Profilaxia de curto prazo com inibidor de C1 e normalização do C4 em paciente com angioedema hereditário: relato de caso
Short-term prophylactic use of C1 inhibitor and C4 normalization in a patient with hereditary angioedema: a case report
Stéphanie Kim Azevedo de Almeida; Gabriela Barbosa Bisson; Flávio Wellington da Silva Ferraz; Marcelo Vivolo Aun; Jorge Kalil; Pedro Giavina-Bianchi
Resumo
O angioedema hereditário (AEH) é uma doença genética rara, caracterizada por episódios recorrentes de edema subcutâneo ou submucoso, frequentemente incapacitantes. As crises de AEH são geralmente imprevisíveis, embora possam ser desencadeadas por fatores como procedimentos dentários ou médicos (incluindo cirurgias), traumas ou estresse. Neste relato, descrevemos o caso de uma paciente com AEH tipo 1 que necessitou de exodontia dos terceiros molares. Foi administrado o inibidor de C1 derivado de plasma humano duas horas antes do procedimento, prevenindo o surgimento de crises de angioedema durante e após a extração. Além disso, observou-se a normalização dos níveis de C4 após a aplicação da medicação.
Palavras-chave
Abstract
Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent episodes of subcutaneous or submucosal edema, which are often debilitating. While HAE attacks are generally unpredictable, they can sometimes be triggered by factors such as dental or medical procedures (including surgery), trauma, or stress. In this report, we present the case of a patient with type 1 HAE who required the extraction of her third molars. Human plasma-derived C1 inhibitor administered 2 hours prior to the extraction prevented angioedema attacks during and after the procedure. Additionally, normalization of C4 levels was observed following the administration of the medication.
Keywords
Referências
1. Maurer M, Magerl M, Betschel S, Aberer W, Ansotegui IJ, Aygoren-Pursun E, et al. The international WAO/EAACI guideline for the management of hereditary angioedema - the 2021 revision and update. Allergy. 2022;1961:1990-77.
2. Campos RA, Serpa FS, Mansour E, Alonso MLO, Arruda LK, Aun MV, et al. Diretrizes brasileiras do angioedema hereditário 2022 - Parte 1: definição, classificação e diagnóstico. Arq Asma Alerg Imunol. 2022;6(2):151-69.
3. Cicardi M, Aberer W, Banerji A, Bas M, Bernstein JA, Bork K, et al.; HAWK under the patronage of EAACI (European Academy of Allergy and Clinical Immunology). Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group. Allergy. 2014 May;69(5):602-16. doi: 10.1111/all.12380.
4. Giavina-Bianchi P, Vivolo Aun M, Giavina-Bianchi M, Ribeiro AJ, Camara Agondi R, Motta AA, et al. Hereditary angioedema classification: Expanding knowledge by genotyping and endotyping. World Allergy Organ J. 2024;17(5):100906.
5. Giavina-Bianchi P, Aun MV, Kalil J. Vascular endothelial growth factor (VEGF) emerging as a mediator of hereditary angioedema (HAE). World Allergy Organ J. 2024;17(8):100942.
6. Davis AE. The pathophysiology of hereditary angioedema. Clin Immunol. 2005;114:3-9.
7. Bork K, Staubach P, Eckhardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am J Gastroenterol. 2006;101:619-27.
8. Cicardi M, Zingale LC, Zanichelli A, Deliliers DL, Caccia S. The use of plasmaderived C1 inhibitor in the treatment of hereditary angioedema. Expert Opin Pharmacother. 2007;8:3173-81.
9. Campos RA, Serpa FS, Mansour E, Alonso MLO, Arruda LK, Aun MV, et al. Diretrizes brasileiras do angioedema hereditário 2022 - Parte 2: terapêutica. Arq Asma Alerg Imunol. 2022;6(2):170-96.
10. Aygoren-Pursun E, Martinez Saguer I, Kreuz W, Klingebiel T, Schwabe D . Risco de angioedema após procedimentos invasivos ou cirúrgicos em HAE tipo I e II - a história natural. Alergia. 2013;68(8):1034-9.
11. Giavina-Bianchi P, Aun MV, Garcia JFB, Gomes LS, Ribeiro AJ, Takejima P, et al. Clinical features of hereditary angioedema and warning signs (H4AE) for its identification. Clinics (Sao Paulo). 2022;77:100023.
12. Craig TJ, Wasserman RL, Levy RJ, Bewtra AK, Schneider L, Packer F, et al. Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema. J Clin Immunol. 2010 Nov;30(6):823-9. doi: 10.1007/s10875-010-9442-1.
13. Martinez-Saguer I, Cicardi M, Suffritti C, Rusicke E, Aygören-Pürsün E, Stoll H, et al. Pharmacokinetics of plasma-derived C1-esterase inhibitor after subcutaneous versus intravenous administration in subjects with mild or moderate hereditary angioedema: the PASSION study. Transfusion. 2014 Jun;54(6):1552-61. doi: 10.1111/trf.12501.
14. Henry LH, Riedl M, Kashkin J. Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema. Clin Rev Allergy Immunol. 2019;56(2):207-18.
15. Bernstein JA, Ritchie B, Levy RJ, Wasserman RL, Bewtra AK, Hurewitz DS, et al. Population pharmacokinetics of plasma-derived C1 esterase inhibitor concentrate used to treat acute hereditary angioedema attacks. Ann Allergy Asthma Immunol. 2010 Aug;105(2):149-54. doi: 10.1016/j.anai.2010.06.005.
Submetido em:
23/01/2025
Aceito em:
06/03/2025
Facebook
Google+
Twitter
LinkedIn
Mendeley
StumbleUpon
CiteULike
Reddit
Email