Attack frequency and associated factors in hereditary angioedema patients: a single-centre experience.

Abstract

Introduction: Hereditary angioedema (HAE) significantly affects patients' quality of life due to its life-threatening potential and the unpredictability of attacks.

Aim: To determine strategic goals to reduce the frequency of attacks and improve the quality of life by evaluating the attack triggers, attack management strategies, and medication use attitudes of HAE patients treated in our clinic.

Material and methods: Patients who were followed up in our clinic with a diagnosis of HAE in 2022-2023 were included in the study. Patient data were obtained by retrospective scanning of file records.

Results: The analysis included 23 HAE patients with an average age of 34.7 years, predominantly female (60.9%). In the patients' family history, there was a high prevalence of HAE (95.7%) and HAE-related death (73.9%). The median duration of HAE diagnostic delay was 9 years (0-50). A significant positive correlation was found between patient age and the duration of diagnostic delay (r = 0.575; p = 0.004). The majority of patients were aware of acute attack triggers and contraindicated medications (91.3%, 70%, respectively). Gastrointestinal, facial, and limb symptoms were common, with emotional stress identified as the leading trigger. The median monthly attack frequency was 6 (1-10), with nearly half experiencing more frequent episodes. Response times to on-demand treatment for icatibant were less than 30 min in 52.2% of patients. Despite experiencing frequent attacks, many patients exhibited pharmacophobia, leading to reluctance towards second-line long-term prophylactic (LTP) therapy.

Conclusions: The high frequency of attacks in our patients was related to the lack of use of LTP agents and personal life stress. Despite severe attacks and a family history of HAE-related deaths, pharmacophobia to LTP was common, with pharmacophobia resulting from side effects of second-line LTP agents. Our findings highlight the need for our patients to have access to psychosocial support programs and first-line LTP agents.