Table 1.
Demographic and clinical data | ||
Age (yrs) | 46 ± 9 | |
Female (n, %) | 28 (82.4%) | |
Smoking (n, %) | 18 (52.9%) | |
Pack-years | 34 ± 22 | |
Time from diagnosis (yrs) | 2.5 (1–13) | |
Diagnosis | ||
Clinical-tomographic (n, %) | 15 (44.1%) | |
Lung biopsy (n, %) | 18 (52.9%) | |
Renal biopsy (n, %) | 1 (3%) | |
Dyspnea (n, %) | 30 (88.2%) | |
mMRC | 2 (1–2) | |
History of pneumothorax (n, %) | 12 (35.3%) | |
SpO2 at rest (%) | 93 (89–95) | |
Use of supplemental oxygen at rest (n, %) | 12 (35.3%) | |
Pulmonary function tests | ||
FEV1 (L) | 1.32 ± 61 | |
FEV1 (% predicted) | 47 ± 20 | |
FVC (L) | 2.47 ± 0.82 | |
FVC (% predicted) | 71 ± 20 | |
FEV1/FVC | 0.53 ± 0.15 | |
TLC (L) | 5.13 ± 1.1 | |
TLC (% predicted) | 104 ± 17 | |
RV (L) | 2.60 ± 0.91 | |
RV (% predicted) | 177 ± 62 | |
RV/TLC | 0.51 ± 0.12 | |
DLCO (mL/min/mm Hg) | 10.08 ± 5.84 | |
DLCO (% predicted) | 38 ± 21 | |
Six-minute walk test | ||
Distance (m) | 392 ± 116 | |
Distance (% predicted) | 96 ± 54 | |
Minimum SpO2 (%) | 79 ± 9 | |
Oxygen desaturation (%) | −12 ± 7 | |
Borg dyspnea score | 7 (3–8) | |
Borg leg discomfort score | 4 (0–7) |
Data are presented as mean ± SD, median (25th–75th percentile), or numbers (percentiles). DLCO = lung diffusing capacity for carbon monoxide, FEV1 = forced expiratory volume in the first second, FVC = forced vital capacity, mMRC = modified Medical Research Council Dyspnea scale, RV = residual volume, SpO2 = oxygen saturation, TLC = total lung capacity.