Arquivos de Asma, Alergia e Imunologia
https://www.aaai-asbai.org.br/article/doi/10.5935/2526-5393.20220020
Arquivos de Asma, Alergia e Imunologia
Artigo Especial

Diretrizes brasileiras de angioedema hereditário 2022 – Parte 2: terapêutica

2022 Brazilian guidelines for hereditary angioedema – Part 2: therapy

Régis A. Campos; Faradiba Sarquis Serpa; Eli Mansour; Maria Luiza Oliva Alonso; Luisa Karla Arruda; Marcelo Vivolo Aun; Maine Luellah Demaret Bardou; Ana Flávia Bernardes; Fernanda Lugão Campinhos; Herberto Jose Chong-Neto; Rosemeire Navickas Constantino-Silva; Jane da Silva; Sérgio Duarte Dortas-Junior; Mariana Paes Leme Ferriani; Joanemile Pacheco de Figueiredo; Pedro Giavina-Bianchi; Lais Souza Gomes; Ekaterini Goudouris; Anete Sevciovic Grumach; Marina Teixeira Henriques; Antônio Abilio Motta; Therezinha Ribeiro Moyses; Fernanda Leonel Nunes; Jorge A. Pinto; Nelson Augusto Rosario-Filho; Norma de Paula M. Rubini; Almerinda Maria do Rêgo Silva; Dirceu Solé; Ana Júlia Ribeiro Teixeira; Eliana Toledo; Camila Lopes Veronez; Solange Oliveira Rodrigues Valle

http://dx.doi.org/10.5935/2526-5393.20220020 Arq Asma Alerg Imunol, vol.6, n2, p.170-196, 2022
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Resumo

O tratamento do angioedema hereditário tem início com a educação dos pacientes e familiares sobre a doença, pois é fundamental o conhecimento da imprevisibilidade das crises, assim como os seus fatores desencadeantes. O tratamento medicamentoso se divide em terapia das crises e profilaxia das manifestações clínicas. As crises devem ser tratadas o mais precocemente possível com o uso do antagonista do receptor de bradicinina, o icatibanto ou o concentrado de C1-inibidor. É necessário estabeler um plano de ação em caso de crises para todos os pacientes. A profilaxia de longo prazo dos sintomas deve ser realizada preferencialmente com medicamentos de primeira linha, como concentrado do C1-inibidor ou o anticorpo monoclonal anti-calicreína, lanadelumabe. Como segunda linha de tratamento temos os andrógenos atenuados. Na profilaxia de curto prazo, antes de procedimentos que podem desencadear crises, o uso do concentrado de C1-inibidor é preconizado. Existem algumas restrições para uso desses tratamentos em crianças e gestantes que devem ser consideradas. Novos medicamentos baseados nos avanços do conhecimento da fisiopatologia do angioedema hereditário estão em desenvolvimento, devendo melhorar a qualidade de vida dos pacientes. O uso de ferramentas padronizadas para monitorização da qualidade de vida, do controle e da atividade da doença são fundamentais no acompanhamento destes pacientes. A criação de associações de pacientes e familiares de pacientes com angioedema hereditário tem desempenhado um papel muito importante no cuidado destes pacientes no nosso país.

Palavras-chave

Angioedema hereditário, tratamento farmacológico, tratamento de emergência, qualidade de vida, tratamento biológico.

Abstract

The treatment of hereditary angioedema begins with the education of patients and their families about the disease, as it is essential to know the unpredictability of attacks as well as their triggering factors. Drug treatment is divided into attack therapy and prophylaxis of clinical manifestations. Attacks should be treated as early as possible with the bradykinin receptor antagonist icatibant or C1-inhibitor concentrate. An action plan needs to be established for all patients with attacks. Long-term prophylaxis of symptoms should preferably be performed with first-line drugs such as C1-inhibitor concentrate or the anti-kallikrein monoclonal antibody lanadelumab. Attenuated androgens are the second line of treatment. In short-term prophylaxis, before procedures that can trigger attacks, the use of C1-inhibitor concentrate is recommended. There are some restrictions for the use of these treatments in children and pregnant women that should be considered. New drugs based on advances in knowledge of the pathophysiology of hereditary angioedema are under development and are expected to improve patient quality of life. The use of standardized tools for monitoring quality of life and controlling disease activity is essential in the follow-up of these patients. The creation of associations of patients and families of patients with hereditary angioedema has played a very important role in the care of these patients in Brazil.

Keywords

Hereditary angioedema, therapeutics, emergency treatment, quality of life, biological therapy.

Referências

1. Maurer M, Aygören-Pürsün E, Banerji A, Bernstein JA, Balle Boysen H, Busse PJ, et al. Consensus on Treatment Goals in Hereditary Angioedema: A Global Delphi Initiative. J Allergy Clin Immunol. 2021;148(6):1526-32. doi: 10.1016/j.jaci.2021.05.016.

2. Busse PJ, Christiansen SC, Riedl MA, Banerji A, Bernstein JA, Castaldo AJ, et al. US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema. J Allergy Clin Immunol Pract. 2021;9(1):132-50.e3. doi: 10.1016/j. jaip.2020.08.046.

3. Maurer M, Magerl M, Betschel S, Aberer W, Ansotegui IJ, AygörenPürsün E, et al. The International WAO/EAACI Guideline for the Management of Hereditary Angioedema-The 2021 Revision and Update. Allergy. 2022. doi: 10.1111/all.15214.

4. Caballero T. Treatment of Hereditary Angioedema. J Investig Allergol Clin Immunol. 2021;31(1):1-16. doi: 10.18176/jiaci.0653.

5. Riedl MA. Creating a Comprehensive Treatment Plan for Hereditary Angioedema. Immunol Allergy Clin North Am. 2013;33(4):471-85. doi: 10.1016/j.iac.2013.07.003.

6. Settipane RA, Bukstein DA, Riedl MA. Hereditary Angioedema and Shared Decision Making. Allergy Asthma Proc. 2020;41(Suppl 1):S55-S60. doi: 10.2500/aap.2020.41.200057.

7. Campos R de A, Valle SOR, Toledo EC. Hereditary Angioedema: A Disease Seldom Diagnosed by Pediatricians. J Pediatr (Rio J) 2021;97 Suppl 1:S10-S16. doi: 10.1016/j.jped.2020.10.011.

8. Farkas H, Martinez-Saguer I, Bork K, Bowen T, Craig T, Frank M, et al. International Consensus on the Diagnosis and Management of Pediatric Patients with Hereditary Angioedema with C1 Inhibitor Deficiency. Allergy. 2017;72(2):300-13. doi: 10.1111/all.13001.

9. Pancholy N, Craig T. Hereditary Angioedema in Children: A Review and Update. Curr Opin Pediatr. 2019;31(6):863-8. doi: 10.1097/ MOP.0000000000000832.

