The place of scales and questionnaires in assessing the disease’s severity and the long-term prophylaxis’s prescribing in patients with hereditary angioedema

Cover Page

Cite item

Full Text

Abstract

Hereditary angioedema is a hereditary life-threatening disease characterized by recurrent angioedema of various strength and localization. To date, definite disease’s severity criteria have not been developed. There are many different factors to consider not only the frequency of episodes, but also their duration, strength, influence on the patient's appearance, the severity of the pain syndrome. Disease related quality of life, the number of work disability days, and the patient's satisfaction with the prescribed treatment are important. In addition, there are no criteria for prescribing long-term prophylaxis, and no parameters for choosing a specific drug. The introduction of specific scales and questionnaires developed to assess such characteristics as disease activity, disease control, quality of life in patients with recurrent angioedema into clinical practice seems to be the best solution for both questions. The comprehensive understanding of the disease’s severity in a particular patient can be carried out with the combined use of assessment tools.

About the authors

Irina A. Manto

State Research Center – Institute of Immunology

Author for correspondence.
Email: irina.manto@yandex.ru
ORCID iD: 0000-0001-6432-394X

науч. сотр.

Russian Federation, Moscow

Elena A. Latysheva

State Research Center – Institute of Immunology; Pirogov Russian National Research Medical University

Email: irina.manto@yandex.ru
ORCID iD: 0000-0002-1606-205X

д-р мед. наук, вед. науч. сотр., доц. каф. клинической иммунологии фак-та МБФ

Russian Federation, Moscow; Moscow

Leya E. Sorokina

State Research Center – Institute of Immunology; Vernadsky Crimean Federal University

Email: irina.manto@yandex.ru
ORCID iD: 0000-0002-1862-6816

ординатор 1-го года обучения, лаборант центральной научно-исследовательской лаб.

Russian Federation, Moscow; Simferopol

Tatiana V. Latysheva

State Research Center – Institute of Immunology; Yevdokimov Moscow State University of Medicine and Dentistry

Email: irina.manto@yandex.ru
ORCID iD: 0000-0003-1508-0640

д-р мед. наук, проф., зав. отд. иммунопатологии, проф. каф. клинической аллергологии и иммунологии лечебного факультета

