Clinical characteristics of subjects with symptoms of α₁- antitrypsin deficiency older than 60 years

Background: The clinical characteristics of elderly subjects with α ₁-antitrypsin deficiency (AATD)-associated COPD have not been described. Methods: The clinical, demographic, health-related quality of life (HRQoL) characteristics and 1-year exacerbation rates of 275 subjects with AATD and COPD receiving augmentation therapy aged > 59 years (mean [± SD] age, 66.3 ± 5.7 years) were compared to those of 354 subjects aged 50 to 59 years (mean age, 54.3 ± 2.8 years) and 293 subjects < 50 years (mean age, 43.9 ± 3.8 years). Results: Older subjects received diagnoses later in life (mean age at diagnosis, 55.0 ± 8.5 years) and had a longer diagnostic delay (mean age at diagnosis, 12.9 ± 14.3 years) than subjects in the other two age groups. Although the proportion of lifetime nonsmokers was higher in the older group, the majority (64%) had significant tobacco exposure but with a longer interval of tobacco abstinence. The mean FEV ₁ values (n = 641) were similar between the three age groups, suggesting a slower disease progression in the oldest group. Subjects in the older group were less symptomatic, had less concomitant asthma, and had significantly better scores in most domains of two HRQoL instruments. During follow-up, older subjects had fewer acute exacerbations. Conclusions: Subjects with AATD-associated COPD who reach an older age exhibit a more indolent clinical course than younger affected individuals, possibly related in part to differences in tobacco exposure. This finding supports current guidelines that recommend screening of all patients with COPD for AATD, regardless of their age and prior smoking history.