Table 1 Characteristics of patients with LAM at registry
Without Pneumothorax (N = 258) | With Pneumothorax (N = 141) | P value | |
|---|---|---|---|
Age at presentation (year) | 37.5 (30.1–43.7) | 29.5 (24.3–38.6) | < 0.001 |
Age at diagnosis (year) | 40.0 (33–47) | 30.0 (28.5–43) | < 0.001 |
Age at registration (year) | 41(35–48) | 36(31–45) | < 0.001 |
Duration of course (year) | 2.3 (0.5–6.2) | 5.4 (2.2–9.8) | < 0.001 |
Body mass index (kg/m2) | 21.3 (19.5–23.6) | 21.1 (19.5–23.0) | 0.517 |
Smoking history | 5 (1.9) | 5 (3.5) | 0.334 |
Postmenopausal status a | 47 (18.2) | 4 (2.8) | < 0.001 |
TSC-LAM | 35 (13.6) | 19 (13.5) | 1.000 |
Renal angiomyolipoma history | 98 (38.0) | 61 (43.3) | 0.336 |
Chylothorax history | 41 (16.2) | 28 (19.9) | 0.407 |
Retroperitoneal mass history | 54 (23.5) | 23 (18.9) | 0.345 |
Serum VEGF-D (pg/ml) | 1948 (957–3385) | 1720 (900–3147) | 0.502 |
CT grade b | 0.004 c | ||
Grade I | 54 (21.0) | 17 (12.1) | |
Grade II | 48 (18.7) | 18 (12.9) | |
Grade III | 155 (60.3) | 105 (75.0) | |
FEV1% predicted (%) | 80.4 (55.0–93.0) N = 223 | 62.4 (47.6–78.7) N = 94 | < 0.001 |
FVC% predicted (%) | 97.9 (88.5–108.3) N = 223 | 85.7 (74.9–100.2) N = 94 | < 0.001 |
DLCO% predicted (%) | 52.6 (33.2–72.9) N = 211 | 43.9 (35–61.5) N = 88 | 0.022 |
6MWT distance (meter) | 499.5 (454.5–544.3) | 489.0 (432.0–535.0) | 0.207 |