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. 2026 Feb 24:10:1-10.
doi: 10.5414/ALX02603E. eCollection 2026.

Achieving a normal life in hereditary angioedema: Quality of life and treatment gaps among German HAE patients

Markus Magerl  1   2 , Thomas Buttgereit  1   2 , Inmaculada Martinez-Saguer  3 , Petra Staubach-Renz  4 , Jens Greve  5 , Emel Aygören-Pürsün  6 , Lucia Schauf  7 , Kathrin Schön  1   2   7
Affiliations

Achieving a normal life in hereditary angioedema: Quality of life and treatment gaps among German HAE patients

Markus Magerl et al. Allergol Select. .

Abstract

Background: Hereditary angioedema (HAE) is a rare genetic disease characterized by recurrent swelling attacks. Current guidelines for HAE management emphasize achieving complete disease control to normalize patients' lives. Quality of life (QoL) differences between patients with 0 attacks and those with persistent attacks remain to be explored.

Materials and methods: The German patient organization for individuals affected by hereditary angioedema, HAE Vereinigung e.V. conducted an online survey with 122 HAE patients in Germany in 2024. Participants were categorized according to their therapy - long-term prophylaxis (LTP) or on-demand therapy (ODT) - and according to their attack frequency over the last 6 months. Patient-reported outcomes for functional, emotional, and social impacts were analyzed to evaluate QoL.

Results: Although 83% of patients expressed satisfaction with their treatment, 59% of patients still had attacks. Patients on LTP reported significantly fewer attacks (p < 0.001) and higher QoL compared to those on ODT (p < 0.001). Patients with 0 attacks consistently showed significantly better outcomes across all QoL domains than those with 1 or more attacks (p < 0.001).

Conclusion: The findings highlight that even minimal residual disease activity can meaningfully reduce QoL. Achieving complete attack freedom, rather than partial control, is necessary to restore normalcy for patients with HAE. Hence, regular adjustments of the HAE management plans based on patient-reported outcomes are crucial to ensure that treatment strategies address both medical and QoL needs.

Keywords: HAE guidelines; attack frequency; emotional impact; hereditary angioedema; long-term prophylaxis; on-demand therapy; patient-reported outcomes; quality of life; social engagement.

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Conflict of interest statement

M. Magerl is or recently was a speaker and/or advisor for and/or has received research funding from BioCryst, Pharming, Takeda, CSL Behring, Intellia, Ionis Pharmaceuticals/ Otsuka, KalVista, and Pharvaris. T. Buttgereit is or recently was a speaker and/or advisor for Almirall, Aquestive, AstraZeneca, Biocryst, CSL-Behring, Galderma, GlaxoSmithKline, Hexal, KalVista, Medac, Novartis, Pharming, Pharvaris, Otsuka, Roche, Sanofi, Swixx BioPharma and Takeda. I. Martinez-Saguer is or recently was a speaker and/or advisor for and/or has received research funding from BioCryst, CSL Behring, KalVista, Pharming, Pharvaris, Octapharma, and Takeda. P. Staubach-Renz is or recently was speaker and/or advisor for BioCryst, CSL Behring, KalVista, Octapharma, and Takeda. J. Greve has received speaker/consultancy fees from CSL Behring, Kalvista, Otsuka, Takeda and BioCryst; he has also received funding to attend conferences/educational events from CSL Behring, Biocryst, and; he has participated as an investigator in a clinical trial/registry for CSL Behring, Pharvaris, BioCryst, and Takeda. E. Aygören-Pürsün has received grants from CSL Behring and Takeda and consulting fees from Astria, CSL Behring, BioCryst, Intellia, KalVista, Pharvaris, and Takeda (honoraria personal or to the institution); she has received speaker fees from CSL Behring, BioCryst, Centogene, Pharming, and Takeda, and has served as an advisor for Astria, BioCryst, BioMarin, Centogene, CSL Behring, Intellia, KalVista, Pharming, Pharvaris, and Takeda. L. Schauf declares no relevant conflicts of interest in relation to this work. K. Schön is or recently was speaker and/or advisor for BioCryst, CSL Behring, and Takeda. Table 1.Patient characteristics. HAE subtypeLTP (n = 68)ODT (n = 54)Total (n = 122) HAE-C1INH type I52 (76.5%)36 (66.7%)88 (72.1%) HAE-C1INH type II5 (7.4%)2 (3.7%)7 (5.7%) HAE-nC1INH7 (10.3%)4 (7.4%)11 (9.0%) HAE type unknown4 (5.9%)12 (22.2%)16 (13.1%)Age group (years)LTP (n = 68)ODT (n = 54)Total (n = 122) < 181 (1.5%)6 (11.1%)7 (5.7%) 18 – 258 (11.8%)6 (11.1%)14 (11.5%) 26 – 3515 (22.1%)17 (31.5%)32 (26.2%) 36 – 459 (13.2%)5 (9.3%)14 (11.5%) 46 – 5515 (22.1%)10 (18.5%)25 (20.5%) 56 – 6516 (23.5%)7 (13.0%)23 (18.9%) 654 (5.9%)3 (5.6%)7 (5.7%)LTP = long-term prophylaxis; ODT = on-demand therapy; HAE-C1INH = hereditary angioedema with C1 inhibitor deficiency; HAE-nC1INH = hereditary angioedema with normal C1 inhibitor. Percentages are within-treatment group totals. Figure 1Comparison of attack frequency in the past 6 months of patients with hereditary angioedema on LTP vs. ODT. LTP = long-term prophylaxis; ODT = on-demand therapy. Figure 2Functional impact by attack frequency in the past 6 months. Figure 3Emotional impact by attack frequency in the past 6 months. Mean scores and standard deviations (SD) for emotional burden by attack frequency category: 0 attacks (green), 1 – 3 attacks (orange), and > 3 attacks (red). Significant differences between groups are indicated by horizontal bars with corresponding p-values. Scores based on a 5-point Likert scale ranging from 1 (not at all) to 5 (very often). Figure 4Social impact by attack frequency in the past 6 months.

Figures

Figure 1
Figure 1. Comparison of attack frequency in the past 6 months of patients with hereditary angioedema on LTP vs. ODT. LTP = long-term prophylaxis; ODT = on-demand therapy.
Figure 2
Figure 2. Functional impact by attack frequency in the past 6 months.
Figure 3
Figure 3. Emotional impact by attack frequency in the past 6 months. Mean scores and standard deviations (SD) for emotional burden by attack frequency category: 0 attacks (green), 1 – 3 attacks (orange), and > 3 attacks (red). Significant differences between groups are indicated by horizontal bars with corresponding p-values. Scores based on a 5-point Likert scale ranging from 1 (not at all) to 5 (very often).
Figure 4
Figure 4. Social impact by attack frequency in the past 6 months.
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