Subjects (n) | 405 | 181 | 163 | |
Age (years) | 53.5±15.9 | 55.4±14.7 | 51.0±17.3 | 0.015 |
Sex, male | 218 (53.8) | 95 (52.5) | 87 (53.4) | 0.869 |
BMI (kg·m−2) | 26.4±4.8 | 26.2±4.9 | 26.3±4.9 | 0.730 |
Smokers | 33 (8) | 3 (2.8) | 22 (23.2) | <0.001 |
Ex-smokers | 222 (54.4) | 111 (61.3) | 3 (44.9) | |
Never-smokers | 149 (36.8) | 66 (36.7) | 68 (41) | |
Tobacco exposure (pack-years) | 24.5±21.9 | 20.3±13.8 | 25.2±25.1 | 0.953 |
Chronic respiratory disease | 267 (66.4) | 146 (81.6) | 82 (50.6) | <0.001 |
COPD | 175 (43.5) | 108 (60.3) | 42 (25.9) | <0.001 |
Emphysema | 163 (40.5) | 107 (59.8) | 40 (24.7) | <0.001 |
Bronchiectasis | 83 (20.6) | 58 (32.4) | 16 (10) | <0.001 |
Asthma | 55 (13.7) | 24 (13.4) | 22 (13.6) | 0.963 |
Liver disease | 42 (10.6) | 24 (13.4) | 16 (10) | 0.322 |
Age at onset of symptoms (years) | 46.1±17.2 | 44.2±16.3 | 47.9±17.5 | 0.063 |
Age at diagnosis of AATD (years) | 46.4±17.4 | 44.5±17.2 | 46.8±18 | 0.354 |
Index case | 286 (70.6) | 135 (74.6) | 106 (65) | 0.053 |
Family screening | 98 (24) | 39 (21.5) | 45 (27.6) | 0.121 |
Charlson index (age corrected) | 3.1±2.1 | 3.2±2 | 2.9±2 | 0.347 |
Cardiovascular disease# | 89 (22.5) | 47 (26.4) | 25 (15.7) | 0.017 |
FVC % pred | 99.4±22.6 | 96.1±26.2 | 103±17.4 | 0.011 |
FEV1 % pred | 81.7±31.4 | 69.8±32.1 | 93.3±26 | <0.001 |
FEV1/FVC % pred | 65±19 | 57±19 | 72±18 | <0.001 |
KCO % pred | 76.8±23.4 | 67.6±22.5 | 84.7±20.8 | <0.001 |
Augmentation therapy | 114 (28.9) | 95 (54) | 13 (8.2) | 0.001 |
AAT (mg·dL−1) | 44.5±24.4 | 24.9±17.6 | 56.7±11.8 | <0.001 |
Number of exacerbations previous year | 0.44±1 | 0.66±1.2 | 0.28±0.8 | <0.001 |
History of pneumonia | 77 (19.3) | 44 (24.6) | 21 (13.1) | 0.007 |
CAT | 7.4±7.2 | 9.4±7.8 | 5.6±6.2 | <0.001 |
BODEx | 1.2±1.8 | 1.8±2 | 0.6 (1.3) | <0.001 |