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Severe alpha‐1‐antitrypsin deficiency increases the risk of venous thromboembolism
Author(s) -
Basil Nawfal,
Ekström Magnus,
Piitulainen Eeva,
Lindberg Anne,
Rönmark Eva,
Jehpsson Lars,
Tanash Hanan
Publication year - 2021
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/jth.15302
Subject(s) - medicine , copd , hazard ratio , alpha 1 antitrypsin deficiency , confidence interval , population , risk factor , disease , proportional hazards model , environmental health
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.