Russian Federation, Moscow; Moscow

References

  1. Близнец Е.А., Викторова Е.А., Вишнева Е.А., и др. Наследственный ангиоотек. Клинические рекомендации (D84.1). Российский аллергологический журнал. 2021;18(2):77-114 [Bliznec EA, Viktorova EA, Vishneva EA, et al. Hereditary angioedema. Clinical guidelines (D84.1). Russian Journal of Allergy. 2021;18(2):77-114 (in Russian)]. doi: 10.36691/RJA1447
  2. Maurer M, Magerl M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2017 revision and update. Allergy. 2018;73(8):1575-96. doi: 10.1111/all.13384
  3. Bouillet L, Launay D, Fain O, et al. Hereditary angioedema with C1 inhibitor deficiency: clinical presentation and quality of life of 193 French patients. Ann Allergy Asthma Immunol. 2013;111(4):290-4. doi: 10.1016/j.anai.2013.07.012
  4. Bowen T, Cicardi M, Farkas H, et al. Canadian 2003 International Consensus Algorithm For the Diagnosis, Therapy, and Management of Hereditary Angioedema. J Allergy Clin Immunol. 2004;114(3):629-37. doi: 10.1016/j.jaci.2004.06.043
  5. 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(1):132-150.e3. doi: 10.1016/j.jaip.2020.08.046
  6. 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;13:73. doi: 10.1186/s13023-018-0815-5
  7. Craig T, Busse P, Gower RG, et al. Long-term prophylaxis therapy in patients with hereditary angioedema with C1 inhibitor deficiency. Ann Allergy Asthma Immunol. 2018;121(6):673-9. doi: 10.1016/j.anai.2018.07.025
  8. Syed YY. Lanadelumab: First Global Approval. Drugs. 2018;78(15):1633-7. doi: 10.1007/s40265-018-0987-2
  9. Riedl MA, Bernstein JA, Craig T, et al. An open-label study to evaluate the long-term safety and efficacy of lanadelumab for prevention of attacks in hereditary angioedema: design of the HELP study extension. Clin Transl Allergy. 2017;7:36. doi: 10.1186/s13601-017-0172-9
  10. Nicola S, Rolla G, Brussino L. Breakthroughs in hereditary angioedema management: a systematic review of approved drugs and those under research Database and key words (MeSH). Drugs Context. 2019;8:212605. doi: 10.7573/dic.212605
  11. Riedl MA, Banerji A, Gower R. Current medical management of hereditary angioedema. Ann Allergy Asthma Immunol. 2021;126(3):264-72. doi: 10.1016/j.anai.2020.10.009
  12. Бодня О.С., Демина Д.В., Кузьменко Н.Б., и др. Долгосрочная профилактика наследственного ангиоотека в России: резолюция Совета экспертов. Российский аллергологический журнал. 2021;18(3):126-30 [Bodnja OS, Demina DV, Kuz'menko NB, et al. Long-term prophylaxis therapy in patients with hereditary angioedema in Russia: resolution of the expert Council. Russian Journal of Allergy. 2021;18(3):126-30 (in Russian)]. doi: 10.36691/RJA1485
  13. Weller K, Magerl M, Peveling-Oberhag A, et al. The Angioedema Quality of Life Questionnaire (AE-QoL) – assessment of sensitivity to change and minimal clinically important difference. Allergy. 2016;71(8):1203-9. doi: 10.1111/all.12900
  14. Weller K, Groffik A, Magerl M, et al. Development, validation, and initial results of the Angioedema Activity Score. Allergy. 2013;68(9):1185-92. doi: 10.1111/all.12209
  15. Forjaz MJ, Ayala A, Caminoa M, et al. HAE-AS, a specific disease activity scale for hereditary angioedema with C1-inhibitor deficiency. J Investig Allergol Clin Immunol. 2021;31(3):246-52. doi: 10.18176/jiaci.0479
  16. Weller K, Donoso T, Magerl M, 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):2050-7.e4. doi: 10.1016/j.jaip.2020.02.038
  17. Bork K, Anderson JT, Caballero T, 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. Published 2021 Apr 19. doi: 10.1186/s13223-021-00537-2
  18. Bygum A, Busse P, Caballero T, Maurer M. Disease severity, activity, impact, and control and how to assess them in patients with hereditary angioedema. Front Med (Lausanne). 2017;4:212. doi: 10.3389/fmed.2017.00212
  19. Buttgereit T, Vera C, Weller K, 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
  20. McMillan CV, Speight J, Relan A, et al. Content Validity of Visual Analog Scales to Assess Symptom Severity of Acute Angioedema Attacks in Adults with Hereditary Angioedema. Patient. 2012;5(2):113-26. doi: 10.2165/11597490-000000000-00000
