Alergologia Polska - Polish Journal of Allergology
eISSN: 2391-6052
ISSN: 2353-3854
Alergologia Polska - Polish Journal of Allergology
Current issue Archive Manuscripts accepted About the journal Supplements Special issues Editorial board Abstracting and indexing Subscription Contact Instructions for authors Publication charge Ethical standards and procedures
Editorial System
Submit your Manuscript
4/2025
vol. 12
 
Share:
Share:
Guidelines/recommendations

Position statement of the HAE Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part II: treatment, monitoring, perioperative management, and the particular aspects of care for women and children

Grzegorz Porębski
1
,
Aleksandra Kucharczyk
2
,
Tomasz Matuszewski
2
,
Hanna Danielewicz
3
,
Jacek Gocki
4
,
Katarzyna Grzela
5
,
Aldona Juchacz
6
,
Marcin Kurowski
7
,
Krzysztof Kuziemski
8
,
Mateusz Łukaszyk
9
,
Małgorzata Sokołowska
10
,
Marcin Stobiecki
1
,
Ewa Trębas-Pietraś
11
,
Marta Tykwińska
12
,
Ewa Czarnobilska
1
,
Marcin Moniuszko
13
,
Krystyna Obtułowicz
1

  1. Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland
  2. Department of Internal Diseases, Pneumonology, Allergology, Clinical Immunology and Rare Diseases, Military Institute of Medicine – National Research Institute, Warsaw, Poland
  3. 1st Clinical Department of Pediatrics, Allergology and Cardiology, Wroclaw Medical University, Wroclaw, Poland
  4. ALERGODERM, Specialist Medical Practice, Bydgoszcz, Poland
  5. Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw, Poland
  6. Greater Poland Center of Pulmonology and Thoracic Surgery, Poznan, Poland
  7. Department of Immunology and Allergy, Central Teaching Hospital of the Medical University of Lodz, Poland
  8. Department of Pulmonology and Allergology, Faculty of Medicine, Medical University of Gdansk, Poland
  9. 1st Department of Lung Diseases and Tuberculosis, Medical University of Bialystok, Poland
  10. Independent Public Clinical Hospital No. 2, Pomeranian Medical University, Szczecin, Poland
  11. Department of Allergology and Lung Diseases, Cardinal Wyszyński Provincial Specialist Hospital SPZOZ, Lublin, Poland
  12. Department of Allergology, Clinical Immunology and Internal Medicine, University Hospital No. 2, Bydgoszcz, Poland
  13. Department of Allergology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
Alergologia Polska – Polish Journal of Allergology 2025; 12, 4: 233–249
Online publish date: 2025/11/21
Article file
- Position statement.pdf  [0.34 MB]
Get citation
 
PlumX metrics:
 
1. Zuraw BL, Bork K, Bouillet L, et al. Hereditary angioedema with normal C1 inhibitor: an updated international consensus paper on diagnosis, pathophysiology, and treatment. Clin Rev Allergy Immunol 2025; 68: 24.
2. 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.
3. Summary of Product Characteristics Berinert http://ec.europa.eu (access 24.09.2024).
4. Summary of Product Characteristics Ruconest https://ec.europa.eu (access 24.09.2024).
5. Summary of Product Characteristics Firazyr http://leki.urpl.gov.pl/ (access 24.09.2024).
6. Riedl MA, Farkas H, Aygören-Pürsün E, et al. Oral sebetralstat for on-demand treatment of hereditary angioedema attacks. N Engl J Med 2024; 391: 32-43.
7. U.S. Food and Drug Administration. Sebetralstat (Ekterly) prescribing information. FDA. https://www.accessdata.fda.gov/drugsatfda_docs/label/2025/219301s000lbl.pdf.
8. Longhurst HJ, Farkas H, Craig T, et al. HAE international home therapy consensus document. Allergy Asthma Clin Immunol 2010; 6: 22.
9. Bygum A, Andersen KE, Mikkelsen CS. Self-administration of intravenous C1-inhibitortherapy for hereditary angioedema and associated quality of life benefits. Eur J Dermatol 2009; 19: 147-51.
