Symptom questionnaire quiz
Hereditary angioedema
Hereditary angioedema (HAE) is a rare disorder characterized by recurring episodes of severe swelling in various locations throughout the body, including the face, extremities, gastrointestinal tract, and airways1.
Symptoms often begin in childhood, worsen during puberty, and persist throughout life with unpredictable severity2.
HAE attacks may gradually worsen over the first 12–36 hours and then subside over 2–5 days3.
Although most HAE attacks occur spontaneously, it is important to identify possible triggers. HAE triggers may include: physical trauma, psychological stress, use of angiotensin-converting enzyme inhibitors, use of estrogen-containing drugs, and infectious diseases3.
HAE affects 1/50,000 people worldwide1
In HAE, 93%
of patients experience abdominal attacks, which are characterized by abdominal pain3
Consequences of HAE attacks if left untreated
- Unnecessary surgical interventions
In HAE, more than 50%
of patients will experience more than one attack of laryngeal edema during their lifetime3
Consequences of HAE attacks if left untreated
- Asphyxia 4
- Lethal laryngeal edema 4
Improved understanding of the pathophysiology of HAE has facilitated the development of new targeted therapies. Attack-terminating drugs have gradually improved patient safety and quality of life. Effective and safe preventive treatments now offer patients with HAE a path to a normal life.
Resourses
- Lopez M A. Hereditary Angioedema Epidemiology. Rare Disease Advisor. (2022)[online]. Link: https://www.rarediseaseadvisor.com/disease-info-pages/hereditary-angioedema-epidemiology/.
- Chong-Neto HJ. A narrative review of recent literature of the quality of life in hereditary angioedema patients. World Allergy Organ J. 2023 Mar 20;16(3):100758. doi: 10.1016/j.waojou.2023.100758. PMID: 36994443; PMCID: PMC10040818. Link: https://pmc.ncbi.nlm.nih.gov/articles/PMC10040818/.
- Bork K, Anderson JT, Caballero T, Craig T, Johnston DT, Li HH, Longhurst HJ, Radojicic C, Riedl MA. Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report. Allergy Asthma Clin Immunol. 2021 Apr 19;17(1):40. doi: 10.1186/s13223-021-00537-2. PMID: 33875020; PMCID: PMC8056543. Link: https://pmc.ncbi.nlm.nih.gov/articles/PMC8056543/.
- Busse PJ, Christiansen SC. Hereditary Angioedema. N Engl J Med. 2020 Mar 19;382(12):1136-1148. doi: 10.1056/NEJMra1808012. PMID: 32187470. Link: https://pubmed.ncbi.nlm.nih.gov/32187470/.
- Doctor discussion guide. (2022). Available at: https://th.knowhae.com/sites/default/files/2022-03/discussion-guide.pdf [Accessed 12 Nov. 2024].
- Henao, M. P., Craig, T., Kraschnewski, J., & Kelbel, T. (2016). Diagnosis and screening of patients with hereditary angioedema in primary care. Therapeutics and Clinical Risk Management, 12, 701. https://doi.org/10.2147/TCRM.S86293.
- Shams, M., Laney, D. A., Jacob, D. A., Yang, J., Dronen, J., Logue, A., Rosen, A., & Riedl, M. (2022). Validation of a suspicion index to identify patients at risk for hereditary angioedema. Journal of Allergy and Clinical Immunology Global, 2(1), 76–78. https://doi.org/10.1016/j.jacig.2022.08.009.
- Stuart, A. (2023). Hereditary Angioedema: Causes, Symptoms, and Treatment. WebMD. Available at: https://www.webmd.com/skin-problems-and-treatments/hereditary-angioedema.
- Maurer, M., Magerl, M., Betschel, S., Aberer, W., Ansotegui, I. J., Aygören‐Pürsün, E., Banerji, A., Bara, N., Boccon‐Gibod, I., Bork, K., Bouillet, L., Boysen, H. B., Brodszki, N., Busse, P. J., Bygum, A., Caballero, T., Cancian, M., Castaldo, A., Cohn, D. M., & Csuka, D. (2022). The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update. Allergy, 77(7), 1947-1980. https://doi.org/10.1111/all.15214.