A Comprehensive Assessment of Health-Related Quality of Life in Hereditary Angioedema as a Patient-Centered Approach
Makaleler > Tam Makale · 2026
Yazarlar: Ezgi Yalcin Gungoren, Esra Karabiber, Melek Yorgun Altunbas, Salim Can, Ece Tusuz Onata, Ozge Atik, Ummugulsum Dikici, Zeynep Meric, Ismail Ozanli, Razin Amirov, Necmiye Ozturk, Selcen Bozkurt, Isil Eser Simsek, Ayca Kiykim, Esra Ozek Yucel, Gulhan Bogatekin, Metin Aydogan, Fatma Merve Tepetam, Oner Ozdemir, Ebru Arik Yilmaz, Emine Nihal METE GÖKMEN, Sevgi Bilgic-Eltan, Safa Baris, Ahmet Ozen, Elif Karakoc-Aydiner
International Archives of Allergy and Immunology, Uluslararası, Wos, 2026, ss 1-11
DOI: 10.1159/000550399
Özet
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.