TY  - JOUR
PB  - BioMed Central
SN  - 1465-993X
IS  - 171
A1  - Fähndrich, Sebastian
A1  - Biertz, Frank
A1  - Karch, Annika
A1  - Kleibrink, Björn
A1  - Koch, Armin
A1  - Teschler, Helmut
A1  - Welte, Tobias
A1  - Kauczor, Hans-Ulrich
A1  - Janciauskiene, Sabina
A1  - Jörres, Rudolf A.
A1  - Greulich, Timm
A1  - Vogelmeier, Claus F.
A1  - Bals, Robert
SP  - 1
AV  - public
ID  - heidok23499
CY  - London
UR  - https://archiv.ub.uni-heidelberg.de/volltextserver/23499/
JF  - Respiratory Research
TI  - Cardiovascular risk in patients with alpha-1-antitrypsin deficiency
Y1  - 2017///
VL  - 18
EP  - 11
N2  - 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.
ER  -