Table 1 Baseline patient characteristics of the population of interest (n = 268)
Variable | n | % | |
|---|---|---|---|
Total | 268 | 100 | |
Sex (n = 268) | Male | 166 | 61.9 |
Female | 102 | 38.1 | |
Age (years) (n = 268) | Mean (standard deviation) | 53.4 (11.9) | |
Diagnostic delay (years) (n = 268) | Median (IQR) | 5.3 (2.2–11.5) | |
< 2 | 62 | 23.1 | |
2–5 | 66 | 24.6 | |
5–10 | 57 | 21.3 | |
> 10 | 83 | 31.0 | |
Phenotype (n = 268) | ZZ | 220 | 82.1 |
SZ | 29 | 10.8 | |
Other | 19 | 7.1 | |
Serum AAT level (g/L) at time of AATD diagnosis (n = 163) | Mean (standard deviation) | 0.33 (0.19) | |
≥ 0.6 | 16 | 9.8 | |
< 0.6 | 147 | 90.2 | |
Body mass index (BMI; kg/m2) (n = 268) | Mean (standard deviation) | 24.2 (4.0) | |
Smoking status (n = 266) | Never | 75 | 28.2 |
Yes, at any time | 191 | 71.8/100 | |
Yes, ex-smoker | 181 | 68.0/94.8 | |
Yes, active smoking | 10 | 3.8/5.2 | |
Reason for being tested (n = 263) | Symptomatic disease | 243 | 92.4 |
Family-based screening | 20 | 7.6 | |
Lung disease, as self-reported by the patient (multiple diagnoses possible) (n = 268) | None | 4 | 1.5 |
Any lung disease | 264 | 98.5 | |
Chronic obstructive pulmonary disease (COPD) | 118 | 44.0 | |
Emphysema | 171 | 63.8 | |
Chronic bronchitis | 74 | 27.6 | |
Asthma | 29 | 10.8 | |
Bronchiectasis | 13 | 4.9 | |
Lung cancer | 0 | 0.0 | |
Respiratory tract-related symptoms (multiple symptoms possible) (n = 268) | None | 0 | 0.0 |
Any respiratory tract-related symptoms | 268 | 100 | |
Cough | 53 | 19.8 | |
Dyspnea | 236 | 88.1 | |
Forced expiratory volume in 1 s (FEV1) in % of the expected value (n = 265) | Mean (standard deviation) | 52.5 (24.7) | |
≤ 50% | 143 | 54.0 | |
> 50% | 122 | 45.5 | |
Cardiovascular comorbidity (n = 268) | No | 244 | 91.0 |
Yes | 24 | 9.0 | |
History of pneumonia (n = 261) | No | 162 | 62.1 |
Yes | 99 | 37.9 | |
History of exacerbation (n = 157) | No | 101 | 64.3 |
Yes | 56 | 35.7 | |
History of lung transplantation (n = 268) | No | 263 | 98.1 |
Yes | 5 | 1.9 | |
History of lung volume reduction surgery (n = 268) | No | 263 | 98.1 |
Yes | 5 | 1.9 | |
Treatment with inhalative antiobstructive agents (n = 268) | No | 25 | 9.3 |
Yes | 243 | 90.7 | |
Long-term oxygen therapy (n = 266) | No | 216 | 81.2 |
Yes | 50 | 18.8 | |
AAT augmentation therapy (n = 268) | No | 157 | 58.6 |
Yes | 111 | 41.4 | |