10. Li HH. Self-administered C1 esterase inhibitor concentrates for the management of hereditary angioedema: usability and patient acceptance. Patient Prefer Adherence 2016; 10: 1727-37.
11. Wilson D, Bork K, Shea E, Rentz A, et al. Economic costs associated with acute attacks and long-term management of hereditary angioedema. Ann Allergy Asthma Immunol 2010; 104: 314-20.
12. Zilberberg MD, Jacobsen T, Tillotson G. The burden of hospitalizations and emergency department visits with hereditary angioedema and angioedema in the United States, 2007. Allergy Asthma Proc 2010; 31: 511-9.
13. Betschel S, Badiou J, Binkley K, et al. The International/Canadian Hereditary Angioedema Guideline. Allergy Asthma Clin Immunol 2019; 15: 72.
14. 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.
15. Bowen T, Cicardi M, Farkas H, et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol 2010; 6: 24.
16. Frank MM. Update on preventive therapy (prophylaxis) for hereditary angioedema. Immunol Allergy Clin North Am 2013; 33: 495-503.
17. Porebski G, Reshef A, Moldovan D. The prophylaxis of hereditary angioedema attacks with recombinant human C1 inhibitor: who will take advantage of the individualized treatment approach? Allergy 2013; 68: 1207-9.
18. Bernstein JA, Manning ME, Li H, et al. Escalating doses of C1 esterase inhibitor (CINRYZE) for prophylaxis in patients with hereditary angioedema. J Allergy Clin Immunol Pract 2014; 2: 77-84.
19. Longhurst H, Cicardi M, Craig T, et al. Prevention of hereditary angioedema attacks with a subcutaneous C1 inhibitor. N Engl J Med 2017; 376: 1131-40.
20. Walsh S, Bartlett M, Salvo-Halloran EM, et al. Network meta-analysis of pharmacological therapies for long-term prophylactic treatment of patients with hereditary angioedema. Drugs R D 2025; 25: 161-78.
21. Banerji A, Riedl MA, Bernstein JA, et al. Effect of lanadelumab compared with placebo on prevention of hereditary angioedema attacks: a randomized clinical trial. JAMA 2018; 320: 2108-21.
22. Kucharczyk A, Porębski G, Rząd M, et al. Lanadelumab demonstrates high efficacy in reducing the frequency of angioedema attacks in patients with severe HAE in real-life settings. Pediatr Med Rodz 2023; 19: 334-42.
23. Magerl M, Bouillet L, Martinez-Saguer I, et al. Real-world effectiveness of lanadelumab in hereditary angioedema: multicountry INTEGRATED observational study. J Allergy Clin Immunol Pract 2025; 13: 378-87.e2.
24. Li HH, Zuraw B, Longhurst HJ, et al. Subcutaneous C1 inhibitor for prevention of attacks of hereditary angioedema: additional outcomes and subgroup analysis of a placebo-controlled randomized study. Allergy Asthma Clin Immunol 2019; 15: 49.
25. Zuraw BL, Cicardi M, Longhurst HJ, et al. Phase II study results of a replacement therapy for hereditary angioedema with subcutaneous C1-inhibitor concentrate. Allergy 2015; 70: 1319-28.
26. Craig T, Zuraw B, Longhurst H, et al. Long-term outcomes with subcutaneous C1-inhibitor replacement therapy for prevention of hereditary angioedema attacks. J Allergy Clin Immunol Pract 2019; 7: 1793-802.e2.
27. Summary of product characteristics Orladeyo https://ec.europa.eu (access 24.09.2024).
28. Farkas H, Stobiecki M, Peter J, et al. Long-term safety and effectiveness of berotralstat for hereditary angioedema: the open-label APeX-S study. Clin Transl Allergy 2021; 11: e12035.
29. Farkas H, Peter JG, Stobiecki M, et al. Long-term safety and efficacy of once-daily berotralstat in patients with hereditary angioedema: APeX-S final results. Ann Allergy Asthma Immunol 2025; 135: 311-9.E6.
30. Craig TJ, Reshef A, Li HH, et al. Efficacy and safety of garadacimab, a factor XIIa inhibitor for hereditary angioedema prevention (VANGUARD): a global, multicentre, randomised, double-blind, placebo-controlled, phase 3 trial. Lancet 2023; 401: 1079-90.