10. Craig T, Aygören-Pürsün E, Bork K, Bowen T, Boysen H, Farkas H, et al. WAO Guideline for the Management of Hereditary Angioedema. World Allergy Organ J. 2012;5(12):182-9. doi: 10.1097/ WOX.0b013e318279affa.

11. Dagen C, Craig TJ. Treatment of Hereditary Angioedema: Items that need to be addressed in practice parameter. Allergy Asthma Clin Immunol. 2010;6(1):11. doi: 10.1186/1710-1492-6-11.

12. Valle SOR, França AT, Campos RA, Grumach AS. Angioedema Hereditário. Rev bras alerg imunopatol. 2010;33(3):80-7.

13. Paige D, Maina N, Anderson JT. Hereditary Angioedema: Comprehensive Management Plans and Patient Support. Allergy Asthma Proc. 2020;41(Suppl 1):S38-S42. doi: 10.2500/ aap.2020.41.200059.

14. Banerji A, Anderson J, Johnston DT. Optimal Management of Hereditary Angioedema: Shared Decision-Making. J Asthma Allergy. 2021;14:119-25. doi: 10.2147/JAA.S284029.

15. Betschel S, Badiou J, Binkley K, Borici-Mazi R, Hébert J, Kanani A, et al. The International/Canadian Hereditary Angioedema Guideline. Allergy Asthma Clin Immunol. 2019;15(1):72. doi: 10.1186/s13223- 019-0376-8.

16. Caballero T, Baeza ML, Cabañas R, Campos A, Cimbollek S, Gómez-Traseira C, 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(5):333-47.

17. Brasil. Ministério da Saúde. Manual dos Centros de Referência para Imunobiológicos Especiais. 5ª ed., 2019.

18. Serpa FS, Mansour E, Aun MV, Giavina-Bianchi P, Chong HJ, Arruda LK, et al. Hereditary Angioedema: How to Approach It at the Emergency Department? Einstein (São Paulo). 2021;19:eRW5498. doi: 10.31744/einstein_journal/2021RW5498.

19. Fijen LM, Levi M, Cohn DM. COVID-19 Vaccination and the Risk of Swellings in Patients with Hereditary Angioedema. J Allergy Clin Immunol Pract. 2021;9(11):4156-8. doi: 10.1016/j. jaip.2021.08.039.

20. Valle SOR, Alonso MLO, Tortora RP, Abe AT, Levy SAP, Dortas SD. Hereditary Angioedema: Screening of First-Degree Blood Relatives and Earlier Diagnosis. Allergy Asthma Proc. 2019;40(4):279-81. doi: 10.2500/aap.2019.40.4213.

21. Giavina-Bianchi P, Arruda LK, Aun MV, Campos RA, Chong-Neto HJ, Constantino-Silva RN, et al. Diretrizes brasileiras para o diagnóstico e tratamento do angioedema hereditário - 2017. Arq Asma Alerg Imunol. 2017;1(1):23-48. doi: 10.5935/2526-5393.20170005.

22. Bork K, Anderson JT, Caballero T, Craig T, Johnston DT, Li HH, et al. Assessment and Management of Disease Burden and Quality of Life in Patients with Hereditary Angioedema: A Consensus Report. Allergy Asthma Clin Immunol. 2021;17(1):40. doi: 10.1186/s13223- 021-00537-2.

23. Cicardi M, Castelli R, Zingale LC, Agostoni A. Side Effects of Long-Term Prophylaxis with Attenuated Androgens in Hereditary Angioedema: Comparison of Treated and Untreated Patients. J Allergy Clin Immunol. 1997;99(2):194-6. doi: 10.1016/s0091- 6749(97)70095-2.

24. Füst G, Farkas H, Csuka D, Varga L, Bork K. Long-Term Efficacy of Danazol Treatment in Hereditary Angioedema. Eur J Clin Invest. 2011;41(3):256-62. doi: 10.1111/j.1365-2362.2010.02402.x.

25. Riedl MA. Critical Appraisal of Androgen Use in Hereditary Angioedema: A Systematic Review. Ann Allergy Asthma Immunol. 2015;114(4):281-288.e7. doi: 10.1016/j.anai.2015.01.003.

26. Cicardi M, Bork K, Caballero T, Craig T, Li HH, Longhurst H, 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(2):147-57. doi: 10.1111/j.1398- 9995.2011.02751.x.

27. Sheffer AL, Austen KF, Rosen FS. Tranexamic Acid Therapy in Hereditary Angioneurotic Edema. N Engl J Med. 1972;287(9):452-4. doi: 10.1056/NEJM197208312870907.

28. Wintenberger C, Boccon-Gibod I, Launay D, Fain O, Kanny G, Jeandel PY, et al. Tranexamic Acid as Maintenance Treatment for Non-Histaminergic Angioedema: Analysis of Efficacy and Safety in 37 Patients. Clin Exp Immunol. 2014;178(1):112-7. doi: 10.1111/ cei.12379.

29. Tengborn L, Blombäck M, Berntorp E. Tranexamic Acid – an Old Drug Still Going Strong and Making a Revival. Thromb Res. 2015;135(2):231-42. doi: 10.1016/j.thromres.2014.11.012.

30. Zuraw BL, Busse PJ, White M, Jacobs J, Lumry W, Baker J, et al. Nanofiltered C1 Inhibitor Concentrate for Treatment of Hereditary Angioedema. N Engl J Med. 2010;363(6):513-22. doi: 10.1056/ NEJMoa0805538.

31. Terpstra FG, Kleijn M, Koenderman AHL, Over J, van Engelenburg FAC, Schuitemaker H, et al. Viral Safety of C1-Inhibitor NF. Biologicals. 2007;35(3):173-81. doi: 10.1016/j.biologicals.2006.08.005.

32. Maurer M, Magerl M, Ansotegui I, Aygören-Pürsün E, Betschel S, Bork K, et al. The International WAO/EAACI Guideline for the Management of Hereditary Angioedema-The 2017 Revision and Update. Allergy. 2018;73(8):1575-1596. doi: 10.1111/all.13384.

33. Bork K, Hardt J. Hereditary Angioedema: Long-Term Treatment with One or More Injections of C1 Inhibitor Concentrate per Week. Int Arch Allergy Immunol. 2011;154(1):81-8. doi: 10.1159/000319213.

34. Waytes AT, Rosen FS, Frank MM. Treatment of Hereditary Angioedema with a Vapor-Heated C1 Inhibitor Concentrate. N Engl J Med. 1996;334(25):1630-4. doi: 10.1056/NEJM199606203342503.

35. Tallroth GA. Long-Term Prophylaxis of Hereditary Angioedema with a Pasteurized C1 Inhibitor Concentrate. Int Arch Allergy Immunol. 2011;154(4):356-9. doi: 10.1159/000321830.

