D2.350 - Exploring the Psychological Burden of Hereditary Angioedema Using the Pictorial Representation of Illness and Self Measure (PRISM): Updated Insights from a Prospective Real-Life Cohort

Patients with hereditary angioedema (HAE) face physical and psychosocial difficulties. While AE-QoL and AECT assess quality of life (QoL) and disease control, they may not fully capture emotional experience. The Pictorial Representation of Illness and Self Measure (PRISM) visually quantifies psychological burden; however, its performance in HAE remains unexplored. This study evaluated PRISM in a real-life cohort and its associations with clinical parameters and long-term prophylaxis (LTP).

This monocentric observational study included consecutive HAE patients during routine follow-up. Psychological burden was evaluated using PRISM Self-Illness Separation (SIS) and Self-Medical Care Separation (SMcS). Parallel assessments included AE-QoL, AECT, demographics, and LTP status. SIS discriminative ability for impaired QoL and poor control was tested using ROC analysis. Patient verbatims were analyzed using an AI-based clustering model.

Fifty-four patients were included (59% female; mean age 33.6 ±15.6 years). Most had HAE-C1INH type 1 (78%); 37% received LTP (lanadelumab n=12, berotralstat n=6, androgens n=2). SIS correlated negatively with AE-QoL (p=0.0001), indicating increased psychosocial burden with worsening QoL. ROC analyses confirmed SIS ability to discriminate impaired QoL (AE-QoL ≥ 24; AUC = 0.81) and poor control (AECT < 10; AUC = 0.79). Patients without LTP had higher SIS values (median 13.5 cm) than those without LTP (median 7.9 cm), suggesting lower psychosocial burden. SMcS did not differ by treatment status and showed no correlation with AE-QoL or AECT. No significant differences were observed between LTP treatments across patient reported outcome (PROs) or PRISM parameters (p>0.05). Verbatim analysis showed distinct thematic profiles: patients without LTP more frequently described attack-related daily impact and disease-management difficulties (16.2% and 12.2% of corpus), whereas those on LTP more often reported integration of lifelong treatment and family-related concerns (25% and 22.5%).

PRISM is a simple and effective tool for assessing psychological burden in HAE. SIS correlates strongly with QoL impairment and shows meaningful discriminative performance for impaired QoL and poor disease control. PRISM captures dimensions beyond traditional PROs and may be useful in populations with communication challenges. Multicentric evaluations are warranted to confirm its responsiveness and refine its role in multidisciplinary HAE care.