Chest
Volume 135, Issue 3, March 2009, Pages 600-608
Journal home page for Chest

Original Research
COPD
Clinical Characteristics of Subjects With Symptoms of α1-Antitrypsin Deficiency Older Than 60 Years

https://doi.org/10.1378/chest.08-1129Get rights and content

Background

The clinical characteristics of elderly subjects with α1-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 FEV1 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.

Section snippets

Materials and Methods

The study comprised 1,062 members of AlphaNet, a not-for-profit health management company that coordinates services for patients with AATD in the United States (including the organization and distribution of augmentation therapy) who consent to participate in a disease management program. The data analyzed here correspond to the clinical outcomes recorded during a pre-intervention 12-month control period. For the study, coordinators (all subjects with AATD employed by AlphaNet) were trained by

Results

Of the 1,062 participants who were enrolled in the study, 34 patients were excluded because of lung transplantation at study entry. During follow-up, 15 patients were excluded because of new lung transplant during the study period, 10 patients withdrew from the study, 6 patients were unavailable for follow-up, and 19 patients died. Subjects not receiving augmentation therapy (n = 42) and with incomplete monthly data collection (n = 14) were excluded from the analysis. Thus, the current analysis

Discussion

Our study shows that individuals with symptomatic AATD-associated COPD who reach the age of 60 years or more, represent a group with particular clinical characteristics. These older individuals have less symptomatic AATD, experience fewer acute disease exacerbations and have better HRQoL scores than younger individuals affected with AATD. Due to an apparent slower decline in lung function, the older individuals experience a longer diagnostic delay and receive a diagnosis later in life. These

Acknowledgment

We thank John Walsh from the Alpha One Foundation, all AlphaNet personnel, particularly Robert Barrett, Bonnie Boyd, and Rosemarie Carillo, and all of the AlphaNet coordinators (Terry Young, Gayle Allison, Roger Bray, Patti Brown, Victoria Cameron, Robert Campbell, Shirley Dennis, Joyce Finch, Phil Freeman, Kathy Haduck, Randy Harwell, Pat MacInness, Darrel Nall, Diana Patterson, Mary Pierce, Judy Rose, Sandy Singleton, Kathleen Sivesind, Marta Strock, Kay Kinsel Swift, Doug Turley, Liz

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    Funding for this study was provided by AlphaNet, Inc., Miami, FL.

    The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

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