levels. After multivariate analysis considering the variables associated with CA-125 levels, higher CA-125 levels were significantly associated with lower percent predicted FEV1 (P < .001), premenopause (P < .001), and a history of pleural effusion (P < .001). Percent predicted Dlco was also a significant predictor of CA-125 level in the multivariate model (P = .022), but its presence in the model reduced the effect of FEV1 (P = .005). Correlations were found across the whole study population; patients in this table were grouped into categories for illustrative purposes only.
Lymphatic involvement includes ascites, pleural effusions, adenopathy, chylous effusions, and/or lymphangioleiomyomas.
Data indicate a history of lymphatic involvement, pleural effusions, and/or AML.
Data on pleural effusion history are unavailable for two patients.