31. Summary of product characteristics Andembry https://ec.europa.eu/health/documents/community-register/2025/20250210165019/anx_165019_pl.pdf; access 22.02.2025.
32. Aribarg A, Sukcharoen N, Chanprasit Y, et al. Suppression of spermatogenesis by testosterone enanthate in Thai men. J Med Assoc Thai 1996; 79: 624-9.
33. Matsumoto AM. Effects of chronic testosterone administration in normal men: safety and efficacy of high dosage testosterone and parallel dose-dependent suppression of luteinizing hormone, follicle-stimulating hormone, and sperm production. J Clin Endocrinol Metab 1990; 70: 282-7.
34. Porębski G, Gocki J, Juchacz A, et al. Postępowanie we wrodzonym obrzęku naczynioruchowym z niedoboru inhibitora C1– stanowisko Sekcji HAE Polskiego Towarzystwa Alergologicznego. Część II: leczenie i zapobieganie napadom, monitorowanie choroby i postępowanie w sytuacjach szczególnych. Pol J Allergol 2018; 5: 109-20.
35. Füst G, Farkas H, Csuka D, et al. Long-term efficacy of danazol treatment in hereditary angioedema. Eur J Clin Invest 2011; 41: 256-62.
36. Zuraw BL, Davis DK, Castaldo AJ, Christiansen SC. Tolerability and effectiveness of 17-α-alkylated androgen therapy for hereditary angioedema: a re-examination. J Allergy Clin Immunol Pract 2016; 4: 948-55.e15.
37. Brunskill PJ. The effects of fetal exposure to danazol. BJOG 1992; 99: 212-5.
38. Wentz AC. Adverse effects of danazol in pregnancy. Ann Intern Med 1982; 96: 672-3.
39. Pagnier A, Dermesropian A, Kevorkian-Verguet C, et al. Hereditary angioedema in children: review and practical perspective for clinical management. Pediatr Allergy Immunol 2024; 35: e14268.
40. Sheffer AL, Austen KF, Rosen FS. Tranexamic acid therapy in hereditary angioneurotic edema. N Engl J Med 1972; 287: 452-4.
41. Blohmé G. Treatment of hereditary angioneurotic oedema with tranexamic acid. A random double-blind cross-over study. Acta Med Scand 1972; 192: 293-8.
42. Jacobs J, Adatia A, Craig T, et al. Treatment with Navenibart (STAR-0215) reduces attack severity and use of rescue medication in patients with hereditary angioedema (HAE): interim results from the ALPHA-STAR Trial. J Allergy Clin Immunol 2025; 155: AB196.
43. Pharvaris. Abstracts on long-term safety and efficacy of oral deucrictibant accepted for presentation at 2025 angioedema congresses. Press release. May 19, 2025. Available from: https://ir.pharvaris.com/news-releases/news-release-details/pharvaris-announces-abstract-acceptance-presentation-key-angioedema-congresses-2025 (accessed 2025).
44. Petersen RS, Bordone L, Riedl MA, et al. A phase 2 open-label extension study of prekallikrein inhibition with donidalorsen for hereditary angioedema. Allergy 2024; 79: 724-34.
45. Cohn DM, Gurugama P, Magerl M, et al. CRISPR-based therapy for hereditary angioedema. N Engl J Med 2025; 392: 458-67.
46. 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.
47. 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.
48. 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: 110-2.
49. Valerieva A, Staevska M, Jesenak M, et al. Recombinant human C1 esterase inhibitor as short-term prophylaxis in patients with hereditary angioedema. J Allergy Clin Immunol Pract 2020; 8: 799-802.
50. Lochbaum R, Hoffmann TK, Greve J, Hahn J. Analysis of prodromal symptoms and need for short-term prophylaxis in angioedema patients under long-term prophylaxis. Orphanet J Rare Dis 2025; 20: 47.
51. Frank MM. Hereditary angioedema: short-term prophylaxis for surgery. Allergy Asthma Proc 2012; 33: 303-4.
52. Aygören-Pürsün E, Martinez Saguer I, Kreuz W, et al. Risk of angioedema following invasive or surgical procedures in HAE type I and II--the natural history. Allergy 2013; 68: 1034-9.
