Hereditary angioedema (HAE) in Belgium: results from a national survey

M M Van der Poorten; R Schrijvers; C Hermans; M Bartiaux; F Haerynck; H Lapeere; M Moutschen; O Michel; V Sabato; D G Ebo; A L Van Gasse

doi:10.3389/falgy.2023.1143897

Hereditary angioedema (HAE) in Belgium: results from a national survey

Front Allergy. 2023 May 26:4:1143897. doi: 10.3389/falgy.2023.1143897. eCollection 2023.

Authors

M M Van der Poorten^{1

2}, R Schrijvers³, C Hermans⁴, M Bartiaux⁵, F Haerynck⁶, H Lapeere⁷, M Moutschen⁸, O Michel⁹, V Sabato¹, D G Ebo¹, A L Van Gasse^{1

2}

Affiliations

¹ Faculty of Medicine and Health Science, Department of Immunology - Allergology - Rheumatology, Antwerp University Hospital and the Infla-Med Centre of Excellence, University of Antwerp, Antwerp, Belgium.
² Faculty of Medicine and Health Science, Department of Paediatrics, University of Antwerp, Antwerp University Hospital, Antwerp, Belgium.
³ Faculty of Medicine, Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium.
⁴ Faculty of Medicine and Health Science, Department of Adult Haematology, Saint-Luc University Hospital, Brussels, Belgium.
⁵ Faculty of Medicine and Health Science, Department of Urgent Medicine, Hôpital Sient-Pierre, Brussels, Belgium.
⁶ Faculty of Medicine and Health Science, Department of Internal Medicine and Paediatrics, Ghent University Hospital, Ghent, Belgium.
⁷ Faculty of Medicine and Health Science, Department of Dermatology, Ghent University Hospital, Ghent, Belgium.
⁸ Faculty of Medicine and Health Science, Department of Internal Medicine and Infectious Diseases, C.H.U. de Liège - Site du Sart Tilman, Liège, Belgium.
⁹ Faculty of Medicine, Department of Immunology and Allergology, C.H.U. Brugmann, Brussels, Belgium.

Abstract

Background: Hereditary angioedema (HAE) is a rare heritable disorder that is characterized by recurrent, circumscribed, nonpitting, nonpruritic, often painful subepithelial swellings of sudden unpredictable onset that generally fade during 48-72 h. Epidemiological data of hereditary angioedema patients in Belgium is lacking.

Methods: We set up a nation-wide, multicentric study involving the 8 Belgian hospitals known to follow-up patients with Type I and II HAE. All Belgium HAE patients were asked to fill out questionnaires that mainly covered demographic data, family history, and detailed information about diagnosis, treatment and burden of their Type I and II HAE.

Results: 112 patients with type I or type II HAE could be included. Median delay between first symptoms and diagnosis was 7 years. 51% of patients had experienced pharyngeal or tongue swelling and 78% had experienced abdominal symptoms, both known to cause an important reduction in quality of life. 60% of symptomatic patients reported to receive long term prophylactic treatment. Human plasma-derived C1-esterase inhibitor concentrate was used by 56.3% of patients. 16.7% and 27.1% of patients used a 17-α-alkylated androgen and tranexamic acid as long term prophylactic therapy.

Conclusions: We present the first nation-wide epidemiological study regarding HAE in Belgium. Our data show that the morbidity of HAE is not to be underestimated. Knowledge and dissemination of this data is critical in raising awareness, encouraging development of therapies and optimising nationwide management.

Keywords: Belgium; diagnosis; epidemiology; hereditary angioedema; nationwide; rare disease.

Grants and funding

Rik Schrijvers, Didier Ebo and Vito Sabato are supported by the Fonds Wetenschappelijk Onderzoek—Vlaanderen National Fund for Scientific Research (FWO) senior clinical investigator fellowship (Project numbers 1805518N, 1804518N and 1800614N respectively).