Comparison of demographic and clinical characteristics of the participants in the Spanish registry of European Alpha-1 Research Collaboration with chronic lung disease and PI*ZZ and PI*SZ genotypes
| Variables | ZZ | SZ | p-value |
| Subjects (n) | 146 | 82 | |
| Age (years) | 58.6±12.3 | 57.6±17.2 | 0.692 |
| Sex, male | 75 (51.4) | 47 (57.8) | 0.386 |
| BMI (kg·m−2) | 26.4±4.8 | 27.6±5.3 | 0.067 |
| Smokers | 2 (1.4) | 14 (17.1) | <0.001 |
| Ex-smokers | 105 (71.9) | 48 (58.5) | |
| Never-smokers | 39 (26.7) | 20 (24.4) | |
| Tobacco exposure (pack-years) | 18.8±9.7 | 20.7±13.5 | 0.460 |
| COPD | 108 (74) | 42 (51.2) | 0.001 |
| Emphysema | 107 (73.3) | 40 (48.8) | <0.001 |
| Bronchiectasis | 58 (39.7) | 16 (19.5) | 0.002 |
| Asthma | 24 (16.4) | 22 (26.8) | 0.061 |
| Liver disease | 17 (11.8) | 8 (9.8) | 0.637 |
| Age at onset of symptoms (years) | 45.6±15 | 49.9±18.1 | 0.008 |
| Age at diagnosis of AATD (years) | 47.4±14.9 | 52.5±18 | 0.003 |
| Index case | 25 (17.1) | 15 (18.3) | 0.824 |
| Family screening | 21 (14.4) | 12 (14.6) | 0.959 |
| Charlson index (age corrected) | 3.5±1.9 | 4±2.2 | 0.105 |
| Cardiovascular comorbidity# | 43 (30.1) | 22 (28.2) | 0.771 |
| FVC % pred | 93.4±26.6 | 100.4±20.7 | 0.046 |
| FEV1 % pred | 61.1±28.2 | 78.7±28.5 | <0.001 |
| FEV1/FVC % pred | 51±17 | 62±19 | <0.001 |
| KCO % pred | 62.9±20.6 | 84.7±20.8) | <0.001 |
| Augmentation therapy | 94 (66.2) | 12 (15.4) | <0.001 |
| AAT (mg·dL−1) | 25.8±19.4 | 57.5±12.2 | <0.001 |
| Number of exacerbations previous year | 0.80±1.3 | 0.43±1 | 0.018 |
| History of pneumonia | 39 (26.9) | 10 (12.7) | 0.014 |
| CAT | 10.9±7.9 | 8.6±7 | 0.036 |
| BODEx | 2.2±2.1 | 1.1±1.7 | <0.001 |
Data presented as mean±sd or n (%), unless otherwise indicated. BMI: body mass index; COPD: chronic obstructive pulmonary disease; AATD: α1-antitrypsin deficiency; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; KCO: transfer coefficient of the lung for carbon monoxide; AAT: α1-antitrypsin; CAT: COPD Assessment Test; BODEx: BMI, Obstruction, Dyspnoea, Exacerbations index. #: cardiovascular disease includes hypertension, ischaemic heart disease, congestive heart failure and peripheral vascular disease.