53. Barbara DW, Ronan KP, Maddox DE, Warner MA. Perioperative angioedema: background, diagnosis, and management. J Clin Anesth 2013; 25: 335-43.
54. Busse PJ, Christiansen SC, Riedl MA, et al. US HAEA Medical Advisory Board 2020 Guidelines for the management of hereditary angioedema. J Allergy Clin Immunol Pract 2021; 9: 132-50.e3.
55. Zarnowski J, Treudler R. Dietary and physical trigger factors in hereditary angioedema: self-conducted investigation and literature overview. Allergol Select 2024; 8: 358-64.
56. Savarese L, Mormile I, Bova M, et al. Psychology and hereditary angioedema: a systematic review. Allergy Asthma Proc 2021; 42: e1-e7.
57. Farkas H, Martinez-Saguer I, Bork K, et al. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency. Allergy 2017; 72: 300-13.
58. Sebastian R, Tobias JD. Perioperative care of a patient with hereditary angioedema. Pediatr Anesthesia Critical Care J 2014; 2: 19-25.
59. Rustecki B, Rychlik A, Żylińska A. Dziedziczny obrzęk naczynioruchowy – przygotowanie pacjenta do zabiegu operacyjnego. Pediatr Med Rodz 2012; 8: 165-7.
60. Kido K, Kato T, Kamiya S, et al. Anesthetic management with remimazolam for a patient with hereditary angioedema:a case report. J Med Invest 2024; 71: 184-6.
61. Magerl M, Sala-Cunill A, Weber-Chrysochoou C, et al. Could it be hereditary angioedema?-Perspectives from different medical specialties. Clin Transl Allergy 2023; 13: e12297.
62. Craig T. Triggers and short-term prophylaxis in patients with hereditary angioedema. Allergy Asthma Proc 2020; 41 (Suppl 1): S30-4.
63. Lodi G, Sardella A, Bez C, et al. Dental experience and self-perceived dental care needs of patients with angioedema. Spec Care Dentist 2001; 21: 27-31.
64. Rosa A, Franco R, Miranda M, et al. The role of anxiety in patients with hereditary angioedema during oral treatment: a narrative review. Front Oral Health 2023; 4: 1257703.
65. Tanaka KA, Mondal S, Morita Y, et al. Perioperative management of patients with hereditary angioedema with special considerations for cardiopulmonary bypass. Anesth Analg 2020; 131: 155-69.
66. Topyildiz E, Senol HD, Gulen F, et al. Successful treatment of post-pericardiotomy syndrome via C1 inhibitor replacement therapy in a hereditary angioedema patient with Marfan syndrome. Turk J Pediatr 2023; 65: 338-43.
67. Lochbaum R, Hoffmann TK, Greve J, Hahn J. Concomitant medication in patients with bradykinin-mediated angioedema - there’s more than ACE inhibitors. J Dtsch Dermatol Ges 2023; 21: 1283-9.
68. Martinez-Saguer I, Farkas H. Erythema marginatum as an early symptom of hereditary angioedema: case report of 2 newborns. Pediatrics 2016; 137: e20152411.
69. Sanchez A, Ecochard A, Maestracci M, Rodiere M. [Hereditary angioedema causing colocolic intussusception]. Arch Pediatr 2008; 15: 271-4.
70. Bork K, Siedlecki K, Bosch S, et al. Asphyxiation by laryngeal edema in patients with hereditary angioedema. Mayo Clin Proc 2000; 75: 349-54.
71. Bork K, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol 2012; 130: 692-7.
72. Caballero T, Baeza ML, Cabañas R, et al. Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part I. Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis. J Investig Allergol Clin Immunol 2011; 21: 333-47; quiz follow 47.
73. Balla Z, Andrási N, Pólai Z, et al. The characteristics of upper airway edema in hereditary and acquired angioedema with C1-inhibitor deficiency. Clin Transl Allergy 2021; 11: e12083.
