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Respiratory Research
Published in: Respiratory Research 1/2017

Open Access 01-12-2017 | Research

Cardiovascular risk in patients with alpha-1-antitrypsin deficiency

Authors: Sebastian Fähndrich, Frank Biertz, Annika Karch, Björn Kleibrink, Armin Koch, Helmut Teschler, Tobias Welte, Hans-Ulrich Kauczor, Sabina Janciauskiene, Rudolf A. Jörres, Timm Greulich, Claus F. Vogelmeier, Robert Bals, on behalf of the COSYCONET investigators

Published in: Respiratory Research | Issue 1/2017

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Abstract

Background

Alpha-1-antitrypsin deficiency (AATD) is a rare inherited condition caused by mutations of the SERPINA1 gene that is associated with the development of a COPD like lung disease. The comorbidities in patients with AATD-related lung diseases are not well defined. The aim of this study was to analyze the clinical phenotype of AATD patients within the German COPD cohort study COSYCONET (“COPD and SYstemic consequences-COmorbidities NETwork”) cohort focusing on the distribution of comorbidities.

Method and results

The data from 2645 COSYCONET patients, including 139 AATD patients (110 with and 29 without augmentation therapy), were analyzed by descriptive statistics and regression analyses. We found significantly lower prevalence of cardiovascular comorbidities in AATD patients as compared to non-AATD COPD patients. After correction for age, pack years, body mass index, and sex, the differences were still significant for coronary artery disease (p = 0.002) and the prevalence of peripheral artery disease as determined by an ankle-brachial-index <= 0.9 (p = 0.035). Also the distribution of other comorbidities such as bronchiectasis differed between AATD and non-deficient COPD.

Conclusion

AATD is associated with a lower prevalence of cardiovascular disease, the underlying mechanisms need further investigation.
Appendix
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Metadata
Title
Cardiovascular risk in patients with alpha-1-antitrypsin deficiency
Authors
Sebastian Fähndrich
Frank Biertz
Annika Karch
Björn Kleibrink
Armin Koch
Helmut Teschler
Tobias Welte
Hans-Ulrich Kauczor
Sabina Janciauskiene
Rudolf A. Jörres
Timm Greulich
Claus F. Vogelmeier
Robert Bals
on behalf of the COSYCONET investigators
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Respiratory Research / Issue 1/2017
Electronic ISSN: 1465-993X
DOI
https://doi.org/10.1186/s12931-017-0655-1

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