Clinical Expressions, Disease Course, Quality of Life, and Resilience in Subgroups of Patients with Angioedema

Background: Patients with angioedema experience recurrent attacks of unpredictable swelling. Recently, new genetic variants have been identified, while patients with unknown causes and no identifiable genetic mutations represent a separate subgroup. As a lifelong illness, angioedema significantly impacts the patient's quality of life and coping ability. Comparing patients representing different subgroups of angioedema is an unmet need. Methods: A cross-sectional, prospective study was conducted to analyze three angioedema subgroups: A: HAE-C1 INH (Types 1 & 2), B: HAE-FXII, and C: AE-UNK; employing validated questionnaires. Results: The study included 111 patients with a mean age of 44.51 years. Mean age of onset was 16.1 years, and mean age at diagnosis was 19.58 years. Subgroups A, B, and C consisted of 72 (64%), 16 (14%), and 23 (20%) patients, respectively. Mean age of onset was earlier in A (10.31 years) than in B (19.70) and C (30.34) subgroups (p < .001). Similarly, mean age at diagnosis was earlier in A (13.85 years) compared to B (24.93) and C (34.73) subgroups (p < .001). Significant differences were found in the triggers of attacks, but not in the body location of the attacks or in experiencing pre-attack prodromes. Subgroup A reported the highest coping ability (p < .001) and total resilience scores (p < .001), mainly in emotion regulation and attention under stress. Conclusions: The designated subgroups differ in both clinical course and coping aptitude. Recognizing the unique characteristics of each AE subgroup can improve the management and quality of life of patients with this lifelong and rare disease.