Sundler Björkman Linda, Persson Barbro, Aronsson David, Skattum Lillemor, Nordenfelt Patrik, Egesten Arne
Respiratory Medicine & Allergology, Department of Clinical Sciences Lund, Lund University and Skåne University Hospital, Lund, Sweden.
Department of Immunology, Genetics and Pathology, The Rudbeck Laboratory, Uppsala University, Uppsala, Sweden.
Clin Transl Allergy. 2022 Mar;12(3):e12135. doi: 10.1002/clt2.12135.
In hereditary angioedema (HAE), low levels (type 1) or defect in function (type 2) of the serine-protease inhibitor C1 Inhibitor protein results in activation of the classical pathway of the complement system as well as the contact system. Here, we investigated the risk of comorbidities in HAE.
Individuals with HAE (n = 239; identified through a physician made diagnosis) and a control cohort from the general population (n = 2383; matched for age, gender, and county of residence) were compared with the Swedish inpatient, cause of death, cancer, and prescription registers. Conditional logistic regression was used to analyze the data.
Increased risk of cardiovascular disease (odds ratio [OR] 1.83; 95% confidence interval [CI] 1.32-2.54), including arterial (OR 6.74; 95% CI 1.89-24.06) and venous thromboembolic disease (OR 4.20; 95% CI 2.42-7.23) as well as hypertension (OR 1.64; 95% CI 1.12-2.39) was seen in HAE. There was also an increased number of individuals diagnosed with hyperlipidemia (OR 2.01; 95% CI 1.16-3.50) among HAE patients. Furthermore, the risk of autoimmune disease was increased (OR 1.65; 95% CI 1.15-2.35) being particularly pronounced for systemic lupus erythematosus (OR 71.87; 95% CI 8.80-586.7). The risk of having two or more autoimmune diseases was also higher among HAE patients (p = 0.017). In contrast, the risk of cancer was not increased. Data from the prescription register revealed higher prescription rates of drugs against hypertension, hypothyroidism, and hyperlipidemia among HAE patients.
The results warrant for awareness and prevention of comorbid conditions, in particular, thromboembolic and autoimmune diseases in HAE. Future prophylactic interventions may modify these risks.
在遗传性血管性水肿（HAE）中，丝氨酸蛋白酶抑制剂C1抑制蛋白水平低（1型）或功能缺陷（2型）会导致补体系统经典途径以及接触系统的激活。在此，我们调查了HAE中共存疾病的风险。
将HAE患者（n = 239；通过医生诊断确定）和来自普通人群的对照队列（n = 2383；按年龄、性别和居住县匹配）与瑞典住院患者、死亡原因、癌症和处方登记册进行比较。使用条件逻辑回归分析数据。
HAE患者出现心血管疾病风险增加（比值比[OR] 1.83；95%置信区间[CI] 1.32 - 2.54），包括动脉（OR 6.74；95% CI 1.89 - 24.06）和静脉血栓栓塞性疾病（OR 4.20；95% CI 2.42 - 7.23）以及高血压（OR 1.64；95% CI 1.12 - 2.39）。HAE患者中被诊断为高脂血症的人数也有所增加（OR 2.01；95% CI 1.16 - 3.50）。此外，自身免疫性疾病风险增加（OR 1.65；95% CI 1.15 - 2.35），系统性红斑狼疮尤为明显（OR 71.87；95% CI 8.80 - 586.7）。HAE患者患两种或更多种自身免疫性疾病的风险也更高（p = 0.017）。相比之下，癌症风险没有增加。处方登记册的数据显示，HAE患者中抗高血压、甲状腺功能减退和高脂血症药物的处方率更高。
这些结果表明有必要认识和预防共存疾病，特别是HAE中的血栓栓塞性疾病和自身免疫性疾病。未来的预防性干预措施可能会改变这些风险。
