Ferramentas para avaliação e acompanhamento do angioedema recorrente
Patient-reported outcome measures for the evaluation and follow-up of recurrent angioedema
Solange Oliveira Rodrigues Valle, Sérgio Duarte Dortas-Junior, Maria Luiza Oliveira Alonso, Eliana Toledo, Fernanda Gontijo Minafra, Fernanda Casares Marcelino, Herberto José Chong-Neto, Jane da Silva, Luisa Karla Arruda, Pedro Giavina-Bianchi, Régis de Albuquerque Campos, Eli Mansour, Anete Sevciovic Grumach, Faradiba Sarquis Serpa
Resumo
Os Patient Reported Outcomes Measures (PROMs) são desfechos relatados pelos pacientes que possibilitam avaliar a atividade, o controle e o impacto na qualidade de vida, permitindo decisões terapêuticas centradas no paciente. Existem cinco instrumentos específicos desenvolvidos para a avaliação de pacientes com angioedema recorrente. O Angioedema Activity Score (AAS) que mensura prospectivamente a atividade da doença, com diferença mínima clinicamente importante (Minimal Clinically Important Difference / MCID) de 8 pontos, o Angioedema Control Test (AECT) que avalia o controle percebido pelo paciente, com MCID de 3 pontos, o Angioedema Quality of Life Questionnaire (AE-QoL) que mede o impacto do angioedema na qualidade de vida com MCID de 6 pontos, e o Hereditary Angioedema Quality of Life Questionnaire (HAE-QoL) que é específico para o angioedema hereditário por deficiência de C1INH (AEH-C1INH). O Hereditary Angioedema Activity Score (HAE-AS) também é específico para o AEH-C1INH e avalia a atividade da doença. No Brasil, o AAS, o AE-QoL e o HAEQoL já foram traduzidos e validados para adultos e o AECT está em validação. Apesar de recomendados por diretrizes nacionais e internacionais, o uso clínico dos PROMs ainda é limitado,representando uma lacuna importante. Sua aplicação favorece acompanhamento sistemático, definição da eficácia terapêutica e obtenção de desfechos desejáveis, sendo indispensável tanto na prática clínica quanto em pesquisas. Melhorias futuras incluem validação pediátrica e ampliação de conteúdos específicos da doença.
Palavras-chave
Abstract
Patient-Reported Outcome Measures (PROMs) are outcomes reported directly by patients that allow assessment of disease activity, control, and impact on quality of life, enabling patient-centered therapeutic decisions. Five disease-specific instruments have been developed for evaluating patients with recurrent angioedema. The Angioedema Activity Score (AAS) measures disease activity prospectively, with a Minimal Clinically Important Difference (MCID) of 8 points; the Angioedema Control Test (AECT) assesses patient-perceived disease control, with an MCID of 3 points; the Angioedema Quality of Life Questionnaire (AE-QoL) measures the impact of angioedema on quality of life, with an MCID of 6 points; and the Hereditary Angioedema Quality of Life Questionnaire (HAE-QoL) is specific for hereditary angioedema due to C1-INH deficiency (C1INH-HAE). The Hereditary Angioedema Activity Score (HAE-AS) is also specificfor C1INH-HAE and assesses disease activity. In Brazil, the AAS, AE-QoL, and HAE-QoL have already been translated and validated for adults, and the AECT is currently undergoing validation. Although recommended by national and international guidelines, clinical use of PROMs remains limited, representing an important gap. Their implementation supports systematic follow-up, determination of therapeutic efficacy, and achievement of meaningful outcomes, making them essential in both clinical practice and research. Future improvements include pediatric validation and expansion of disease-specific content.
Keywords
References
1. Reshef A, Buttgereit T, Betschel SD, Caballero T, Farkas H, Grumach AS, et al. Definition, acronyms, nomenclature, and classification of angioedema (DANCE): AAAAI, ACAAI, ACARE, and APAAACI DANCE consensus. J Allergy Clin Immunol. 2024;154(2):398-411.e1. https://doi.org/10.1016/j.jaci.2024.03.024. PMid:38670233.
2. Can PK, Degi Rmentepe EN, Etikan P, Kiziltaç K, Gelincik A, Demir S, et al. Assessment of disease activity and quality of life in patients with recurrent bradykinin-mediated versus mast cellmediated angioedema. World Allergy Organ J. 2021;14(7):100554. https://doi.org/10.1016/j.waojou.2021.100554. PMid:34221217.
3. Sussman G, Abuzakouk M, Bérard F, Canonica W, Oude Elberink H, Giménez-Arnau A, et al. Angioedema in chronic spontaneous urticaria is underdiagnosed and has a substantial impact: analyses from ASSURE-CSU. Allergy. 2018;73(8):1724-34. https://doi.org/10.1111/all.13430. PMid:29460968.
