Cardiovascular risk in patients with alpha-1-antitrypsin deficiency

• Published in: Respiratory research 2017-09, Vol.18 (1), p.171-171, Article 171
• Main Authors: Fähndrich, Sebastian, Biertz, Frank, Karch, Annika, Kleibrink, Björn, Koch, Armin, Teschler, Helmut, Welte, Tobias, Kauczor, Hans-Ulrich, Janciauskiene, Sabina, Jörres, Rudolf A, Greulich, Timm, Vogelmeier, Claus F, Bals, Robert
• Format: Article
• Language: English
• Subjects: A1 gene; Aged; alpha 1-Antitrypsin - genetics; Alpha 1-antitrypsin deficiency; alpha 1-Antitrypsin Deficiency - diagnosis; alpha 1-Antitrypsin Deficiency - epidemiology; alpha 1-Antitrypsin Deficiency - genetics; Ankle; Augmentation; Body mass; Body mass index; Bronchiectasis; Bronchitis; Carbon monoxide; Cardiovascular disease; Cardiovascular diseases; Cardiovascular Diseases - diagnosis; Cardiovascular Diseases - epidemiology; Cardiovascular Diseases - genetics; Chronic obstructive lung disease; Chronic obstructive pulmonary disease; Classification; Cohort Studies; Comorbidity; Complications and side effects; Coronary artery; Coronary artery disease; Coronary heart disease; Development and progression; Emphysema; Female; Health risk assessment; Health risks; Heart diseases; Humans; Lung diseases; Male; Middle Aged; Mutation; Pathogenesis; Patients; Personalized medicine; Phenotype; Prognosis; Prospective Studies; Regression analysis; Risk Factors; Statistical analysis; Vascular diseases
• ISSN: 1465-993X; 1465-9921; 1465-993X; 1465-9921
• DOI: 10.1186/s12931-017-0655-1

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. 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