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Severe alpha-1-antitrypsin deficiency increases the risk of venous thromboembolism

Basil, Nawfal LU ; Ekström, Magnus LU orcid ; Piitulainen, Eeva LU ; Lindberg, Anne ; Rönmark, Eva ; Jehpsson, Lars LU and Tanash, Hanan LU (2021) In Journal of Thrombosis and Haemostasis 19(6). p.1519-1525
Abstract

Background: Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is associated with increased risk of liver disease and chronic obstructive pulmonary disease (COPD), but the risk of venous thromboembolism (VTE) is unknown. Our aim was to evaluate the risk of VTE in individuals with severe AATD compared with control subjects from the general population. Methods: Individuals with severe AATD (n = 1577) were recruited from the Swedish national AATD register. Control subjects (n = 5969) were selected from the OLIN (Obstructive Lung Disease in Northern Sweden) studies, that include a random general population sample. Longitudinal data on VTE and diagnoses were obtained from the Swedish National Patient Registry. Associations were... (More)

Background: Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is associated with increased risk of liver disease and chronic obstructive pulmonary disease (COPD), but the risk of venous thromboembolism (VTE) is unknown. Our aim was to evaluate the risk of VTE in individuals with severe AATD compared with control subjects from the general population. Methods: Individuals with severe AATD (n = 1577) were recruited from the Swedish national AATD register. Control subjects (n = 5969) were selected from the OLIN (Obstructive Lung Disease in Northern Sweden) studies, that include a random general population sample. Longitudinal data on VTE and diagnoses were obtained from the Swedish National Patient Registry. Associations were analyzed using multivariable Cox regression. Results: At inclusion, 46% of the AATD individuals and 53% of the controls were never-smokers. COPD was present in 46% of the AATD individuals compared with 4% of the controls. During a median follow-up of 18 years, 116 (7%) of the AATD individuals and 89 (1%) of the control subjects developed VTE, unadjusted hazard ratio 6.5 (95% confidence interval 4.9–8.6). Risk factors for incident VTE were male gender, age, COPD, cancer, and liver disease. Adjusting for these factors, the AATD individuals had a significantly higher risk of incident VTE, adjusted hazard ratio 4.2 (95% confidence interval 2.9–6.2) as compared with the controls. Conclusion: Subjects with severe AATD have considerably increased risk of developing VTE compared with the general population, even after accounting for risk factors. This calls for optimized risk factor management and clinical follow-up of this patient group.

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author
; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
alpha-1-antitrypsin deficiency, COPD, deep vein thrombosis, pulmonary embolism, venous thromboembolism
in
Journal of Thrombosis and Haemostasis
volume
19
issue
6
pages
7 pages
publisher
Wiley-Blackwell
external identifiers
  • scopus:85104075506
  • pmid:33763945
ISSN
1538-7933
DOI
10.1111/jth.15302
language
English
LU publication?
yes
id
ac943c7e-4583-407d-beb4-596f521e4efd
date added to LUP
2021-04-21 08:23:36
date last changed
2024-07-13 12:36:15
@article{ac943c7e-4583-407d-beb4-596f521e4efd,
  abstract     = {{<p>Background: Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is associated with increased risk of liver disease and chronic obstructive pulmonary disease (COPD), but the risk of venous thromboembolism (VTE) is unknown. Our aim was to evaluate the risk of VTE in individuals with severe AATD compared with control subjects from the general population. Methods: Individuals with severe AATD (n = 1577) were recruited from the Swedish national AATD register. Control subjects (n = 5969) were selected from the OLIN (Obstructive Lung Disease in Northern Sweden) studies, that include a random general population sample. Longitudinal data on VTE and diagnoses were obtained from the Swedish National Patient Registry. Associations were analyzed using multivariable Cox regression. Results: At inclusion, 46% of the AATD individuals and 53% of the controls were never-smokers. COPD was present in 46% of the AATD individuals compared with 4% of the controls. During a median follow-up of 18 years, 116 (7%) of the AATD individuals and 89 (1%) of the control subjects developed VTE, unadjusted hazard ratio 6.5 (95% confidence interval 4.9–8.6). Risk factors for incident VTE were male gender, age, COPD, cancer, and liver disease. Adjusting for these factors, the AATD individuals had a significantly higher risk of incident VTE, adjusted hazard ratio 4.2 (95% confidence interval 2.9–6.2) as compared with the controls. Conclusion: Subjects with severe AATD have considerably increased risk of developing VTE compared with the general population, even after accounting for risk factors. This calls for optimized risk factor management and clinical follow-up of this patient group.</p>}},
  author       = {{Basil, Nawfal and Ekström, Magnus and Piitulainen, Eeva and Lindberg, Anne and Rönmark, Eva and Jehpsson, Lars and Tanash, Hanan}},
  issn         = {{1538-7933}},
  keywords     = {{alpha-1-antitrypsin deficiency; COPD; deep vein thrombosis; pulmonary embolism; venous thromboembolism}},
  language     = {{eng}},
  month        = {{06}},
  number       = {{6}},
  pages        = {{1519--1525}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Journal of Thrombosis and Haemostasis}},
  title        = {{Severe alpha-1-antitrypsin deficiency increases the risk of venous thromboembolism}},
  url          = {{http://dx.doi.org/10.1111/jth.15302}},
  doi          = {{10.1111/jth.15302}},
  volume       = {{19}},
  year         = {{2021}},
}