  21. Lumry WR, Castaldo AJ, Vernon MK, et al. The humanistic burden of hereditary angioedema: Impact on health-related quality of life, productivity, and depression. Allergy Asthma Proc. 2010;31(5):407-14. doi: 10.2500/aap.2010.31.3394
  22. Aabom A, Andersen K, Perez-Fernández E, et al. Health-related Quality of Life in Danish Patients with Hereditary Angioedema. Acta Derm Venereol. 2015;95(2):225-6. doi: 10.2340/00015555-1835
  23. Lumry WR, Miller DP, Newcomer S, et al. Quality of life in patients with hereditary angioedema receiving therapy for routine prevention of attacks. Allergy Asthma Proc. 2014;35(5):371-6. doi: 10.2500/aap.2014.35.3783
  24. Sánchez MD, Cuervo J, Rave D, et al. Hereditary angioedema in Medellín (Colombia): Clinical evaluation and quality of life appraisal. Biomedica. 2015;35(3):419-28. doi: 10.7705/biomedica.v35i3.2417
  25. Weller K, Groffik A, Magerl M, et al. Development and construct validation of the angioedema quality of life questionnaire. Allergy. 2012;67(10):1289-98. doi: 10.1111/all.12007
  26. Kulthanan K, Chularojanamontri L, Rujitharanawong C, et al. Angioedema quality of life questionnaire (AE-QoL) – interpretability and sensitivity to change. Health Qual Life Outcomes. 2019;17(1):160. doi: 10.1186/s12955-019-1229-3
  27. Morioke S, Takahagi S, Kawano R, et al. A validation study of the Japanese version of the Angioedema Activity Score (AAS) and the Angioedema Quality of Life Questionnaire (AE-QoL). Allergol Int. 2021;70(4):471-9. doi: 10.1016/j.alit.2021.04.006
  28. Kulthanan K, Chularojanamontri L, Rujitharanawong C, et al. Angioedema Activity Score (AAS): A Valid and Reliable Tool to Use in Asian Patients. BioMed Research Int. 2019;2019:9157895. doi: 10.1155/2019/9157895
  29. Weller K, Donoso T, Magerl M, 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
  30. Agostoni A, Aygoren-Pursun E, Binkley KE, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol. 2004;114(3 Suppl.):S51-131. doi: 10.1016/j.jaci.2004.06.047
  31. Bygum A, Fagerberg CR, Ponard D, et al. Mutational spectrum and phenotypes in Danish families with hereditary angioedema because of C1 inhibitor deficiency. Allergy. 2011;66(1):76-84. doi: 10.1111/j.1398-9995.2010.02456.x
  32. Bafunno V, Bova M, Loffredo S, et al. Mutational spectrum of the C1 inhibitor gene in a cohort of italian patients with hereditary angioedema: Description of nine novel mutations. Ann Hum Genet. 2014;78(2):73-82. doi: 10.1111/ahg.12052
  33. Andrejević SS, Korošec P, Šilar M, et al. Hereditary Angioedema Due to C1 Inhibitor Deficiency in Serbia: Two Novel Mutations and Evidence of Genotype-Phenotype Association. PLoS One. 2015;10(11):e0142174. doi: 10.1371/journal.pone.0142174
  34. Кузьменко Н.В., Викторова Е.А., Павлова А.В., и др. Характеристика молекулярно-генетических дефектов и клинических особенностей в группе пациентов с наследственным ангионевротическим отеком 1-го и 2-го типов. Вопросы гематологии/онкологии и иммунопатологии в педиатрии. 2017;16(4):35-42 [Kuzmenko NB, Viktorova EA, Pavlova AV, et al. Genetic and clinical characteristics of a group of patients with hereditary angioedema type 1 and 2. Pediatric Hematology/Oncology and Immunopathology. 2017;16(4):35-42 (in Russian)]. doi: 10.24287/1726-1708-2017-16-4-35-42
  35. Maia LSM, Moreno AS, Ferriani MPL, et al. Genotype-phenotype correlations in Brazilian patients with hereditary angioedema due to C1 inhibitor deficiency. Allergy. 2019;74(5):1013-6. doi: 10.1111/all.13699
  36. Prior N, Remor E, Gómez-Traseira C, 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;10:82. doi: 10.1186/1477-7525-10-82
  37. Zuberbier T, Abdul Latiff AH, Abuzakouk M, et al. The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria. Allergy. 2021. doi: 10.1111/all.15090
  38. Moxie GmbH. Our products. Commercial use. Accessed November 5, 2021. Available at: https://moxie-gmbh.de/our-products/commercial-use. Accessed: 25.10.2021

Supplementary files

Supplementary Files
Action
1. JATS XML
2. Fig. 1. Classification of hereditary angioedema.

Download (251KB)

Copyright (c) 2021 Consilium Medicum

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
 
 


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies