Clinical characteristics of subjects with symptoms of alpha1-antitrypsin deficiency older than 60 years.
BACKGROUND
The clinical characteristics of elderly subjects with alpha(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.
Campos
Michael A
MA
Miller School of Medicine, University of Miami, Miami, FL. Electronic address: mcampos1@med.miami.edu.
Alazemi
Saleh
S
Miller School of Medicine, University of Miami, Miami, FL.
Zhang
Guoyan
G
Miami-Dade Health Department, Miami, FL.
Salathe
Matthias
M
Miller School of Medicine, University of Miami, Miami, FL.
Wanner
Adam
A
Miller School of Medicine, University of Miami, Miami, FL.
Sandhaus
Robert A
RA
AlphaNet, Miami, FL.
Baier
Horst
H
Miller School of Medicine, University of Miami, Miami, FL.
D000328:Adult; D000367:Age Factors; D000368:Aged; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume; D006296:Health Services / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D008875:Middle Aged; D029424:Pulmonary Disease, Chronic Obstructive / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D011788:Quality of Life; D012907:Smoking; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy