Interpretation of international guideline on the diagnosis and management of pediatric patients with hereditary angioedema_Chinese Journal of Evidence-Based Medicine

Authors：

YANG Jia , LI Ting , ZHU Shuzhen ,  YE Sheng

Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, P. R. China;

Corresponding?author：

YE Sheng, Email: yeshengchina@zju.edu.cn

Keywords：

Pediatric guideline; Children; Hereditary angioedema (HAE); C1inhibitor deficiency

DOI：

10.7507/1672-2531.202603139

Video：

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In 2026, the Hereditary Angioedema Working Group and the International Steering Committee issued the international guideline on the diagnosis and management of pediatric patients with hereditary angioedema (HAE). These guidelines elaborate on the definition, clinical features, diagnosis, and treatment of the disease, providing a decision-making basis for the clinical practice of pediatric HAE. This article interprets the main content of the guidelines.

Citation： YANG Jia, LI Ting, ZHU Shuzhen, YE Sheng. Interpretation of international guideline on the diagnosis and management of pediatric patients with hereditary angioedema. Chinese Journal of Evidence-Based Medicine, 2026, 26(8): 975-985. doi: 10.7507/1672-2531.202603139 Copy

1.	Minafra FG, Gon?alves TR, Alves TM, et al. The mortality from hereditary angioedema worldwide: a review of the real-world data literature[J]. Clin Rev Allergy Immunol, 2022, 62(1): 232-239.
2.	Liu S, Wang X, Xu Y, et al. Risk factors for diagnostic delay in chinese patients with hereditary angioedema[J]. Allergy Asthma Proc, 2019, 40(5): 343-349.
3.	Henriette F, Inmaculada M, Konrad B, et al. International guideline on the diagnosis and management of pediatric patients with hereditary angioedema[J]. Allergy, 2026, [Epub a head of print].
4.	Rosi-Schumacher M, Shah SJ, Craig T, et al. Clinical manifestations of hereditary angioedema and a systematic review of treatment options[J]. Laryngoscope Investig Otolaryngol, 2021, 6(3): 394-403.
5.	中國醫師協會變態反應醫師分會, 中華醫學會變態反應學分會, 中國罕見病聯盟北京罕見病診療與保障學會變態反應學分會, 等. 中國遺傳性血管性水腫診斷與治療專家共識(2024版)[J]. 中華臨床免疫和變態反應雜志, 2025, 19(1): 1-10.
6.	姚我, 吳定錢, 沈穎, 等. 遺傳性血管性水腫診治的“院內多學科會診協作網聯動線上線下全程管理”模式的初探[J]. 中華預防醫學雜志, 2024, 58(3): 406-413.
7.	Pagnier A, Dermesropian A, Kevorkian-Verguet C, et al. Hereditary angioedema in children: review and practical perspective for clinical management[J]. Pediatr Allergy Immunol, 2024, 35(12): e14268.
8.	Shuang L, Qun XU, Yingyang XU, et al. Current status of the management of hereditary angioedema in China: a patient-based, cross-sectional survey[J]. Eur J Dermatol, 2020, 30(2): 169-176.
9.	Wang X, Lei S, Xu Y, et al. Mutation update of SERPING1 related to hereditary angioedema in the Chinese population[J]. Hereditas, 2022, 159(1): 28.
10.	Grumach AS, Veronez CL, Csuka D, et al. Angioedema without wheals: challenges in laboratorial diagnosis[J]. Front Immunol, 2021, 12: 785736.
11.	徐迎陽, 支玉香. 中國遺傳性血管性水腫的診療現狀與未來[J]. 罕見病研究, 2025, 4(3): 283-286.
12.	Wong JCY, Lam DLY, Yim JSH, et al. Validating and utilizing dried blood spots for family screening: screening programme providing outreach for testing hereditary angioedema (SPPOT-HAE)[J]. J Allergy Clin Immunol Glob, 2025, 4(1): 100381.
13.	Kesh S, Bernstein JA. Isolated angioedema: a review of classification and update on management[J]. Ann Allergy Asthma Immunol, 2022, 129(6): 692-702.
14.	中華醫學會皮膚性病學分會, 中國醫師協會皮膚科醫師分會. 遺傳性血管性水腫診療路徑皮膚科專家共識(2025版)[J]. 中華皮膚科雜志, 2025, 58(6): 497-502.
15.	遺傳性血管性水腫消化科診療協作組. 遺傳性血管性水腫消化科診療路徑[J]. 中華消化雜志, 2024, 44(5): 289-295.
16.	周寧, 韓小彤, 陳松, 等. 遺傳性血管性水腫急診科診療路徑[J]. 中國急救醫學, 2024, 44(2): 99-105.
17.	周敏, 羅新, 周琪琳, 等. 遺傳性血管性水腫患者診療流程與健康管理模式[J]. 中華預防醫學雜志, 2023, 57(8): 1280-1285.
18.	Syed YY. Lanadelumab: first global approval[J]. Drugs, 2018, 78(15): 1633-1637.
19.	Busse PJ, Christiansen SC, Riedl MA, et al. US HAEA medical advisory board 2020 guidelines for the management of hereditary angioedema[J]. J Allergy Clin Immunol Pract, 2021, 9(1): 132-150.
20.	Ocak M, Nain E, Akarsu A, et al. Health-related quality of life in children with hereditary angioedema compared with patients with histaminergic angioedema[J]. Allergy Asthma Proc, 2021, 42(4): 325-332.
21.	Forjaz MJ, Ayala A, Caminoa M, et al. HAE-AS: a specific disease activity scale for hereditary angioedema with C1-inhibitor deficiency[J]. J Investig Allergol Clin Immunol, 2021, 31(3): 246-252.
22.	Mak HWF, Wong JCY, So SWM, et al. Validation and correlations of the Angioedema Activity Score (AAS), Angioedema Quality of Life (AE-QoL) questionnaire, and Angioedema Control Test (AECT) in Chinese patients with angioedema[J]. J Allergy Clin Immunol Glob, 2024, 3(4): 100295.
23.	Stephen B, Karen B, Rozita B, et al. The international/Canadian hereditary angioedema guideline[J]. Allergy Asthma Clin Immunol, 2026, 22(1): 24.Stephen B, Karen B, Rozita B, et al. The international/Canadian hereditary angioedema guideline[J]. Allergy Asthma Clin Immunol, 2026, 22(1): 24.
24.	Piotrowicz-Wójcik K, Bulanda M, Czarnobilska E, et al. Clinical characteristics and quality of life in a cohort of polish pediatric patients with hereditary angioedema[J]. Children (Basel), 2024, 11(2): 237.
25.	Tachdjian R, Kaplan AP. A comprehensive management approach in pediatric and adolescent patients with hereditary angioedema[J]. Clin Pediatr (Phila), 2023, 62(9): 973-980.
26.	Honda D, Li PH, Jindal AK, et al. Uncovering the true burden of hereditary angioedema due to C1-inhibitor deficiency: a focus on the Asia-Pacific region[J]. J Allergy Clin Immunol, 2024, 153(1): 42-54.
27.	Sharma R, Basu S, Tyagi R, et al. Quality of life in patients with hereditary angioedema correlates with angioedema control: our experience at Chandigarh, India[J]. Asia Pac Allergy, 2025, 15(1): 1-6.
28.	Riedl MA, Raffi T, Lumry WR, et al. Efficacy and safety of donidalorsen for hereditary angioedema[J]. N Engl J Med, 2024, 391(1): 21-31.
29.	Bernatoniene J, Bourgoin-Heck M, Cancian M, et al. Oral berotralstat for hereditary angioedema prophylaxis in patients aged 2 to <12 years: APeX-P interim results[J]. Ann Allergy Asthma Immunol, 2025, 135(6): 681-688.

