Clinical outcomes of dental procedures in patients with hereditary angioedema

Year 2025, Volume: 38 Issue: 3, 221 - 226, 10.10.2025

Hazal Kayıkçı , Ebru Damadoglu Melek Cihanbeylerden , Çise Tüccar Ayşegül Pehlivanlar Ustaoglu Gül Karakaya , Ali Fuat Kalyoncu

https://doi.org/10.5472/marumj.1798681

Abstract

Objective: To investigate the relationship between angioedema (AE) attacks and dental procedures in patients with hereditary
angioedema (HAE) and to identify potential risk factors.
Patients and Methods: Patients diagnosed with HAE between 1999 and 2024 at a tertiary adult allergy clinic were retrospectively
reviewed for dental procedures and AE attacks.
Results: Of 102 HAE patients, 74 were included after excluding 28 with incomplete data. Dental procedures were performed in 47
patients (63.5%), totaling 85 interventions, most commonly tooth extractions (50.6%), restorations (34.1%), and root canal treatments
(11.8%). Preprocedural prophylaxis was administered in 46 cases (54.1%), primarily with plasma-derived C1-inhibitor (pdC1-INH) (n
= 36), followed by danazol (n = 7) and icatibant (n = 3). A total of 17 AE attacks (20%) were recorded, all involving the oropharyngeal
region. Prophylaxis significantly reduced attack rates (p = 0.022), with no significant difference between pdC1-INH and danazol (p =
0.572). In the pdC1-INH group, attack rates were similar for 500 IU and 1000 IU doses (11.1% vs. 14.8%, p = 0.781).
Conclusion: Preprocedural prophylaxis reduced AE attack frequency in dental procedures, with similar outcomes for pdC1-INH and
danazol. Similar results were also observed between 500 IU and 1000 IU pdC1-INH.

Keywords

Hereditary angioedema , Dental procedures , Angioedema prophylaxis

References

• Caballero T, Baeza ML, Cabañas R, et al. Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. Treatment, follow-up, and special situations. J Investig Allergol Clin Immunol 2011;21:422-3. doi: 10.18176/jiaci.00000.
• Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med. 2008;359(10):1027-36. doi: 10.1056/NEJMcp0803977.
• Maurer M, Magerl M, Betschel S, et al. The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update. Allergy 2022;77:1961-90. doi: 10.1111/all.15214.
• Agostoni A, Cicardi M. Hereditary and acquired C1- inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore). 1992;71:206-15. doi: 10.1097/00005.792.199207000-00003.
• Nordenfelt P, Dawson S, Wahlgren CF, Lindfors A, Mallbris L, Bjorkander J. Quantifying the burden of disease and perceived health state in patients with hereditary angioedema in Sweden. Allergy Asthma Proc 2014;35:185-90. doi: 10.2500/ aap.2014.35.3738.
• Bork K, Hardt J, Staubach-Renz P, Witzke G. Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: a retrospective study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:58-64. doi: 10.1016/j.tripleo.2011.02.034.
• Bork K, Barnstedt SE. Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. J Am Dent Assoc 2003;134:1088-94. doi: 10.14219/jada.archive.2003.0323.
• Farkas H, Zotter Z, Csuka D, et al. Short-term prophylaxis in hereditary angioedema due to deficiency of the C1- inhibitor—A long-term survey. Allergy 2012;67:1586-93. doi: 10.1111/all.12032.
• Magerl M, Frank M, Lumry W, et al. Short-term prophylactic use of C1-inhibitor concentrate in hereditary angioedema: Findings from an international patient registry. Ann Allergy Asthma Immunol 2017;118(1):110-12. doi: 10.1016/j. anai.2016.10.006.
• Craig T, Aygören-Pürsün E, Bork K, et al. WAO guideline for the management of hereditary angioedema. World Allergy Organ J 2012;5:182-99. doi: 10.1097/WOX.0b013e318279affa.
• Varandas C, Esteves Caldeira L, Silva SL, et al. Hereditary angioedema: 24 years of experience in a Portuguese Reference Center. Eur Ann Allergy Clin Immunol 2024;56:210-8. doi: 10.23822/EurAnnACI.1764-1489.278.
• Bork K, Gül D, Hardt J, Dewald G. Hereditary angioedema with normal C1 inhibitor: clinical symptoms and course. Am J Med 2007;120:987-92. doi: 10.1016/j.amjmed.2007.08.021.
• Zanichelli A, Magerl M, Longhurst H, Fabien V, Maurer M. Hereditary angioedema with C1 inhibitor deficiency: delay in diagnosis in Europe. Allergy Asthma Clin Immunol 2013;9:29. doi: 10.1186/1710-1492-9-29.
• Guryanova I, Suffritti C, Parolin D, et al. Hereditary angioedema due to C1 inhibitor deficiency in Belarus: epidemiology, access to diagnosis and seven novel mutations in SERPING1 gene. Clin Mol Allergy 2021;19:3. doi: 10.1186/ s12948.021.00141-0.
• Cicardi M, Bork K, Caballero T, et al. Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group. Allergy 2012;67:147-57. doi: 10.1111/j.1398-9995.2011.02751.x.
• Papamanthos M, Matiakis A, Tsirevelou P, Kolokotronis A, Skoulakis H. Hereditary angioedema: three cases report, members of the same family. J Oral Maxillofac Res 2010;1:e9. doi: 10.5037/jomr.2010.1109.
• Iturri Clavero F, González Uriarte A, Tamayo Medel G, Gamboa Setién PM. Prophylactic use of icatibant before tracheal intubation of a patient with hereditary angioedema type III. (A literature review of perioperative management of patients with hereditary angioedema type III). Rev Esp Anestesiol Reanim 2014;61:375-81. doi: 10.1016/j.redar.2014.01.011.
• Caballero T. Treatment of hereditary angioedema. J Investig Allergol Clin Immunol 2021;31:1-16. doi: 10.18176/jiaci.0653.
• Caballero Molina T, Pedrosa Delgado M, Gómez Traseira C. Angioedema hereditario. Med Clin (Barc) 2015;145:356-65. doi: 10.1016/j.medcli.2014.12.012.
• Jurado-Palomo J, Muñoz-Caro JM, López-Serrano MC, et al. Management of dental-oral procedures in patients with hereditary angioedema due to C1 inhibitor deficiency. J Investig Allergol Clin Immunol 2013;23:1-6.