Cardiovascular risk in patients with alpha-1-antitrypsin deficiency
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 st...
| Main Authors: | , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2017-09-01
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| Series: | Respiratory Research |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12931-017-0655-1 |
| _version_ | 1818035285218295808 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Sebastian Fähndrich |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-10T06:52:37Z |
| format | Article |
| id | doaj.art-5c47882dc8d444e890dfbb2956966950 |
| institution | Directory Open Access Journal |
| issn | 1465-993X |
| language | English |
| last_indexed | 2024-12-10T06:52:37Z |
| publishDate | 2017-09-01 |
| publisher | BMC |
| record_format | Article |
| series | Respiratory Research |
| spelling | doaj.art-5c47882dc8d444e890dfbb29569669502022-12-22T01:58:30ZengBMCRespiratory Research1465-993X2017-09-0118111110.1186/s12931-017-0655-1Cardiovascular risk in patients with alpha-1-antitrypsin deficiencySebastian Fähndrich0Frank Biertz1Annika Karch2Björn Kleibrink3Armin Koch4Helmut Teschler5Tobias Welte6Hans-Ulrich Kauczor7Sabina Janciauskiene8Rudolf A. Jörres9Timm Greulich10Claus F. Vogelmeier11Robert Bals12on behalf of the COSYCONET investigatorsDepartment of Internal Medicine V, Pulmonology, Allergology, Intensive Care Medicine, Saarland University HospitalInstitute for Biostatistics, Hannover Medical SchoolInstitute for Biostatistics, Hannover Medical SchoolDepartment of Pneumology, Ruhrlandklinik, West German Lung Center, and University Hospital Essen, University Duisburg-EssenInstitute for Biostatistics, Hannover Medical SchoolDepartment of Pneumology, Ruhrlandklinik, West German Lung Center, and University Hospital Essen, University Duisburg-EssenClinic for Pneumology, Hannover Medical School, Member of the German Center for Lung ResearchDepartment of Diagnostic and Interventional Radiology, University of HeidelbergClinic for Pneumology, Hannover Medical School, Member of the German Center for Lung ResearchInstitute and Outpatient Clinic for Occupational, Social and Environmental Medicine, Ludwig-Maximilians-Universität MünchenDepartment of Medicine, Pulmonary and Critical Care Medicine, University Medical Center Giessen and Marburg, Philipps-Universität MarburgDepartment of Medicine, Pulmonary and Critical Care Medicine, University Medical Center Giessen and Marburg, Philipps-Universität MarburgDepartment of Internal Medicine V, Pulmonology, Allergology, Intensive Care Medicine, Saarland University HospitalAbstract 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.http://link.springer.com/article/10.1186/s12931-017-0655-1Alpha-1-antitrypsin deficiencyPhenotypeCardiovascular diseaseEmphysemaPersonalized medicine |
| spellingShingle | 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 Cardiovascular risk in patients with alpha-1-antitrypsin deficiency Respiratory Research Alpha-1-antitrypsin deficiency Phenotype Cardiovascular disease Emphysema Personalized medicine |
| title | Cardiovascular risk in patients with alpha-1-antitrypsin deficiency |
| title_full | Cardiovascular risk in patients with alpha-1-antitrypsin deficiency |
| title_fullStr | Cardiovascular risk in patients with alpha-1-antitrypsin deficiency |
| title_full_unstemmed | Cardiovascular risk in patients with alpha-1-antitrypsin deficiency |
| title_short | Cardiovascular risk in patients with alpha-1-antitrypsin deficiency |
| title_sort | cardiovascular risk in patients with alpha 1 antitrypsin deficiency |
| topic | Alpha-1-antitrypsin deficiency Phenotype Cardiovascular disease Emphysema Personalized medicine |
| url | http://link.springer.com/article/10.1186/s12931-017-0655-1 |
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