Department of Respiratory Medicine, Skåne University Hospital, Lund University, Malmö, Sweden.
Department of Clinical Sciences Lund, Respiratory Medicine and Allergology, Faculty of Medicine, Lund University, Lund, Sweden.
J Thromb Haemost. 2021 Jun;19(6):1519-1525. doi: 10.1111/jth.15302. Epub 2021 Apr 9.
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.
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.
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.
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.
严重的α-1-抗胰蛋白酶缺乏症（AATD），表型 PiZZ，与肝病和慢性阻塞性肺疾病（COPD）的风险增加有关，但静脉血栓栓塞症（VTE）的风险尚不清楚。我们的目的是评估与一般人群中的对照相比，严重 AATD 个体发生 VTE 的风险。
从瑞典国家 AATD 登记处招募了 1577 名严重 AATD 患者。对照（n=5969）从 OLIN（瑞典北部阻塞性肺病）研究中选择，包括随机的一般人群样本。从瑞典国家患者登记处获得关于 VTE 和诊断的纵向数据。使用多变量 Cox 回归分析关联。
在纳入时，46%的 AATD 患者和 53%的对照组从未吸烟。与对照组的 4%相比，46%的 AATD 患者患有 COPD。在中位随访 18 年期间，116（7%）名 AATD 患者和 89（1%）名对照组患者发生 VTE，未调整的危险比为 6.5（95%置信区间 4.9-8.6）。发生 VTE 的危险因素是男性、年龄、COPD、癌症和肝病。在调整这些因素后，与对照组相比，AATD 患者发生 VTE 的风险显著增加，调整后的危险比为 4.2（95%置信区间 2.9-6.2）。
与一般人群相比，严重 AATD 患者发生 VTE 的风险显著增加，即使考虑到危险因素也是如此。这需要优化该患者群体的危险因素管理和临床随访。