74. Zanichelli A, Magerl M, Longhurst H, et al. Hereditary angioedema with C1 inhibitor deficiency: delay in diagnosis in Europe. Allergy Asthma Clin Immunol 2013; 9: 29.
75. Pedrosa M, Phillips-Angles E, López-Lera A, et al. Complement study versus CINH gene testing for the diagnosis of type I hereditary angioedema in children. J Clin Immunol 2016; 36: 16-8.
76. Summary of product characteristics Takhzyro https://www.ema.europa.eu/pl/documents/product-information/takhzyro-epar-product-information_pl.pdf; access 17.02.2025.
77. Farkas H. Pediatric hereditary angioedema due to C1-inhibitor deficiency. Allergy Asthma Clin Immunol 2010; 6: 18.
78. Agostoni A, Cicardi M. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore) 1992; 71: 206-15.
79. Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med 2006; 119: 267-74.
80. Bouillet L. Hereditary angioedema in women. Allergy Asthma Clin Immunol 2010; 6: 17.
81. Banerji A, Riedl M. Managing the female patient with hereditary angioedema. Womens Health 2016; 12: 351-61.
82. Cumming DC. Use of spironolactone in treatment of hirsutism. Cleve Clin J Med 1990; 57: 285-7.
83. Cumming DC, Yang JC, Rebar RW, Yen SS. Treatment of hirsutism with spironolactone. JAMA 1982; 247: 1295-8.
84. Bouillet L, Longhurst H, Boccon-Gibod I, et al. Disease expression in women with hereditary angioedema. Am J Obstet Gynecol 2008; 199: 484.e1-4.
85. Sim TC, Grant JA. Hereditary angioedema: its diagnostic and management perspectives. Am J Med 1990; 88: 656-64.
86. Caballero T, Farkas H, Bouillet L, et al. International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. J Allergy Clin Immunol 2012; 129: 308-20.
87. Borradori L, Marie O, Rybojad M, et al. Hereditary angioedema and oral contraception. Dermatologica 1990; 181: 78-9.
88. ESHRE Capri Workshop Group. Intrauterine insemination. Hum Reprod Update 2009; 15: 265-77.
89. Obtułowicz K, Porębski G, Bilo B, et al. Przebieg wrodzonego obrzęku naczynioruchowego z niedoboru C1 inhibitora u kobiet podczas ciąży i porodu. Alerg Astma Immunol 2008; 13: 91-9.
90. Czaller I, Visy B, Csuka D, et al. The natural history of hereditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey. Eur J Obstet Gynecol Reprod Biol 2010; 152: 44-9.
91. Martinez-Saguer I, Rusicke E, Aygören-Pürsün E, et al. Characterization of acute hereditary angioedema attacks during pregnancy and breast-feeding and their treatment with C1 inhibitor concentrate. Am J Obstet Gynecol 2010; 203: 131.e1-7.
92. Prematta M, Gibbs JG, Pratt EL, et al. Fresh frozen plasma for the treatment of hereditary angioedema. Ann Allergy Asthma Immunol 2007; 98: 383-8.
93. Visy B, Füst G, Varga L, et al. Sex hormones in hereditary angioneurotic oedema. Clin Endocrinol (Oxf) 2004; 60: 508-15.
94. Ott HW, Mattle V, Hadziomerovic D, et al. Treatment of hereditary angioneurotic oedema (HANE) with tibolone. Clin Endocrinol (Oxf) 2007; 66: 180-4.
95. Rousset-Jablonski C, Thalabard JC, Gompel A. Tamoxifen contraindicated in women with hereditary angioedema? Ann Oncol 2009; 20: 1281-2.
96. Lacey JV Jr, Brinton LA, Barnes WA, et al. Use of hormone replacement therapy and adenocarcinomas and squamous cell carcinomas of the uterine cervix. Gynecol Oncol 2000; 77: 149-54.
97. Obtułowicz K. Current status of diagnosis, registry and management of hereditary angioedema (HAE) in Poland. Allergol Immunol 2016; 3-4: 19-21.
Copyright: © Polish Society of Allergology This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No Derivatives 4.0 International (CC BY-NC-SA 4.0). License (http://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.


Quick links
© 2025 Termedia Sp. z o.o.
Developed by Bentus.