Table 1 Baseline characteristics of the patients*
Characteristic | N = 58 |
|---|---|
Age, yrs | 35.6 ± 10.8 |
Female sex, n (%) | 31 (53) |
Race, n (%) | |
White | 55 (95) |
Other | 3 (5) |
Smoking history, pack-years | 21 ± 17 |
At diagnosis, n (%) | |
Current smokers | 56 (97) |
Ex-smokers | 2 (3) |
At inclusion, n (%) | |
Current smokers | 39 (67) |
Clinical features, n (%) | |
Asymptomatic | 21 (36) |
Cough | 30 (52) |
Dyspnoea | 26 (45) |
NYHA class II/III | 23/2 |
History of pneumothorax | 11 (19) |
Constitutional symptoms† | 6 (10) |
Pulmonary function testing | |
FEV1 | |
Volume, ml | 2974 ± 839 |
% predicted | 87 ± 18 |
FVC | |
Volume, ml | 3787 ± 1036 |
% predicted | 93 ± 18 |
FEV1/FVC, % | 75.5 ± 8.8 |
TLC, % predicted | 100.6 ± 15.3 |
RV, % predicted | 116.5 ± 36.2 |
RV/TLC, % predicted | 114.4 ± 30.2 |
DLCO, % predicted | 64.3 ± 13.2 |
Normal lung function, n (%) | 7 (12) |
Restriction, n (%)‡ | 5 (9) |
Obstruction, n (%)‡ | 15 (26) |
Bronchial hyperreactivity, n (%)‡ | 6 (10) |
DLCO <80% predicted, n (%)‡ | 49 (87) |
PaO2, mm Hg | 87 ± 10 |
6-Minute walk distance, m | 514 ± 93 |
HRCT nodular score§ | 8 ± 4.5 |
Nodular score subgroup, n (%) | |
Low | 26 (46) |
Intermediate | 20 (36) |
High | 10 (18) |
HRCT cystic score§ | 8.2 ± 5 |
Cystic score subgroup, n (%) | |
Low | 30 (54) |
Intermediate | 18 (32) |
High | 6 (11) |
Very high | 2 (3) |
SGRQ score║ | 20.2 ± 18.8 |