Objective: Hereditary angioedema (HAE) is a rare disease associated with significant morbidity and mortality, particularly due to laryngeal attacks. This study aimed to describe the demographic and clinical characteristics of adults with HAE followed at a single center, with a focus on triggers, diagnostic delay, and treatment approaches.

Materials and Methods: This retrospective cohort study included 15 patients aged ≥18 years who were followed at our center between 2019 and 2024. Clinical and laboratory data were obtained through retrospective review of medical records.

Results: Fifteen HAE patients were included; 73.3% were female (median age: 34 years, range: 21–73). The diagnostic delay was 8 years (range: 0–49), and patient age was positively correlated with diagnostic delay (r=0.706, p=0.003). Thirteen patients had HAE-1 and two HAE-2; attacks most commonly involved the face/extremities (46.7%) and gastrointestinal tract (33.3%). Laryngeal attacks occurred in 60% of patients, with 13.3% requiring intensive care unit (ICU) follow-up but no intubation. Attacks were most frequently reported by patients in association with stress or fatigue (86.7%), followed by trauma. Among female patients, 27.3% reported a temporal association between menstrual cycles and attack occurrence. Prodromal symptoms occurred in 66.7%, most commonly skin tightness and tingling. Icatibant was used on demand (median response: 60 min); short-term prophylaxis during procedures (46.7%) prevented attacks. Three patients receiving long-term prophylaxis (LTP) with danazol reported a reduction in attack frequency. Patients with prior ICU admission were more likely to receive LTP (p = 0.029), likely reflecting greater disease severity and reverse causality rather than a causal effect of LTP on ICU admission.

Conclusion: This study provides descriptive real-world data on adults with HAE in a setting with limited access to first-line prophylactic therapies. Stress-related factors were frequently reported in association with attacks, and diagnostic delay appeared to be shorter among younger patients. Restricted access to first-line LTP remains a relevant clinical challenge.