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

Subjects (n)14682
Age (years)58.6±12.357.6±17.20.692
Sex, male75 (51.4)47 (57.8)0.386
BMI (kg·m−2)26.4±4.827.6±5.30.067
Smokers2 (1.4)14 (17.1)<0.001
Ex-smokers105 (71.9)48 (58.5)
Never-smokers39 (26.7)20 (24.4)
Tobacco exposure (pack-years)18.8±9.720.7±13.50.460
COPD108 (74)42 (51.2)0.001
Emphysema107 (73.3)40 (48.8)<0.001
Bronchiectasis58 (39.7)16 (19.5)0.002
Asthma24 (16.4)22 (26.8)0.061
Liver disease17 (11.8)8 (9.8)0.637
Age at onset of symptoms (years)45.6±1549.9±18.10.008
Age at diagnosis of AATD (years)47.4±14.952.5±180.003
Index case25 (17.1)15 (18.3)0.824
Family screening21 (14.4)12 (14.6)0.959
Charlson index (age corrected)3.5±1.94±2.20.105
Cardiovascular comorbidity#43 (30.1)22 (28.2)0.771
FVC % pred93.4±26.6100.4±20.70.046
FEV1 % pred61.1±28.278.7±28.5<0.001
FEV1/FVC % pred51±1762±19<0.001
KCO % pred62.9±20.684.7±20.8)<0.001
Augmentation therapy94 (66.2)12 (15.4)<0.001
AAT (mg·dL−1)25.8±19.457.5±12.2<0.001
Number of exacerbations previous year0.80±1.30.43±10.018
History of pneumonia39 (26.9)10 (12.7)0.014

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.