36. Kreuz W, Martinez-Saguer I, Aygören-Pürsün E, Rusicke E, Heller C, Klingebiel T. C1-Inhibitor Concentrate for Individual Replacement Therapy in Patients with Severe Hereditary Angioedema Refractory to Danazol Prophylaxis. Transfusion. 2009;49(9):1987-95. doi: 10.1111/j.1537-2995.2009.02230.x.

37. Frank MM. Hereditary Angiodema: A Current State-of-the-Art Review, VI: Novel Therapies for Hereditary Angioedema. Ann Allergy Asthma Immunol 2008;100(1 Suppl 2):S23-29. doi: 10.1016/s1081- 1206(10)60583-2.

38. Bernstein JA, Ritchie B, Levy RJ, Wasserman RL, Bewtra AK, Hurewitz DS, et al. Population Pharmacokinetics of PlasmaDerived C1 Esterase Inhibitor Concentrate Used to Treat Acute Hereditary Angioedema Attacks. Ann Allergy Asthma Immunol. 2010;105(2):149-54. doi: 10.1016/j.anai.2010.06.005.

39. Zuraw BL, Cicardi M, Longhurst HJ, Bernstein JA, Li HH, Magerl M, et al. Phase II Study Results of a Replacement Therapy for Hereditary Angioedema with Subcutaneous C1-Inhibitor Concentrate. Allergy. 2015;70(10):1319-28. doi: 10.1111/all.12658.

40. Reshef A, Moldovan D, Obtulowicz K, Leibovich I, Mihaly E, Visscher S, et al. Recombinant Human C1 Inhibitor for the Prophylaxis of Hereditary Angioedema Attacks: A Pilot Study. Allergy. 2013;68(1):118-24. doi: 10.1111/all.12060.

41. Farkas H, Varga L. Human Plasma-Derived, Nanofiltered, C1- Inhibitor Concentrate (Cinryze®), a Novel Therapeutic Alternative for the Management of Hereditary Angioedema Resulting from C1-Inhibitor Deficiency. Biol Ther. 2012;2:2. doi: 10.1007/s13554- 012-0002-5.

42. Craig TJ, Bewtra AK, Bahna SL, Hurewitz D, Schneider LC, Levy RJ, et al. C1 Esterase Inhibitor Concentrate in 1085 Hereditary Angioedema Attacks - Final Results of the I.M.P.A.C.T.2 Study. Allergy. 2011;66(12):1604-1611. doi: 10.1111/j.1398-9995.2011.02702.x.

43. Gandhi PK, Gentry WM, Bottorff MB. Thrombotic Events Associated with C1 Esterase Inhibitor Products in Patients with Hereditary Angioedema: Investigation from the United States Food and Drug Administration Adverse Event Reporting System Database. Pharmacotherapy. 2012;32(10):902-9. doi: 10.1002/j.1875- 9114.2012.01126.

44. Farkas H, Kőhalmi KV, Veszeli N, Zotter Z, Várnai K, Varga L. Risk of Thromboembolism in Patients with Hereditary Angioedema Treated with Plasma-Derived C1-Inhibitor. Allergy Asthma Proc. 2016;37(2):164-70. doi: 10.2500/aap.2016.37.3933.

45. Crowther M, Bauer KA, Kaplan AP. The Thrombogenicity of C1 Esterase Inhibitor (Human): Review of the Evidence. Allergy Asthma Proc. 2014;35(6):444-53. doi: 10.2500/aap.2014.35.3799.

46. Longhurst H, Cicardi M, Craig T, Bork K, Grattan C, Baker J, et al. Prevention of Hereditary Angioedema Attacks with a Subcutaneous C1 Inhibitor. N Engl J Med. 2017;376(12):1131-40. doi: 10.1056/ NEJMoa1613627.

47. Brasill. Ministério Da Saúde. Agência Nacional de Vigilância Sanitária (ANVISA). Bulário Eletrônico. Berinert: Pó Liofilizado Para Solução Injetável. Bula Profissional do Medicamento [Internet]. Brasília (DF): ANVISA; 2021. Disponível em: https://Consultas.Anvisa.Gov.Br/#/Bulario/q/?NomeProduto=berinert. 2021.

48. Craig T, Zuraw B, Longhurst H, Cicardi M, Bork K, Grattan C, et al. Long-Term Outcomes with Subcutaneous C1-Inhibitor Replacement Therapy for Prevention of Hereditary Angioedema Attacks. J Allergy Clin Immunol Pract. 2019;7(6):1793-1802.e2. doi: 10.1016/j. jaip.2019.01.054.

49. Zuraw B, Cicardi M, Levy RJ, Nuijens JH, Relan A, Visscher S, et al. Recombinant Human C1-Inhibitor for the Treatment of Acute Angioedema Attacks in Patients with Hereditary Angioedema. J Allergy Clin Immunol. 2010;126(4):821-7.e14. doi: 10.1016/j. jaci.2010.07.021.

50. Riedl MA, Bernstein JA, Li H, Reshef A, Lumry W, Moldovan D, et al. Recombinant Human C1-Esterase Inhibitor Relieves Symptoms of Hereditary Angioedema Attacks: Phase 3, Randomized, PlaceboControlled Trial. Ann Allergy Asthma Immunol. 2014;112(2):163-9. e1. doi: 10.1016/j.anai.2013.12.004.

51. Farrell C, Hayes S, Relan A, van Amersfoort ES, Pijpstra R, Hack CE. Population Pharmacokinetics of Recombinant Human C1 Inhibitor in Patients with Hereditary Angioedema. Br J Clin Pharmacol. 2013;76(6):897-907. doi: 10.1111/bcp.12132.

52. Hemperly SE, Agarwal NS, Xu Y-Y, Zhi Y-X, Craig TJ. Recent Advances in the Management of Hereditary Angioedema. J Am Osteopath Assoc. 2013;113(7):546-55. doi: 10.7556/jaoa.2013.006.

53. Banerji A, Riedl MA, Bernstein JA, Cicardi M, Longhurst HJ, Zuraw BL, et al. Effect of Lanadelumab Compared With Placebo on Prevention of Hereditary Angioedema Attacks: A Randomized Clinical Trial. JAMA. 2018;320(20):2108-21. doi: 10.1001/jama.2018.16773.

54. Riedl MA, Maurer M, Bernstein JA, Banerji A, Longhurst HJ, Li HH, et al. Lanadelumab Demonstrates Rapid and Sustained Prevention of Hereditary Angioedema Attacks. Allergy 2020;75(11):2879-2887. doi: 10.1111/all.14416.

55. Brasil. Ministério da Saúde. Agência Nacional de Vigilância Sanitária (ANVISA). Bulário Eletrônico. Takhzyro: Solução Injetável. Bula Profissional do Medicamento [Internet]. Brasília (DF): ANVISA; 2021. Disponível em: https://Consultas.Anvisa.Gov.Br/#/Bulario/q/?NomeProduto=TAKHZYRO. 2021.

