Comorbidities in hereditary angioedema—A population-based cohort study
Sundler Björkman, Linda LU
; Persson, Barbro
; Aronsson, David
LU
; Skattum, Lillemor
LU
; Nordenfelt, Patrik
and Egesten, Arne
LU
(2022)
In Clinical and Translational Allergy
12(3).
- Abstract
Background: 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. Methods: 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. Results: Increased risk of cardiovascular disease (odds ratio [OR] 1.83; 95%... (More)
Background: 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. Methods: 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. Results: 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. Conclusions: 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.
(Less)
- author
- Sundler Björkman, Linda
LU
; Persson, Barbro
; Aronsson, David
LU
; Skattum, Lillemor
LU
; Nordenfelt, Patrik
and Egesten, Arne
LU
- organization
- publishing date
- 2022
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- autoimmunity, cardiovascular disease, complement, epidemiology, hereditary angioedema (HAE)
- in
- Clinical and Translational Allergy
- volume
- 12
- issue
- 3
- article number
- e12135
- publisher
- BioMed Central (BMC)
- external identifiers
-
- pmid:35344299
- scopus:85127288939
- ISSN
- 2045-7022
- DOI
- 10.1002/clt2.12135
- language
- English
- LU publication?
- yes
- id
- 0948daf2-64b5-48b0-b35d-eb0907e68ea4
- date added to LUP
- 2022-05-16 13:28:17
- date last changed
- 2024-07-20 21:14:06
@article{0948daf2-64b5-48b0-b35d-eb0907e68ea4,
abstract = {{<p>Background: 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. Methods: 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. Results: 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. Conclusions: 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.</p>}},
author = {{Sundler Björkman, Linda and Persson, Barbro and Aronsson, David and Skattum, Lillemor and Nordenfelt, Patrik and Egesten, Arne}},
issn = {{2045-7022}},
keywords = {{autoimmunity; cardiovascular disease; complement; epidemiology; hereditary angioedema (HAE)}},
language = {{eng}},
number = {{3}},
publisher = {{BioMed Central (BMC)}},
series = {{Clinical and Translational Allergy}},
title = {{Comorbidities in hereditary angioedema—A population-based cohort study}},
url = {{http://dx.doi.org/10.1002/clt2.12135}},
doi = {{10.1002/clt2.12135}},
volume = {{12}},
year = {{2022}},
}