International archives of allergy and immunology · 2026
A Comprehensive Assessment of Health-Related Quality of Life in Hereditary Angioedema as a Patient-Centered Approach.
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Yalcin Gungoren Ezgi, Karabiber Esra, Yorgun Altunbas Melek, Can Salim, Tusuz Onata Ece, Atik Ozge, Dikici Ummugulsum, Meric Zeynep, Ozanli Ismail, Amirov Razin, Ozturk Necmiye, Bozkurt Selcen, Eser Simsek Isil, Kiykim Ayca, Ozek Yucel Esra, Bogatekin Gulhan, Aydogan Metin, Tepetam Fatma Merve, Ozdemir Oner, Yilmaz Ebru Arik, Mete Gokmen Emine Nihal, Bilgic-Eltan Sevgi, Baris Safa, Ozen Ahmet, Karakoc-Aydiner Elif
Аннотация
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.
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.