TABLE 1

Demographic and clinical characteristics of the participants in the Spanish registry of the European Alpha-1 Research Collaboration and comparison between PI*ZZ and PI*SZ genotypes

VariablesGlobalZZSZp-value
Subjects (n)405181163
Age (years)53.5±15.955.4±14.751.0±17.30.015
Sex, male218 (53.8)95 (52.5)87 (53.4)0.869
BMI (kg·m−2)26.4±4.826.2±4.926.3±4.90.730
Smokers33 (8)3 (2.8)22 (23.2)<0.001
Ex-smokers222 (54.4)111 (61.3)3 (44.9)
Never-smokers149 (36.8)66 (36.7)68 (41)
Tobacco exposure (pack-years)24.5±21.920.3±13.825.2±25.10.953
Chronic respiratory disease267 (66.4)146 (81.6)82 (50.6)<0.001
COPD175 (43.5)108 (60.3)42 (25.9)<0.001
Emphysema163 (40.5)107 (59.8)40 (24.7)<0.001
Bronchiectasis83 (20.6)58 (32.4)16 (10)<0.001
Asthma55 (13.7)24 (13.4)22 (13.6)0.963
Liver disease42 (10.6)24 (13.4)16 (10)0.322
Age at onset of symptoms (years)46.1±17.244.2±16.347.9±17.50.063
Age at diagnosis of AATD (years)46.4±17.444.5±17.246.8±180.354
Index case286 (70.6)135 (74.6)106 (65)0.053
Family screening98 (24)39 (21.5)45 (27.6)0.121
Charlson index (age corrected)3.1±2.13.2±22.9±20.347
Cardiovascular disease#89 (22.5)47 (26.4)25 (15.7)0.017
FVC % pred99.4±22.696.1±26.2103±17.40.011
FEV1 % pred81.7±31.469.8±32.193.3±26<0.001
FEV1/FVC % pred65±1957±1972±18<0.001
KCO % pred76.8±23.467.6±22.584.7±20.8<0.001
Augmentation therapy114 (28.9)95 (54)13 (8.2)0.001
AAT (mg·dL−1)44.5±24.424.9±17.656.7±11.8<0.001
Number of exacerbations previous year0.44±10.66±1.20.28±0.8<0.001
History of pneumonia77 (19.3)44 (24.6)21 (13.1)0.007
CAT7.4±7.29.4±7.85.6±6.2<0.001
BODEx1.2±1.81.8±20.6 (1.3)<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.