ArticleClinical and translational allergy2025
C1 inhibitor deficient hereditary angioedema is related to endothelial dysfunction in young adult and middle-aged patients.
Article in Clinical and translational allergy, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- Eosinophilic inflammation in hereditary angioedema: a single-center real-world retrospective chart review study.Frontiers in immunology · 2026Article
- Treatment of Hereditary Angioedema With Plasma-Derived C1 Inhibitor: A Review.Clinical and translational allergy · 2025Review
- Delphi Consensus on Attenuated Androgen Use for Long-Term Prophylaxis in Hereditary Angioedema: AURA Project.Clinical and translational allergy · 2025Article
- C1 inhibitor deficient hereditary angioedema is related to endothelial dysfunction in young adult and middle-aged patients.Clinical and translational allergy · 2025Article
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8 authors.
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Abstract
backgroundHereditary angioedema (HAE) is a rare, autosomal dominantly inherited disease characterised by mucocutaneous oedema attacks. Vasoactive mediators and the endothelium play an important role in the pathogenesis of HAE. We aimed to evaluate the endothelial dysfunction in HAE.
methodsThe study included 35 C1 inhibitor deficient (C1-INH) HAE patients aged 18-50 years old without any risk factor that could cause endothelial dysfunction, and 25 sex- and age-matched healthy controls (HCs). Bilateral carotid intima-media thickness (CIMT), flow-mediated dilation (FMD) measurements, and transthoracic echocardiography (ECHO) imaging were performed. Demographic and clinical features of the patients were evaluated.
resultsThe percentage of FMD (FMD [%]) in C1-INH HAE patients was significantly lower than in the HCs (p < 0.001). There was no significant difference in the CIMT between C1-INH HAE patients and HCs. In addition, the findings of the ECHO were similar between the groups. C4 and C1 INH levels at diagnosis, gender, age, disease severity, presence of long-term prophylaxis treatment and attack frequency were not associated with FMD (%), whereas disease duration was correlated with lower FMD (%) (r = -0.480, p = 0.003).
conclusionThe present study indicated the presence of structural endothelial dysfunction in C1-INH HAE in the absence of atherosclerosis. Moreover, the study revealed that endothelial dysfunction was associated with disease duration, irrespective of disease severity. Further studies are required in order to assess mortality and morbidity due to endothelial dysfunction in C1-INH HAE and to determine the molecular mechanisms underlying endothelial dysfunction in C1-INH HAE.
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