56. Buttgereit T, Vera C, Weller K, Gutsche A, Grekowitz EM, Aykanat S, et al. Lanadelumab Efficacy, Safety, and Injection Interval Extension in HAE: A Real-Life Study. J Allergy Clin Immunol Pract. 2021;9(10):3744-51. doi: 10.1016/j.jaip.2021.04.072.

57. Garcia JFB, Takejima P, Veronez CL, Aun MV, Motta AA, Kalil J, et al. Use of PdC1-INH Concentrate for Long-Term Prophylaxis during Pregnancy in Hereditary Angioedema with Normal C1-INH. J Allergy Clin Immunol Pract. 2018;6(4):1406-8. doi: 10.1016/j. jaip.2017.12.022.

58. Bork K. Diagnosis and Treatment of Hereditary Angioedema with Normal C1 Inhibitor. Allergy Asthma Clin Immunol. 2010;6(1):15. doi: 10.1186/1710-1492-6-15.

59. Bork K, Wulff K, Hardt J, Witzke G, Staubach P. Hereditary Angioedema Caused by Missense Mutations in the Factor XII Gene: Clinical Features, Trigger Factors, and Therapy. J Allergy Clin Immunol. 2009;124(1):129-34. doi: 10.1016/j.jaci.2009.03.038.

60. Saule C, Boccon-Gibod I, Fain O, Kanny G, Plu-Bureau G, Martin L, et al. Benefits of Progestin Contraception in Non-Allergic Angioedema. Clin Exp Allergy. 2013;43(4):475-82. doi: 10.1111/cea.12055.

61. Bork K, Wulff K, Witzke G, Hardt J. Treatment for Hereditary Angioedema with Normal C1-INH and Specific Mutations in the F12 Gene (HAE-FXII). Allergy. 2017;72(2):320-4. doi: 10.1111/ all.13076.

62. Bernstein JA. Managing Hereditary Angioedema Patients Undergoing Otolaryngeal Procedures. Am J Rhinol Allergy. 2013;27(6):522-7. doi: 10.2500/ajra.2013.27.3964.

63. Aygören-Pürsün E, Martinez Saguer I, Kreuz W, Klingebiel T, Schwabe D. Risk of Angioedema Following Invasive or Surgical Procedures in HAE Type I and II - the Natural History. Allergy. 2013;68(8):1034-9. doi: 10.1111/all.12186.

64. Jurado-Palomo J, Muñoz-Caro JM, López-Serrano MC, Prior N, Cabañas R, Pedrosa M, 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):1-6.

65. 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(1):58-64. doi: 10.1016/j. tripleo.2011.02.034.

66. Zanichelli A, Ghezzi M, Santicchia I, Vacchini R, Cicardi M, Sparaco A, et al. Short-Term Prophylaxis in Patients with Angioedema Due to C1-Inhibitor Deficiency Undergoing Dental Procedures: An Observational Study. PLoS One. 2020;15(3):e0230128. doi: 10.1371/journal.pone.0230128.

67. Bork K. Pasteurized and Nanofiltered, Plasma-Derived C1 Esterase Inhibitor Concentrate for the Treatment of Hereditary Angioedema. Immunotherapy. 2014;6(5):533-51. doi: 10.2217/imt.14.33.

68. Magerl M, Frank M, Lumry W, Bernstein J, Busse P, Craig T, 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):110-2. doi: 10.1016/j.anai.2016.10.006.

69. Navarro Ruiz A, Crespo Diz C, Poveda Andrés JL, Cebollero de Torre A. Algoritmo de diagnóstico y tratamiento del angioedema hereditario como herramienta para su. Farmacia Hospitalaria. 2013;(6):521-9. doi: 10.7399/FH.2013.37.6.980.

70. Galan HL, Reedy MB, Starr J, Knight AB. Fresh Frozen Plasma Prophylaxis for Hereditary Angioedema during Pregnancy. A Case Report. J Reprod Med. 1996;41(7):541-4.

71. Bhardwaj N, Craig TJ. Treatment of Hereditary Angioedema: A Review (CME). Transfusion. 2014;54(11):2989-96. doi: 10.1111/ trf.12674.

72. Lumry WR. Management and Prevention of Hereditary Angioedema Attacks. Am J Manag Care. 2013;19(7 Suppl):s111-8.

73. Farkas H, Gyeney L, Gidófalvy E, Füst G, Varga L. The Efficacy of Short-Term Danazol Prophylaxis in Hereditary Angioedema Patients Undergoing Maxillofacial and Dental Procedures. J Oral Maxillofac Surg. 1999;57(4):404-8. doi: 10.1016/s0278-2391(99)90280-x.

74. Vázquez DO, Josviak DO, Fantini CA, Fili NL, Berardi AM, Zwiener RD, et al. Consenso argentino de diagnóstico y tratamiento del angioedema hereditario. Revista Alergia México. 2021;68(Suplemento 2):s1-s22. doi: 10.29262/ram.v68i6.914.

75. Longhurst HJ, Bork K. Hereditary Angioedema: An Update on Causes, Manifestations and Treatment. Br J Hosp Med. 2019;80(7):391-8. doi: 10.12968/hmed.2019.80.7.391.

76. Banerji A, Busse P, Christiansen SC, Li H, Lumry W, Davis-Lorton M, et al. Current State of Hereditary Angioedema Management: A Patient Survey. Allergy Asthma Proc 2015;36(3):213-217. doi: 10.2500/aap.2015.36.3824.

77. Christiansen SC, Bygum A, Banerji A, Busse P, Li H, Lumry W, et al. Before and after, the Impact of Available on-Demand Treatment for HAE. Allergy Asthma Proc. 2015;36(2):145-50. doi: 10.2500/ aap.2015.36.3831.

78. Moellman JJ, Bernstein JA, Lindsell C, Banerji A, Busse PJ, Camargo CA, et al. A Consensus Parameter for the Evaluation and Management of Angioedema in the Emergency Department. Acad Emerg Med. 2014;21(4):469-84. doi: 10.1111/acem.12341.

79. Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, Course, and Complications of Abdominal Attacks in Hereditary Angioedema Due to C1 Inhibitor Deficiency. Am J Gastroenterol. 2006;101(3):619-7. doi: 10.1111/j.1572-0241.2006.00492.x.

80. Cicardi M, Bellis P, Bertazzoni G, Cancian M, Chiesa M, Cremonesi P, et al. Guidance for Diagnosis and Treatment of Acute Angioedema in the Emergency Department: Consensus Statement by a Panel of Italian Experts. Intern Emerg Med. 2014;9(1):85-92. doi: 10.1007/ s11739-013-0993-z.

81. Brasil. Ministério Da Saúde. Agência Nacional de Vigilância Sanitária (ANVISA). Bulário Eletrônico. Cinryze: Pó Liofilizado. Bula Profissional do Medicamento [Internet]. Brasília (DF): ANVISA; 2022. Disponível em: https://consultas.anvisa.gov.br/#/bulario/q/?nomeProduto=Cinryze . 2022.

