Table 2.
Baseline characteristics categorised according to disease entity.
| Variable* |
LAM |
PLCH |
BHDS |
Unclassified |
All |
|
|---|---|---|---|---|---|---|
| N (%) | 6 (50.0) | 3 (25.0) | 2 (16.7) | 1 (8.3) | 12 (100.0) | |
| Gender (F:M) | 6:0 | 2:1 | 0:2 | 1:0 | 9:3 | |
| Age (years) | 54.0 ± 18.3 | 43.7 ± 17.5 | 47.5 ± 2.1 | 34.0 ± 0.0 | 48.7 ± 15.8 | |
| BMI (kg m–2) | 23.3 ± 5.3 | 23.7 ± 2.5 | 24.5 ± 0.7 | 18.0 ± 0.0 | 23.2 ± 4.1 | |
| FEV1 (litres) | 1.6 ± 1.1 | 1.9 ± 0.8 | 4.2 ± 0.5 | 3.3 ± 0.0 | 2.3 ± 1.3 | |
| FEV1 (% pred.) | 56.8 ± 29.5 | 63.0 ± 24.3 | 97.0 ± 12.7 | 91.0 ± 0.0 | 67.9 ± 28.2 | |
| FVC (litres) | 3.0 ± 1.4 | 3.4 ± 1.2 | 6.2 ± 0.3 | 4.7 ± 0.0 | 3.8 ± 1.6 | |
| FVC (% pred.) | 92.3 ± 30.4 | 91.7 ± 12.4 | 113.5 ± 6.4 | 112.0 ± 0.0 | 97.3 ± 23.3 | |
| FEV1/FVC (%) | 0.5 ± 0.2 | 0.6 ± 0.1 | 0.7 ± 0.1 | 0.7 ± 0.0 | 0.6 ± 02 | |
| TLC (litres) | 5.0 ± 0.9 | 5.5 ± 1.5 | 8.8 ± 0.1 | 6.3 ± 0.0 | 6.0 ± 1.7 | |
| TLC (% pred.) | 94.0 ± 13.3 | 98.0 ± 6.6 | 107.0 ± 2.8 | 104.0 ± 0.0 | 98.4 ± 10.4 | |
| RV (litres) | 1.7 ± 0.3 | 2.0 ± 0.6 | 2.5 ± 0.1 | 1.7 ± 0.0 | 3.6 ± 5.7 | |
| RV (% pred.) | 95.4 ± 8.7 | 115.3 ± 35.6 | 107.5 ± 0.7 | 99.0 ± 0.0 | 103.4 ± 19.1 | |
| DLCO (% pred.) | 56.6 ± 10.4 | 54.0 ± 5.6 | 97.5 ± 7.8 | 77. ± 0.0 | 65.2 ± 18.8 | |
| KCO (% pred.) | 62.2 ± 5.4 | 56.7 ± 4.9 | 92.0 ± 7.1 | 76.0 ± 0.0 | 67.4 ± 14.1 | |
| Smoking (%) | Never | 5 (83.3) | 0 (0.0) | 2 (100.0) | 0 (0.0) | 7 (58.3) |
| Former | 0 (0.0) | 2 (66.7) | 0 (0.0) | 0 (0.0) | 2 (16.7) | |
| Present | 1 (16.7) | 1 (33.3) | 0 (0.0) | 1 (100.0) | 3 (25.0) | |
| Disease duration# | 84.7 ± 49.5 | 43.3 ± 62.0 | 4 ± 0.0 | 33 ± 0.0 | 61.3 ± 49.2 | |
Abbreviations: M = male. F = female. SD = standard deviation. BMI = body mass index. FEV1 = forced expiratory volume in 1 sec. FVC = forced vital capacity. TLC = total lung capacity. RV = residual volume. DLCO = diffusion capacity of the lung for carbon monoxide. KCO = carbon monoxide coefficient (diffusion constant).
* Continuous data are expressed as mean ± standard deviation (SD); categorical data as number and percentage (%).
# Disease duration in months from time of diagnosis to study date.