Akademik Veri Yönetim Sistemi
International Archives of Allergy and Immunology, ss.1-11, 2026 (SCI-Expanded, Scopus)
Introduction: Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH) has a substantial impact on patients' health-related quality of life (HRQoL). While generic instruments are frequently used for assessment, they may not fully capture the disease-specific burden. This study aimed to evaluate HRQoL in patients with HAE-C1INH types I and II using validated, angioedema-specific tools. The aims of the study were to assess HRQoL and disease control in patients with HAE-C1INH using validated angioedema-specific tools and secondarily to explore the association between attack localization and domain-specific quality-of-life impairments. Methods: In this multicenter cross-sectional study, 82 patients with HAE-C1INH from six referral centers completed the Angioedema Control Test (AECT), Angioedema Activity Score (AAS)-28, and Angioedema Quality of Life questionnaire (AE-QoL). Family screening was conducted among those who consented. Results: Patients experiencing attacks in the extremities (p < 0.001) and laryngeal/pharyngeal regions (p = 0.028) had significantly lower AECT scores, indicating poorer disease control. Higher AAS-28 scores were associated with attacks in the extremities (p = 0.008), abdomen (p = 0.018), face/neck (p = 0.004), larynx/pharynx (p < 0.001), and tongue/uvula/palate (p < 0.001). AE-QoL scores revealed impaired quality of life across all attack locations. Subdomain analysis showed that abdominal attacks significantly impacted the functioning (p = 0.005) and food (p = 0.037) domains. Attacks involving the face/neck and larynx/pharynx were associated with substantial impairment in fatigue/mood (p = 0.002, p = 0.003), functioning (p = 0.044, p = 0.002), and fears/shame (p = 0.039, p = 0.005) subdomains. Conclusion: While attack frequency and disease control remain the primary determinants of HRQoL in HAE-C1INH, our findings indicate that attack localization is associated with distinct domain-specific quality-of-life impairments. Beyond assessing attack frequency, the use of disease-specific QoL tools allows for the identification of domain-specific impairments. Personalized treatment strategies targeting both symptom control and quality-of-life domains are essential for alleviating the long-term burden on patients and their families.