1. Minafra FG, Gon?alves TR, Alves TM, et al. The mortality from hereditary angioedema worldwide: a review of the real-world data literature[J]. Clin Rev Allergy Immunol, 2022, 62(1): 232-239.
2. Liu S, Wang X, Xu Y, et al. Risk factors for diagnostic delay in chinese patients with hereditary angioedema[J]. Allergy Asthma Proc, 2019, 40(5): 343-349.
3. Henriette F, Inmaculada M, Konrad B, et al. International guideline on the diagnosis and management of pediatric patients with hereditary angioedema[J]. Allergy, 2026, [Epub a head of print].
4. Rosi-Schumacher M, Shah SJ, Craig T, et al. Clinical manifestations of hereditary angioedema and a systematic review of treatment options[J]. Laryngoscope Investig Otolaryngol, 2021, 6(3): 394-403.
5. 中國醫師協會變態反應醫師分會, 中華醫學會變態反應學分會, 中國罕見病聯盟北京罕見病診療與保障學會變態反應學分會, 等. 中國遺傳性血管性水腫診斷與治療專家共識(2024版)[J]. 中華臨床免疫和變態反應雜志, 2025, 19(1): 1-10.
6. 姚我, 吳定錢, 沈穎, 等. 遺傳性血管性水腫診治的“院內多學科會診協作網聯動線上線下全程管理”模式的初探[J]. 中華預防醫學雜志, 2024, 58(3): 406-413.
7. Pagnier A, Dermesropian A, Kevorkian-Verguet C, et al. Hereditary angioedema in children: review and practical perspective for clinical management[J]. Pediatr Allergy Immunol, 2024, 35(12): e14268.
8. Shuang L, Qun XU, Yingyang XU, et al. Current status of the management of hereditary angioedema in China: a patient-based, cross-sectional survey[J]. Eur J Dermatol, 2020, 30(2): 169-176.
9. Wang X, Lei S, Xu Y, et al. Mutation update of SERPING1 related to hereditary angioedema in the Chinese population[J]. Hereditas, 2022, 159(1): 28.
10. Grumach AS, Veronez CL, Csuka D, et al. Angioedema without wheals: challenges in laboratorial diagnosis[J]. Front Immunol, 2021, 12: 785736.
11. 徐迎陽, 支玉香. 中國遺傳性血管性水腫的診療現狀與未來[J]. 罕見病研究, 2025, 4(3): 283-286.
12. Wong JCY, Lam DLY, Yim JSH, et al. Validating and utilizing dried blood spots for family screening: screening programme providing outreach for testing hereditary angioedema (SPPOT-HAE)[J]. J Allergy Clin Immunol Glob, 2025, 4(1): 100381.
13. Kesh S, Bernstein JA. Isolated angioedema: a review of classification and update on management[J]. Ann Allergy Asthma Immunol, 2022, 129(6): 692-702.
14. 中華醫學會皮膚性病學分會, 中國醫師協會皮膚科醫師分會. 遺傳性血管性水腫診療路徑皮膚科專家共識(2025版)[J]. 中華皮膚科雜志, 2025, 58(6): 497-502.
15. 遺傳性血管性水腫消化科診療協作組. 遺傳性血管性水腫消化科診療路徑[J]. 中華消化雜志, 2024, 44(5): 289-295.
16. 周寧, 韓小彤, 陳松, 等. 遺傳性血管性水腫急診科診療路徑[J]. 中國急救醫學, 2024, 44(2): 99-105.
17. 周敏, 羅新, 周琪琳, 等. 遺傳性血管性水腫患者診療流程與健康管理模式[J]. 中華預防醫學雜志, 2023, 57(8): 1280-1285.
18. Syed YY. Lanadelumab: first global approval[J]. Drugs, 2018, 78(15): 1633-1637.
19. Busse PJ, Christiansen SC, Riedl MA, et al. US HAEA medical advisory board 2020 guidelines for the management of hereditary angioedema[J]. J Allergy Clin Immunol Pract, 2021, 9(1): 132-150.
20. Ocak M, Nain E, Akarsu A, et al. Health-related quality of life in children with hereditary angioedema compared with patients with histaminergic angioedema[J]. Allergy Asthma Proc, 2021, 42(4): 325-332.
21. Forjaz MJ, Ayala A, Caminoa M, et al. HAE-AS: a specific disease activity scale for hereditary angioedema with C1-inhibitor deficiency[J]. J Investig Allergol Clin Immunol, 2021, 31(3): 246-252.
22. Mak HWF, Wong JCY, So SWM, et al. Validation and correlations of the Angioedema Activity Score (AAS), Angioedema Quality of Life (AE-QoL) questionnaire, and Angioedema Control Test (AECT) in Chinese patients with angioedema[J]. J Allergy Clin Immunol Glob, 2024, 3(4): 100295.
23. Stephen B, Karen B, Rozita B, et al. The international/Canadian hereditary angioedema guideline[J]. Allergy Asthma Clin Immunol, 2026, 22(1): 24.Stephen B, Karen B, Rozita B, et al. The international/Canadian hereditary angioedema guideline[J]. Allergy Asthma Clin Immunol, 2026, 22(1): 24.
24. Piotrowicz-Wójcik K, Bulanda M, Czarnobilska E, et al. Clinical characteristics and quality of life in a cohort of polish pediatric patients with hereditary angioedema[J]. Children (Basel), 2024, 11(2): 237.
25. Tachdjian R, Kaplan AP. A comprehensive management approach in pediatric and adolescent patients with hereditary angioedema[J]. Clin Pediatr (Phila), 2023, 62(9): 973-980.
26. Honda D, Li PH, Jindal AK, et al. Uncovering the true burden of hereditary angioedema due to C1-inhibitor deficiency: a focus on the Asia-Pacific region[J]. J Allergy Clin Immunol, 2024, 153(1): 42-54.
27. Sharma R, Basu S, Tyagi R, et al. Quality of life in patients with hereditary angioedema correlates with angioedema control: our experience at Chandigarh, India[J]. Asia Pac Allergy, 2025, 15(1): 1-6.
28. Riedl MA, Raffi T, Lumry WR, et al. Efficacy and safety of donidalorsen for hereditary angioedema[J]. N Engl J Med, 2024, 391(1): 21-31.
29. Bernatoniene J, Bourgoin-Heck M, Cancian M, et al. Oral berotralstat for hereditary angioedema prophylaxis in patients aged 2 to <12 years: APeX-P interim results[J]. Ann Allergy Asthma Immunol, 2025, 135(6): 681-688.

Chinese Journal of Evidence-Based Medicine

Interpretation of international guideline on the diagnosis and management of pediatric patients with hereditary angioedema

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