82. Brasil. Ministério da Saúde. Agência Nacional de Vigilância Sanitária (ANVISA). Bulário Eletrônico. Firazyr: Solução Injetável. Bula Profissional do Medicamento [Internet]. Brasília (DF): ANVISA; 2019 [citado 2022 Mar 29]. Disponível em: http://www.Anvisa.Gov.Br/Datavisa/Fila_bula/Index.Asp. 2019.

83. Bork K, Bernstein JA, Machnig T, Craig TJ. Efficacy of Different Medical Therapies for the Treatment of Acute Laryngeal Attacks of Hereditary Angioedema Due to C1-Esterase Inhibitor Deficiency. J Emerg Med. 2016;50(4):567-80.e1. doi: 10.1016/j. jemermed.2015.11.008.

84. Valerieva A, Staevska MT, Grivcheva-Panovska V, Jesenak M, Kőhalmi KV, Hrubiskova K, et al. Recombinant Human C1 Esterase Inhibitor for Hereditary Angioedema Attacks: A European Registry. World Allergy Organ J. 2021;14(4):100535. doi: 10.1016/j. waojou.2021.100535.

85. Cicardi M, Banerji A, Bracho F, Malbrán A, Rosenkranz B, Riedl M, et al. Icatibant, a New Bradykinin-Receptor Antagonist, in Hereditary Angioedema. N Engl J Med. 2010;363(6):532-41. doi: 10.1056/ NEJMoa0906393.

86. Lumry WR, Li HH, Levy RJ, Potter PC, Farkas H, Moldovan D, et al. Randomized Placebo-Controlled Trial of the Bradykinin B Receptor Antagonist Icatibant for the Treatment of Acute Attacks of Hereditary Angioedema: The FAST-3 Trial. Ann Allergy Asthma Immunol. 2011;107(6):529-37. doi: 10.1016/j.anai.2011.08.015.

87. Maurer M, Aberer W, Bouillet L, Caballero T, Fabien V, Kanny G, et al. Hereditary Angioedema Attacks Resolve Faster and Are Shorter after Early Icatibant Treatment. PLoS One. 2013;8(2):e53773. doi: 10.1371/journal.pone.0053773.

88. Aberer W, Maurer M, Reshef A, Longhurst H, Kivity S, Bygum A, et al. Open-Label, Multicenter Study of Self-Administered Icatibant for Attacks of Hereditary Angioedema. Allergy. 2014;69(3):305-14. doi: 10.1111/all.12303.

89. Cicardi M, Levy RJ, McNeil DL, Li HH, Sheffer AL, Campion M, et al. Ecallantide for the Treatment of Acute Attacks in Hereditary Angioedema. N Engl J Med. 2010;363(6):523-31. doi: 10.1056/ NEJMoa0905079.

90. Jindal AK, Reshef A, Longhurst H; GEHM workgroup (Global Equity in HAE Management). Mitigating Disparity in Health-Care Resources Between Countries for Management of Hereditary Angioedema. Clin Rev Allergy Immunol. 2021;61(1):84-97. doi: 10.1007/s12016-021- 08854-5.

91. Christiansen SC, Davis DK, Castaldo AJ, Zuraw BL. Pediatric Hereditary Angioedema: Onset, Diagnostic Delay, and Disease Severity. Clin Pediatr (Phila). 2016;55(10):935-42. doi: 10.1177/0009922815616886.

92. Araújo-Simões J, Boanova AGP, Constantino-Silva RN, Fragnan NTML, Pinto JA, Minafra FG, et al. The Challenges in the Follow-Up and Treatment of Brazilian Children with Hereditary Angioedema. Int Arch Allergy Immunol. 2021;182(7):585-91. doi: 10.1159/000512944.

93. Bork K, Gül D, Hardt J, Dewald G. Hereditary Angioedema with Normal C1 Inhibitor: Clinical Symptoms and Course. Am J Med. 2007;120(11):987-92. doi: 10.1016/j.amjmed.2007.08.021.

94. Johnston DT, Smith RC. Hereditary Angioedema: Special Considerations in Children. Allergy Asthma Proc. 2020;41(Suppl 1):S43-S46. doi: 10.2500/aap.2020.41.200042.

95. Ocak M, Nain E, ahiner ÜM, Akin M , Karabiber E, ekerel BE, et al. Recurrent Angioedema in Childhood: Hereditary Angioedema or Histaminergic Angioedema? Pediatr Dermatol. 2021;38(1):143-8. doi: 10.1111/pde.14467.

96. Farkas H, Kőhalmi KV, Visy B, Veszeli N, Varga L. Clinical Characteristics and Safety of Plasma-Derived C1-Inhibitor Therapy in Children and Adolescents with Hereditary Angioedema-A LongTerm Survey. J Allergy Clin Immunol Pract. 2020;8(7):2379-83. doi: 10.1016/j.jaip.2020.02.043.

97. Aygören-Pürsün E, Soteres D, Moldovan D, Christensen J, Van Leerberghe A, Hao J, et al. Preventing Hereditary Angioedema Attacks in Children Using Cinryze®: Interim Efficacy and Safety Phase 3 Findings. Int Arch Allergy Immunol. 2017;173(2):114-9. doi: 10.1159/000477541.

98. Farkas H, Reshef A, Aberer W, Caballero T, McCarthy L, Hao J, et al. Treatment Effect and Safety of Icatibant in Pediatric Patients with Hereditary Angioedema. J Allergy Clin Immunol Pract. 2017;5(6):1671-8.e2. doi: 10.1016/j.jaip.2017.04.010.

99. Fijen LM, Bork K, Cohn DM. Current and Prospective Targets of Pharmacologic Treatment of Hereditary Angioedema Types 1 and 2. Clin Rev Allergy Immunol. 2021;61(1):66-76. doi: 10.1007/ s12016-021-08832-x.

100. Cancian M, Perego F, Senter R, Arcoleo F, De Pasquale T, Zoli A, et al. Pediatric Angioedema: Essential Features and Preliminary Results from the Hereditary Angioedema Global Registry in Italy. Pediatr Allergy Immunol. 2020;31 Suppl 24:22-24. doi: 10.1111/ pai.13170.

101. Wahn V, Aberer W, Aygören-Pürsün E, Bork K, Eberl W, Faßhauer M, et al. Hereditary Angioedema in Children and Adolescents - A Consensus Update on Therapeutic Strategies for GermanSpeaking Countries. Pediatr Allergy Immunol. 2020;31(8):974-89. doi: 10.1111/pai.13309.

102. Gompel A, Fain O, Boccon-Gibod I, Gobert D, Bouillet L. Exogenous Hormones and Hereditary Angioedema. International Immunopharmacology. 2020;78:106080. doi: 10.1016/j. intimp.2019.106080.

103. Caballero T, Farkas H, Bouillet L, Bowen T, Gompel A, Fagerberg C, 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(2):308-20. doi: 10.1016/j. jaci.2011.11.025.

