PMID- 33763945 OWN - NLM STAT- MEDLINE DCOM- 20210708 LR - 20230829 IS - 1538-7836 (Electronic) IS - 1538-7836 (Linking) VI - 19 IP - 6 DP - 2021 Jun TI - Severe alpha-1-antitrypsin deficiency increases the risk of venous thromboembolism. PG - 1519-1525 LID - 10.1111/jth.15302 [doi] AB - 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. CI - (c) 2021 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis. FAU - Basil, Nawfal AU - Basil N AD - Department of Respiratory Medicine, Skane University Hospital, Lund University, Malmo, Sweden. FAU - Ekstrom, Magnus AU - Ekstrom M AD - Department of Clinical Sciences Lund, Respiratory Medicine and Allergology, Faculty of Medicine, Lund University, Lund, Sweden. FAU - Piitulainen, Eeva AU - Piitulainen E AD - Department of Respiratory Medicine, Skane University Hospital, Lund University, Malmo, Sweden. FAU - Lindberg, Anne AU - Lindberg A AD - Division of Medicine, Department of Public Health and Clinical Medicine, Umea University, Umea, Sweden. FAU - Ronmark, Eva AU - Ronmark E AD - The OLIN Unit, Division of Occupational and Environmental Medicine, Department of Public Health and Clinical Medicine, Umea University, Umea, Sweden. FAU - Jehpsson, Lars AU - Jehpsson L AD - Department of Clinical Sciences, Skane University Hospital, Lund University, Lund, Sweden. FAU - Tanash, Hanan AU - Tanash H AD - Department of Respiratory Medicine, Skane University Hospital, Lund University, Malmo, Sweden. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20210409 PL - England TA - J Thromb Haemost JT - Journal of thrombosis and haemostasis : JTH JID - 101170508 SB - IM MH - Humans MH - Male MH - *Pulmonary Disease, Chronic Obstructive/complications/diagnosis/epidemiology MH - Risk Factors MH - Sweden/epidemiology MH - *Venous Thromboembolism/diagnosis/epidemiology MH - *alpha 1-Antitrypsin Deficiency/complications/diagnosis/epidemiology OTO - NOTNLM OT - COPD OT - alpha-1-antitrypsin deficiency OT - deep vein thrombosis OT - pulmonary embolism OT - venous thromboembolism EDAT- 2021/03/26 06:00 MHDA- 2021/07/09 06:00 CRDT- 2021/03/25 06:52 PHST- 2021/02/23 00:00 [revised] PHST- 2020/12/15 00:00 [received] PHST- 2021/03/03 00:00 [accepted] PHST- 2021/03/26 06:00 [pubmed] PHST- 2021/07/09 06:00 [medline] PHST- 2021/03/25 06:52 [entrez] AID - S1538-7836(22)00785-1 [pii] AID - 10.1111/jth.15302 [doi] PST - ppublish SO - J Thromb Haemost. 2021 Jun;19(6):1519-1525. doi: 10.1111/jth.15302. Epub 2021 Apr 9.