Table 2 Univariable analysis of overall survival (OS)
Variable | n | 5-year survival rate, % | 10-year survival rate, % | 15-year survival rate, % | p value (log-rank test) | |
|---|---|---|---|---|---|---|
Total | 268 | 96.2 | 92.0 | 85.5 | – | |
Sex (n = 268) | Male | 166 | 96.1 | 90.0 | 83.3 | 0.745 |
Female | 102 | 96.5 | 96.5 | 89.6 | ||
Age at inclusion into registry (years) (n = 268) | ≤ 65 | 224 | 96.8 | 92.8 | 86.6 | 0.042 |
> 65 | 44 | 93.4 | 87.9 | 80.6 | ||
Diagnostic delay (years) (n = 268) | ≤ 2 | 62 | 100 | 100 | 95.2 | 0.080 |
> 2 | 206 | 94.8 | 88.9 | 81.7 | ||
Phenotype (n = 268) | Pi*ZZ | 220 | 96.9 | 92.4 | 85.6 | 0.633 |
other | 48 | 91.7 | 91.7 | 91.7 | ||
Body mass index (BMI) at inclusion into registry (kg/m2) (n = 268) | ≤ 25 | 168 | 95.1 | 91.2 | 82.9 | 0.071 |
> 25 | 100 | 98.7 | 93.8 | 90.4 | ||
Smoking status at time of inclusion into registry (n = 266) | Never | 75 | 98.5 | 95.9 | 86.5 | 0.002 |
Yes, ex-smoker | 181 | 95.8 | 92.0 | 86.5 | ||
Yes, active smoking | 10 | 80.0 | 0.0 | 0.0 | ||
Active smoking at time of inclusion into registry (n = 266) | No | 256 | 96.7 | 93.2 | 86.6 | < 0.001 |
Yes | 10 | 80.0 | 0.0 | 0.0 | ||
Reason for being tested (n = 263) | Symptomatic disease | 243 | 96.4 | 92.5 | 84.9 | 0.324 |
Family-based screening | 20 | 94.1 | 85.6 | 85.6 | ||
Diagnosis of chronic obstructive pulmonary disease (COPD) at inclusion into registry, as self-reported by the patient (n = 268) | No | 150 | 96.7 | 93.4 | 88.8 | 0.101 |
Yes | 118 | 95.4 | 88.1 | 70.2 | ||
Diagnosis of emphysema at inclusion into registry, as self-reported by the patient (n = 268) | No | 97 | 100 | 95.7 | 95.7 | 0.092 |
Yes | 171 | 94.7 | 90.5 | 82.6 | ||
Forced expiratory volume in 1 s (FEV1) in % of the expected value at inclusion into registry (n = 265) | ≤ 50% | 143 | 94.5 | 89.3 | 80.2 | 0.004 |
> 50% | 122 | 99.0 | 96.7 | 96.7 | ||
Cardiovascular comorbidity (n = 268) | No | 244 | 96.5 | 92.8 | 85.5 | 0.180 |
Yes | 24 | 91.7 | 83.3 | 83.3 | ||
History of exacerbation at inclusion into registry (n = 157) | No | 101 | 98.8 | 95.0 | 95.0 | 0.011 |
Yes | 56 | 89.6 | 83.6 | 54.9 | ||
Treatment with inhalative antiobstructive agents at inclusion into registry (n = 268) | No | 25 | 100 | 88.9 | 88.9 | 0.561 |
Yes | 243 | 95.8 | 92.2 | 85.1 | ||
Long-term oxygen therapy at inclusion into registry (n = 266) | No | 216 | 98.0 | 93.5 | 90.6 | 0.002 |
Yes | 50 | 88.2 | 84.6 | 68.5 | ||
AAT augmentation therapy at inclusion into registry (n = 268) | No | 157 | 92.4 | 88.0 | 85.4 | 0.308 |
Yes | 111 | 100 | 95.8 | 86.8 | ||