104. Zuraw BL, Bork K, Binkley KE, Banerji A, Christiansen SC, Castaldo A, et al. Hereditary Angioedema with Normal C1 Inhibitor Function: Consensus of an International Expert Panel. Allergy Asthma Proc. 2012;33 Suppl 1:S145-56. doi: 10.2500/aap.2012.33.3627.

105. Martinez-Saguer I, Rusicke E, Aygören-Pürsün E, Heller C, Klingebiel T, Kreuz W. Characterization of Acute Hereditary Angioedema Attacks during Pregnancy and Breast-Feeding and Their Treatment with C1 Inhibitor Concentrate. Am J Obstet Gynecol. 2010;203(2):131.e1-7. doi: 10.1016/j.ajog.2010.03.003.

106. Bouillet L, Longhurst H, Boccon-Gibod I, Bork K, Bucher C, Bygum A, et al. Disease Expression in Women with Hereditary Angioedema. Am J Obstet Gynecol. 2008;199(5):484.e1-4. doi: 10.1016/j.ajog.2008.04.034.

107. Czaller I, Visy B, Csuka D, Füst G, Tóth F, Farkas H. The Natural History of Hereditary Angioedema and the Impact of Treatment with Human C1-Inhibitor Concentrate during Pregnancy: A LongTerm Survey. Eur J Obstet Gynecol Reprod Biol. 2010;152(1):44-9. doi: 10.1016/j.ejogrb.2010.05.008.

108. Debreczeni ML, Németh Z, Kajdácsi E, Farkas H, Cervenak L. Molecular Dambusters: What Is Behind Hyperpermeability in Bradykinin-Mediated Angioedema? Clin Rev Allergy Immunol. 2021;60(3):318-47. doi: 10.1007/s12016-021-08851-8.

109. Bork K, Wulff K, Witzke G, Hardt J. Hereditary Angioedema with Normal C1-INH with versus without Specific F12 Gene Mutations. Allergy. 2015;70(8):1004-12. doi: 10.1111/all.12648.

110. Feray S, Fain O, Kayem G, Sabourdin N, Constant I, Rigouzzo A. Repeated Attacks of Type III Hereditary Angioedema with Factor XII Mutation during Pregnancy. Int J Obstet Anesth. 2018;36:114-8. doi: 10.1016/j.ijoa.2018.07.003.

111. Veronez CL, Moreno AS, Constantino-Silva RN, Maia LSM, Ferriani MPL, Castro FFM, et al. Hereditary Angioedema with Normal C1 Inhibitor and F12 Mutations in 42 Brazilian Families. The Journal of Allergy and Clinical Immunology: In Practice. 2018;6(4):1209- 16.e8. doi: 10.1016/j.jaip.2017.09.025.

112. Machado AM, Pires RM, Martins RO, Grumach AS. Pregnancy and Postpartum in Hereditary Angioedema With C1 Inhibitor Deficit in Women Who Have No Access to Therapy. J Investig Allergol Clin Immunol. 2017;27(5):322-3. doi: 10.18176/jiaci.0175.

113. González-Quevedo T, Larco JI, Marcos C, Guilarte M, Baeza ML, Cimbollek S, et al. Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency. J Investig Allergol Clin Immunol. 2016;26(3):161-7. doi: 10.18176/jiaci.0037.

114. Yakaboski E, Motazedi T, Banerji A. Hereditary Angioedema: Special Considerations in Women. Allergy Asthma Proc. 2020;41(Suppl 1):S47-S50. doi: 10.2500/aap.2020.41.200077.

115. Moldovan D, Bernstein JA, Hakl R, Porebski G, Poarch K, Lumry WR, et al. Safety of Recombinant Human C1 Esterase Inhibitor for Hereditary Angioedema Attacks during Pregnancy. J Allergy Clin Immunol Pract. 2019;7(8):2938-40. doi: 10.1016/j. jaip.2019.05.042.

116. Hakl R, Kuklínek P, Kr mová I, Králí ková P, Freiberger T, Jank P, et al. Treatment of Hereditary Angioedema Attacks with Icatibant and Recombinant C1 Inhibitor During Pregnancy. J Clin Immunol. 2018;38(7):810-5. doi: 10.1007/s10875-018-0553-4.

117. Kaminsky LW, Kelbel T, Ansary F, Craig T. Multiple Doses of Icatibant Used during Pregnancy. Allergy Rhinol (Providence). 2017;8(3):178-81. doi: 10.2500/ar.2017.8.0210.

118. Brooks JP, Radojicic C, Riedl MA, Newcomer SD, Banerji A, Hsu FI. Experience with Intravenous Plasma-Derived C1-Inhibitor in Pregnant Women with Hereditary Angioedema: A Systematic Literature Review. J Allergy Clin Immunol Pract. 2020;8(6):1875- 80.e3. doi: 10.1016/j.jaip.2020.03.009.

119. Gibbons KR, Abraham T, Sandhu M, Peppers BP, Girzhel JF, Hostoffer RW. Successful Perinatal Management of Hereditary Angioedema with Normal C1 Esterase Inhibitor and Factor XII Mutation Using C1 Esterase Inhibitor Therapy. Ann Allergy Asthma Immunol. 2017;119(6):558-9. doi: 10.1016/j.anai.2017.08.015.

120. Levy DS, Farkas H, Riedl MA, Hsu FI, Brooks JP, Cicardi M, et al. Long-Term Efficacy and Safety of Subcutaneous C1-Inhibitor in Women with Hereditary Angioedema: Subgroup Analysis from an Open-Label Extension of a Phase 3 Trial. Allergy Asthma Clin Immunol. 2020;16:8. doi: 10.1186/s13223-020-0409-3.

121. Andarawewa S, Aygören-Pürsün E. Subcutaneous C1-Inhibitor Concentrate for Prophylaxis during Pregnancy and Lactation in a Patient with C1-INH-HAE. Clin Case Rep. 2021;9(3):1273-5. doi: 10.1002/ccr3.3743.

122. Gompels MM, Lock RJ, Abinun M, Bethune CA, Davies G, Grattan C, et al. C1 Inhibitor Deficiency: Consensus Document. Clin Exp Immunol. 2005;139(3):379-94. doi: 10.1111/j.1365- 2249.2005.02726.x.

123. Moraes CG de FB de, Mikami LR, Ferrari LP, Pesquero JB, ChongNeto HJ, Rosario Filho NA. Short-Term Prophylaxis for Delivery in Pregnant Women with Hereditary Angioedema with Normal C1-Inhibitor. Rev Bras Ginecol Obstet. 2020;42(12):845-8. doi: 10.1055/s-0040-1718955.

124. Chinniah N, Katelaris CH. Hereditary Angioedema and Pregnancy. Aust N Z J Obstet Gynaecol. 2009;49(1):2-5. doi: 10.1111/j.1479- 828X.2008.00945.x.