4. Maurer M, Magerl M, Betschel S, Aberer W, Ansotegui IJ, Aygören-Pürsün E, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update. Allergy. 2022;77(7):1961-90. https://doi.org/10.1111/all.15214. PMid:35006617.
5. Zuberbier T, Abdul Latiff AH, Abuzakouk M, Aquilina S, Asero R, Baker D, et al. The international EAACI/GA2LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria. Allergy. 2022;77(3):734-66. https://doi.org/10.1111/all.15090. PMid:34536239.
6. Valle SOR, Dortas-Junior SD, Dias GAC, Motta AA, Falcao-Amaral CS, Martins EAPR, et al. Ferramentas para avaliação e acompanhamento da urticária crônica. Arq Asma Alerg Imunol. 2018;2(2):209-24. https://doi.org/10.5935/2526-5393.20180021.
7. Campos RA, Serpa FS, Mansour E, Alonso MLO, Arruda LK, Aun MV, et al. Diretrizes brasileiras de angioedema hereditário 2022 - Parte 2: terapêutica. Arq Asma Alerg Imunol. 2022;6(2):170-96. https://doi.org/10.5935/2526-5393.20220020.
8. 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. https://doi.org/10.1111/all.12209. PMid:23919330.
9. 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. https://doi.org/10.1111/all.12007. PMid:22913638.
10. Prior N, Remor E, Gómez-Traseira C, López-Serrano C, Cabañas R, Contreras J, et al. Development of a disease-specific quality of life questionnaire for adult patients with hereditary angioedema due to C1 inhibitor deficiency (HAE-QoL): spanish multi-centre research project. Health Qual Life Outcomes. 2012;10(1):82. https://doi.org/10.1186/1477-7525-10-82. PMid:22817696.
11. 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. https://doi.org/10.1111/all.14144. PMid:31815297.
12. Forjaz MJ, Ayala A, Caminoa M, Prior N, Pérez-Fernández E, Caballero T. HAE-AS: a specific disease activity scale for hereditary angioedema with C1-inhibitor deficiency. J Investig Allergol Clin Immunol. 2021;31(3):246-52. https://doi.org/10.18176/jiaci.0479. PMid:31932270.
13. Heibati B, Borle J, Ritchie B, Adatia A. Disease control and disease activity in hereditary angioedema: two sides of the same coin? Front Immunol. 2025;16:1631448. https://doi.org/10.3389/fimmu.2025.1631448. PMid:40766317.
14. Martins-Aquino B, Lacerda AE, Marcelino FC, Gonçalves-Silva B, Nunes ICC, Solé D, et al. Tradução e adaptação transcultural para o idioma português (Brasil) do instrumento “Angioedema Activity Score”. Arq Asma Alerg Imunol. 2018;2(3):351-6.
15. Prior N, Remor E, Pérez-Fernández E, Caminoa M, Gómez-Traseira 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):464-473.e4. https://doi.org/10.1016/j.jaip.2015.12.010. PMid:26969268.
16. HAE International. 12th C1-Inhibitor deficiency & angioedema workshop. Lausanne: Frontiers Media SA; 2021 doi:https://doi.org/10.3389/978-2-88966-549-5.
17. Weller K, Magerl M, Peveling-Oberhag A, Martus P, Staubach P, Maurer M. The Angioedema Quality of Life Questionnaire (AEQoL) - Assessment of sensitivity to change and minimal clinically important difference. Allergy. 2016;71(8):1203-9. https://doi.org/10.1111/all.12900. PMid:27038109.
18. Weller K, Donoso T, Magerl M, Aygören-Pürsün E, Staubach P, Martinez-Saguer I, et al. Validation of the Angioedema Control Test (AECT)-A patient-reported outcome instrument for assessing angioedema control. J Allergy Clin Immunol Pract. 2020;8(6):2050-2057.e4. https://doi.org/10.1016/j.jaip.2020.02.038. PMid:32173507.
19. Fijen LM, Vera C, Buttgereit T, Bonnekoh H, Maurer M, Magerl M, et al. Sensitivity to change and minimal clinically important difference of the angioedema control test. Clin Transl Allergy. 2023;13(9):e12295. https://doi.org/10.1002/clt2.12295. PMid:37746798.
20. Chong-Neto HJ. A narrative review of recent literature of the quality of life in hereditary angioedema patients. World Allergy Organ J. 2023;16(3):100758. https://doi.org/10.1016/j.waojou.2023.100758. PMid:36994443.
21. Baroni I, Paglione G, De Angeli G, Angolani M, Callus E, Magon A, et al. A COSMIN systematic review of instruments for evaluating health-related quality of life in people with Hereditary Angioedema. Health Qual Life Outcomes. 2025;23(1):12. https://doi.org/10.1186/s12955-025-02342-6. PMid:39948647.
Submitted date:
11/23/2025
Accepted date:
02/12/2026
Facebook
LinkedIn
Mendeley
StumbleUpon
Reddit
Email