Table 1 Baseline characteristics of LAM patients and a COPD comparison cohort (data are presented as mean ± SD and [median])

n

58

58

–

Female sex, n

58 (100%)

58 (100%)

–

Age, ys

48.2 ± 10.3 [48.4]

59.9 ± 11.6 [59.4]

< 0.001

BMI, kg/m²

22.9 ± 5.9 [21.6]

24.60 ± 7.19 [22.4]

0.071

Smoking status, never/former/current/unknown

35/17/2/4

7/45/6/0

< 0.001

FEV1, l

1.32 ± 0.74 [1.13]

1.00 ± 0.45 [0.91]

0.043

FEV1, %predicted

45.8 ± 24 [42.8]

45.4 ± 21.7 [41.5]

0.965

IVC, l

2.5 ± 1.0 [2.3]

1.9 ± 0.7 [1.9]

0.003

IVC, %predicted

72.1 ± 24.9 [74.4]

51.2 ± 15.9 [53.4]

0.001

DLCO, %pred.

40.7 ± 17.8 [37.2]

44.3 ± 17.1 [43.4]

0.481

PaO2, mmHg

65.7 ± 12.1 [63.9]

59.0 ± 10.4 [59.5]

< 0.001

PaCO2, mmHg

35.3 ± 5.2 [34.1]

41.0 ± 8.8 [38.4]

< 0.001

CRP, mg/l

3.2 ± 4.0 [2.0]

12.5 ± 28.0 [4.2]

< 0.001

Creatinine, mg/dl

0.76 ± 0.24 [0.74]

0.82 ± 0.23 [0.80]

0.028

Long-term oxygen therapy, n

36 (62%)

41 (71%)

0.326

Oxygen supplementation at rest, lpm

1.4 ± 1.5 [1]

1.2 ± 1.1 [1.5]

0.766

Oxygen supplementation during exercise, lpm

2.4 ± 1.9 [2]

1.8 ± 1.4 [2.0]

0.085

6MWD, m

338 ± 167 [330]

287 ± 121 [275]

0.097

6MWD, %predicted

47.7 ± 22.7 [48.3]

47.1 ± 20.2 [42.1]

0.850

SpO₂ nadir during 6MWD

79.4 ± 6.8 [84]

84.9 ± 7.9 [87]

< 0.001

Listed for lung transplantation, n

28 (48%)

23 (40%)

0.350

SF-36 physical health component score

26.9 ± 12.8 [30.2]

30.8 ± 11.7 [29.6]

0.139

SF-36 mental health component score

44.0 ± 13.5 [45.4]

41.2 ± 16.3 [42.8]

0.359