125. Bhardwaj NR, Espey E. Lactation and Contraception. Curr Opin Obstet Gynecol. 2015;27(6):496-503. doi: 10.1097/ GCO.0000000000000216.

126. Pregnancy and Breastfeeding Warnings. Drugs.com [site na Internet]. Disponível em: https://www.drugs.com/pregnancy/ .Acessado em: 03/04/2022.

127. Aygören-Pürsün E, Magerl M, Maetzel A, Maurer M. Epidemiology of Bradykinin-Mediated Angioedema: A Systematic Investigation of Epidemiological Studies. Orphanet J Rare Dis. 2018;13(1):73. doi: 10.1186/s13023-018-0815-5.

128. Farkas H, Stobiecki M, Peter J, Kinaciyan T, Maurer M, AygörenPürsün E, et al. Long-Term Safety and Effectiveness of Berotralstat for Hereditary Angioedema: The Open-Label APeX-S Study. Clin Transl Allergy. 2021;11(4):e12035. doi: 10.1002/clt2.12035.

129. Hofman ZLM, Clark CC, Hack CE, de Maat S, Maas C. Detecting Oral Kallikrein-Targeting Therapy through Triggered Contact Activation: A Phase I Study. J Allergy Clin Immunol. 2020;146(6):1446-9.e5. doi: 10.1016/j.jaci.2020.03.038.

130. ANZCTR - Australian New Zealand Clinical Trials Registry. Registration. n.d. [Internet]. Disponível em: https://www.anzctr.org.au/Trial/Registration/TrialReview. aspx?id=375857&isReview=true. Acessado em: 03/04/2022.

131. KalVista Pharmaceuticals, Inc. KVD824 for HAE. [Internet]. Disponível em: https://www.kalvista.com/products-pipeline/kvd824-hae. Acessado em: 03/04/2022.

132. A Phase II, Cross-over Clinical Trial Evaluating the Efficacy and Safety of KVD900 in the On-Demand Treatment of Angioedema Attacks in Adult Subjects With Hereditary Angioedema Type I or II - Tabular View - ClinicalTrials.Gov. [Internet]. Disponível em: https://clinicaltrials.gov/ct2/show/record/NCT04208412. Acessado em: 03/04/2022.

133. Duckworth EJ, Murugesan N, Li L, Rushbrooke LJ, Lee DK, De Donatis GM, et al. Pharmacological Suppression of the Kallikrein Kinin System with KVD900: An Orally Available Plasma Kallikrein Inhibitor for the on-Demand Treatment of Hereditary Angioedema. Clin Exp Allergy. 2022. doi: 10.1111/cea.14122.

134. Maetzel A, Smith MD, Duckworth EJ, Hampton SL, De Donatis GM, Murugesan N, et al. KVD900, an Oral on-Demand Treatment for Hereditary Angioedema: Phase 1 Study Results. J Allergy Clin Immunol. 2022;S0091-6749(21)02666-X. doi: 10.1016/j. jaci.2021.10.038.

135. Cohn DM, Viney NJ, Fijen LM, Schneider E, Alexander VJ, Xia S, et al. Antisense Inhibition of Prekallikrein to Control Hereditary Angioedema. N Engl J Med. 2020;383(13):1242-7. doi: 10.1056/ NEJMoa1915035.

136. Ferrone JD, Bhattacharjee G, Revenko AS, Zanardi TA, Warren MS, Derosier FJ, et al. IONIS-PKKRx a Novel Antisense Inhibitor of Prekallikrein and Bradykinin Production. Nucleic Acid Ther. 2019;29(2):82-91. doi: 10.1089/nat.2018.0754.

137. Fijen LM, Riedl MA, Bordone L, Bernstein JA, Raasch J, Tachdjian R, et al. Inhibition of Prekallikrein for Hereditary Angioedema. N Engl J Med. 2022;386(11):1026-33. doi: 10.1056/NEJMoa2109329.

138. Craig T, Magerl M, Levy DS, Reshef A, Lumry WR, MartinezSaguer I, et al. Prophylactic Use of an Anti-Activated Factor XII Monoclonal Antibody, Garadacimab, for Patients with C1-Esterase Inhibitor-Deficient Hereditary Angioedema: A Randomised, Double-Blind, Placebo-Controlled, Phase 2 Trial. The Lancet. 2022;399(10328):945-55. doi: 10.1016/S0140- 6736(21)02225-X.

139. Lesage A, Gibson C, Marceau F, Ambrosi H-D, Saupe J, Katzer W, et al. In Vitro Pharmacological Profile of a New Small Molecule Bradykinin B2 Receptor Antagonist. Front Pharmacol. 2020;11:916. doi: 10.3389/fphar.2020.00916.

140. ClinicalTrials.gov. [Internet]. Pharvaris Netherlands B.V. A Phase II, Double-Blind, Placebo-Controlled, Randomized, Dose-Ranging, Parallel Group Study to Evaluate the Safety and Efficacy of PHA022121 Administered Orally for Prophylaxis Against Angioedema Attacks in Patients With Hereditary Angioedema Due to C1-Inhibitor Deficiency (Type I or Type II). Clinical trial registration; 2022.

141. ClinicalTrials.gov. [Internet]. Dose-Ranging Study of Oral PHA022121 for Acute Treatment of Angioedema Attacks in Patients with Hereditary Angioedema - Tabular View. Disponível em: https://clinicaltrials.gov/ct2/show/record/NCT04618211. Acessado em: 03/04/2022.

142. Biomarin. BMN 331 for Hereditary Angioedema (HAE). [Internet]. Disponível em: https://www.biomarin.com/our-treatments/pipeline/bmn-331-for-hae/. Acessado em: 03/04/2022.

143. Qiu T, Chiuchiolo MJ, Whaley AS, Russo AR, Sondhi D, Kaminsky SM, et al. Gene Therapy for C1 Esterase Inhibitor Deficiency in a Murine Model of Hereditary Angioedema. Allergy. 2019;74(6):1081-9. doi: 10.1111/all.13582.

144. REGENXBIO Expands Pipeline Using NAV Vectors to Deliver Therapeutic Antibodies for the Treatment of Hereditary Angioedema and Neurodegenerative Diseases | REGENXBIO Inc. n.d. Disponível em: http://ir.regenxbio.com/news-releases/news-release-details/regenxbio-expands-pipeline-using-navvectors-deliver-therapeutic/. Acessado em: 03/04/2022.

145. Forget AL. A Modular CRISPR/Cas9 Genome Editing Platform for Durable Therapeutic Knockout and Targeted Gene Insertion Applications. 16th Annual Meeting of the Oligonucleotide Therapeutics Society. [Internet]. Disponível em: https://3o5c4w3neipl16yvhj3nfqam-wpengine.netdna-ssl.com/wp-content/uploads/Intellia-Therapeutics_Tony-Forget_OTS2020_final_092920.pdf . Acessado em: 03/04/2022.

