- D1.340 - Attack burden and patient-reported outcomes in adults with hereditary angioedema: associations with quality of life, pain, and coping strategies

Hereditary angioedema (HAE) is characterized by recurrent and unpredictable attacks that substantially impair patients’ quality of life (QoL). Although attack frequency is a key clinical outcome, its relationship with disease-specific and generic patient-reported outcomes (PROs) and coping strategies has not been fully elucidated.

We conducted a cross-sectional analysis of adults with HAE (N=55). Attack burden during the previous 6 months was recorded using an ordinal 5-level scale. PROs included the disease-specific HAE-QoL, the Angioedema Quality of Life questionnaire (AE-QoL, partial dataset), the SF-36 Health Survey, and the Brief COPE questionnaire. Associations between attack burden and PROs were assessed using Spearman correlation coefficients. Group differences by demographic and clinical variables were evaluated using non-parametric tests. Multivariable ordinal logistic regression was performed to identify independent predictors of higher attack burden.

Attack burden data were available for 51 participants. Higher attack categories were strongly associated with worse generic health status, particularly SF-36 General Health (ρ = −0.51, p < 0.001) and SF-36 Bodily Pain (ρ = −0.51, p < 0.001), as well as with worse disease-specific QoL measured by HAE-QoL (ρ = 0.41, p = 0.002). A moderate association was observed with maladaptive coping strategies, particularly denial (Brief COPE; ρ = 0.41, p = 0.003). Higher attack burden was also associated with recent hospitalization (median attack category 3 vs 1, p = 0.049) and receipt of acute on-demand treatment during the previous 6 months (p = 0.013). In subgroup analyses, female patients reported worse disease-specific quality of life, whereas no significant differences in patient-reported outcomes were observed according to long-term prophylaxis or availability of on-demand treatment at home. In multivariable ordinal logistic regression, SF-36 Bodily Pain remained independently associated with higher attack burden (OR 0.97 per 1-point increase; 95% CI 0.94–1.00; p = 0.033).

In adults with HAE, recent attack burden is consistently associated with impaired quality of life, increased pain, and adverse coping patterns. Generic health measures, particularly bodily pain, appear highly sensitive to attack burden and may complement traditional clinical endpoints in HAE management and research.

Keywords: hereditary angioedema; quality of life; patient-reported outcomes; SF-36; HAE-QoL; attack burden