Hereditary angioedema (HAE) in Belgium: results from a national survey
BackgroundHereditary 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 patient...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-05-01
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| Series: | Frontiers in Allergy |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/falgy.2023.1143897/full |
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| author | MM Van der Poorten MM Van der Poorten R Schrijvers C Hermans M Bartiaux F Haerynck H Lapeere M Moutschen O Michel V Sabato DG Ebo AL Van Gasse AL Van Gasse |
| author_facet | MM Van der Poorten MM Van der Poorten R Schrijvers C Hermans M Bartiaux F Haerynck H Lapeere M Moutschen O Michel V Sabato DG Ebo AL Van Gasse AL Van Gasse |
| author_sort | MM Van der Poorten |
| collection | DOAJ |
| description | BackgroundHereditary 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.MethodsWe 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.Results112 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.ConclusionsWe 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. |
| first_indexed | 2024-03-13T09:29:06Z |
| format | Article |
| id | doaj.art-4d1c5e88febe460ebaa71d0febe341ca |
| institution | Directory Open Access Journal |
| issn | 2673-6101 |
| language | English |
| last_indexed | 2024-03-13T09:29:06Z |
| publishDate | 2023-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Allergy |
| spelling | doaj.art-4d1c5e88febe460ebaa71d0febe341ca2023-05-26T04:27:22ZengFrontiers Media S.A.Frontiers in Allergy2673-61012023-05-01410.3389/falgy.2023.11438971143897Hereditary angioedema (HAE) in Belgium: results from a national surveyMM Van der Poorten0MM Van der Poorten1R Schrijvers2C Hermans3M Bartiaux4F Haerynck5H Lapeere6M Moutschen7O Michel8V Sabato9DG Ebo10AL Van Gasse11AL Van Gasse12Faculty of Medicine and Health Science, Department of Immunology – Allergology – Rheumatology, Antwerp University Hospital and the Infla-Med Centre of Excellence, University of Antwerp, Antwerp, BelgiumFaculty of Medicine and Health Science, Department of Paediatrics, University of Antwerp, Antwerp University Hospital, Antwerp, BelgiumFaculty of Medicine, Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, BelgiumFaculty of Medicine and Health Science, Department of Adult Haematology, Saint-Luc University Hospital, Brussels, BelgiumFaculty of Medicine and Health Science, Department of Urgent Medicine, Hôpital Sient-Pierre, Brussels, BelgiumFaculty of Medicine and Health Science, Department of Internal Medicine and Paediatrics, Ghent University Hospital, Ghent, BelgiumFaculty of Medicine and Health Science, Department of Dermatology, Ghent University Hospital, Ghent, BelgiumFaculty of Medicine and Health Science, Department of Internal Medicine and Infectious Diseases, C.H.U. de Liège - Site du Sart Tilman, Liège, BelgiumFaculty of Medicine, Department of Immunology and Allergology, C.H.U. Brugmann, Brussels, BelgiumFaculty of Medicine and Health Science, Department of Immunology – Allergology – Rheumatology, Antwerp University Hospital and the Infla-Med Centre of Excellence, University of Antwerp, Antwerp, BelgiumFaculty of Medicine and Health Science, Department of Immunology – Allergology – Rheumatology, Antwerp University Hospital and the Infla-Med Centre of Excellence, University of Antwerp, Antwerp, BelgiumFaculty of Medicine and Health Science, Department of Immunology – Allergology – Rheumatology, Antwerp University Hospital and the Infla-Med Centre of Excellence, University of Antwerp, Antwerp, BelgiumFaculty of Medicine and Health Science, Department of Paediatrics, University of Antwerp, Antwerp University Hospital, Antwerp, BelgiumBackgroundHereditary 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.MethodsWe 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.Results112 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.ConclusionsWe 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.https://www.frontiersin.org/articles/10.3389/falgy.2023.1143897/fullhereditary angioedemaepidemiologynationwideBelgiumdiagnosisrare disease |
| spellingShingle | MM Van der Poorten MM Van der Poorten R Schrijvers C Hermans M Bartiaux F Haerynck H Lapeere M Moutschen O Michel V Sabato DG Ebo AL Van Gasse AL Van Gasse Hereditary angioedema (HAE) in Belgium: results from a national survey Frontiers in Allergy hereditary angioedema epidemiology nationwide Belgium diagnosis rare disease |
| title | Hereditary angioedema (HAE) in Belgium: results from a national survey |
| title_full | Hereditary angioedema (HAE) in Belgium: results from a national survey |
| title_fullStr | Hereditary angioedema (HAE) in Belgium: results from a national survey |
| title_full_unstemmed | Hereditary angioedema (HAE) in Belgium: results from a national survey |
| title_short | Hereditary angioedema (HAE) in Belgium: results from a national survey |
| title_sort | hereditary angioedema hae in belgium results from a national survey |
| topic | hereditary angioedema epidemiology nationwide Belgium diagnosis rare disease |
| url | https://www.frontiersin.org/articles/10.3389/falgy.2023.1143897/full |
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