146. Crochet J, Lepelley M, Yahiaoui N, Vermorel C, Bosson J-L, Pralong P, et al. Bradykinin Mechanism Is the Main Responsible for Death by Isolated Asphyxiating Angioedema in France. Clin Exp Allergy. 2019;49(2):252-4. doi: 10.1111/cea.13297.

147. Banerji A. The Burden of Illness in Patients with Hereditary Angioedema. Ann Allergy Asthma Immunol. 2013;111(5):329-36. doi: 10.1016/j.anai.2013.08.019.

148. Longhurst H, Bygum A. The Humanistic, Societal, and PharmacoEconomic Burden of Angioedema. Clin Rev Allergy Immunol. 2016;51(2):230-9. doi: 10.1007/s12016-016-8575-2.

149. Savarese L, Bova M, De Falco R, Guarino MD, De Luca Picione R, Petraroli A, et al. Emotional Processes and Stress in Children Affected by Hereditary Angioedema with C1-Inhibitor Deficiency: A Multicenter, Prospective Study. Orphanet J Rare Dis. 2018;13(1):115. doi: 10.1186/s13023-018-0871-x.

150. Savarese L, Bova M, Maiello A, Petraroli A, Mormile I, Cancian M, et al. Psychological Processes in the Experience of Hereditary Angioedema in Adult Patients: An Observational Study. Orphanet J Rare Dis. 2021;16(1):23. doi: 10.1186/s13023-020-01643-x.

151. Arce-Ayala YM, Diaz-Algorri Y, Craig T, Ramos-Romey C. Clinical Profile and Quality of Life of Puerto Ricans with Hereditary Angioedema. Allergy Asthma Proc. 2019;40(2):103-10. doi: 10.2500/aap.2019.40.4200.

152. Farkas H. Hereditary Angioedema: Examining the Landscape of Therapies and Preclinical Therapeutic Targets. Expert Opin Ther Targets. 2019;23(6):457-9. doi: 10.1080/14728222.2019.1608949.

153. Kuman Tunçel Ö, Gökmen NM, Demir E, Gülbahar O, Pırıldar. The Impact of Hereditary Angioedema on Quality of Life and Family Planning Decisions. Int J Psychiatry Med. 2019;54(6):377-94. doi: 10.1177/0091217419837068.

154. Liu S, Xu Y, Liu Y, Zhi Y. Hereditary Angioedema: A Chinese Perspective. Eur J Dermatol. 2019;29(1):14-20. doi: 10.1684/ ejd.2018.3487.

155. Germenis AE, Margaglione M, Pesquero JB, Farkas H, Cichon S, Csuka D, et al. International Consensus on the Use of Genetics in the Management of Hereditary Angioedema. The Journal of Allergy and Clinical Immunology: In Practice. 2020;8(3):901-11. doi: 10.1016/j.jaip.2019.10.004.

156. Caballero T, Prior N. Burden of Illness and Quality-of-Life Measures in Angioedema Conditions. Immunol Allergy Clin North Am. 2017;37(3):597-616. doi: 10.1016/j.iac.2017.04.005.

157. Sirgy MJ, Michalos AC, Ferriss AL, Easterlin RA, Patrick D, Pavot W. The Quality-of-Life (QOL) Research Movement: Past, Present, and Future. Social Indicators Research. 2006;76(3):343-466. doi: 10.1007/s11205-005-2877-8.

158. Sirgy MJ, Reilly NP, Wu J, Efraty D. A Work-Life Identity Model of Well-Being: Towards a Research Agenda Linking Quality-ofWork-Life (QWL) Programs with Quality of Life (QOL). Applied Research in Quality of Life. 2008;3(3):181-202. doi: 10.1007/ s11482-008-9054-6.

159. Prior N, Remor E, Pérez-Fernández E, Caminoa M, GómezTraseira C, Gayá F, et al. Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults: HAE-QoL. J Allergy Clin Immunol Pract. 2016;4(3):464-73.e4. doi: 10.1016/j. jaip.2015.12.010.

160. Nunes FL, Ferriani MPL, Moreno AS, Langer SS, Maia LSM, Ferraro MF, et al. Decreasing Attacks and Improving Quality of Life through a Systematic Management Program for Patients with Hereditary Angioedema. Int Arch Allergy Immunol. 2021;182(8):697-708. doi: 10.1159/000513896.

161. Squeglia V, Barbarino A, Bova M, Gravante C, Petraroli A, Spadaro G, et al. High Attack Frequency in Patients with Angioedema Due to C1-Inhibitor Deficiency Is a Major Determinant in Switching to Home Therapy: A Real-Life Observational Study. Orphanet J Rare Dis. 2016;11(1):133. doi: 10.1186/s13023-016-0518-8.

162. Weller K, Groffik A, Magerl M, Tohme N, Martus P, Krause K, et al. Development and Construct Validation of the Angioedema Quality of Life Questionnaire. Allergy. 2012;67(10):1289-98. doi: 10.1111/all.12007.

163. Lumry WR, Weller K, Magerl M, Banerji A, Longhurst HJ, Riedl MA, et al. Impact of Lanadelumab on Health-Related Quality of Life in Patients with Hereditary Angioedema in the HELP Study. Allergy. 2021;76(4):1188-98. doi: 10.1111/all.14680.

164. Weller K, Groffik A, Magerl M, Tohme N, Martus P, Krause K, et al. Development, Validation, and Initial Results of the Angioedema Activity Score. Allergy. 2013;68(9):1185-92. doi: 10.1111/ all.12209.

165. Weller K, Donoso T, Magerl M, Aygören-Pürsün E, Staubach P, Martinez-Saguer I, et al. Development of the Angioedema Control Test-A Patient-Reported Outcome Measure That Assesses Disease Control in Patients with Recurrent Angioedema. Allergy. 2020;75(5):1165-77. doi: 10.1111/all.14144.

166. Aymé S, Kole A, Groft S. Empowerment of Patients: Lessons from the Rare Diseases Community. Lancet. 2008;371(9629):2048-51. doi: 10.1016/S0140-6736(08)60875-2.

167. Abranghe [site na Internet]. Disponível em: https://www.abranghe. org.br. Acessado em: 03/04/2022.

168. Brasil. Ministério da Saúde. Portaria Nº 199, de 30 de janeiro de 2014. Institui a Política Nacional de Atenção Integral às Pessoas com Doenças Raras, aprova as Diretrizes para Atenção Integral às Pessoas com Doenças Raras no âmbito do Sistema Único de Saúde (SUS) e institui incentivos financeiros de custeio. Disponível em: https://bvsms.saude.gov.br/bvs/saudelegis/gm/2014/prt0199_30_01_2014.html . Acessado em: 03/04/2022.

169. Pharma Boardroom [site na Internet]. The Role of Patient Associations in Rare Disease Drug Development. Disponível em: https://pharmaboardroom.com/articles/patient-associations-rolein-rare-disease-drug-development/ . Acessado em: 03/04/2022.

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