Literaturverzeichnis

Aktuelle Leitlinien und Konsens-Empfehlungen für die Therapie des HAE

Maurer M et al., The international WAO/EAACI guideline for the management of hereditaryangioedema-The 2021 revision and update. Allergy. 2022 Jan 10 https://pubmed.ncbi.nlm.nih.gov/35006617/

Maurer M et al., Consensus on treatment goals in hereditary angioedema: A global Delphi initiative. J Allergy Clin Immunol. 2021 May 25  https://pubmed.ncbi.nlm.nih.gov/34048855/

Busse PJ et al., US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema, The Journal of Allergy and Clinical Immunology: In Practice, Volume 9, Issue 1, 2021, Pages 132-150.e3, ISSN 2213-2198,  https://jhu.pure.elsevier.com/en/publications/us-haea-medical-advisory-board-2020-guidelines-for-the-management

Wahn V et al. Hereditary angioedema in children and adolescents – A consensus update on therapeutic strategies for German‐speaking countries, Pediatr Allergy Immunol. 2020;31:974–989  https://onlinelibrary.wiley.com/doi/full/10.1111/pai.13309

Betsche S et al., The International/Canadian Hereditary Angioedema Guideline. Allergy Asthma Clin Immunol. 2019 Nov 25;15:72. doi: 10.1186/s13223-019-0376-8. Erratum in: Allergy Asthma Clin Immunol. 2020 May 6;16:33. PMID: 31788005; PMCID: PMC6878678. https://pubmed.ncbi.nlm.nih.gov/31788005/

Bork K. et al., Guideline: Hereditary angioedema due to C1 inhibitor deficiency. Allergo J Int 28, 16–29 (2019). https://link.springer.com/article/10.1007/s40629-018-0088-5

Maurer M et al., The international WAO/EAACI guideline for the management of hereditary angioedema-The 2017 revision and update. Allergy. 2018 Aug;73(8):1575-1596. doi: 10.1111/all.13384. Epub 2018 Mar 12. PMID: 29318628.   https://pubmed.ncbi.nlm.nih.gov/29318628/

Krankheitslast durch HAE und HAE-Management

Bork K, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol. 2012 Sep;130(3):692-7. doi: 10.1016/j.jaci.2012.05.055. Epub 2012 Jul 28. PMID: 22841766. https://pubmed.ncbi.nlm.nih.gov/22841766/

Banerji A, Davis KH, Brown TM, Hollis K, Hunter SM, Long J, Jain G, Devercelli G. Patient-reported burden of hereditary angioedema: findings from a patient survey in the United States. Ann Allergy Asthma Immunol. 2020 Jun;124(6):600-607. doi: 10.1016/j.anai.2020.02.018. Epub 2020 Mar 10. PMID: 32169514. https://pubmed.ncbi.nlm.nih.gov/32169514/

Mendivil J, Murphy R, de la Cruz M, Janssen E, Boysen HB, Jain G, Aygören-Pürsün E, Hirji I, Devercelli G. Clinical characteristics and burden of illness in patients with hereditary angioedema: findings from a multinational patient survey. Orphanet J Rare Dis. 2021 Feb 18;16(1):94. doi: 10.1186/s13023-021-01717-4. PMID: 33602292; PMCID: PMC7893968. https://pubmed.ncbi.nlm.nih.gov/33602292/

Hahn J, Hoess A, Friedrich DT, Mayer B, Schauf L, Hoffmann TK, Greve J. Unnecessary abdominal interventions in patients with hereditary angioedema. J Dtsch Dermatol Ges. 2018 Dec;16(12):1443-1449. doi: 10.1111/ddg.13698. Epub 2018 Nov 21. PMID: 30462881. https://pubmed.ncbi.nlm.nih.gov/30462881/

Lumry WR, Settipane RA. Hereditary angioedema: Epidemiology and burden of disease. Allergy Asthma Proc. 2020 Nov 1;41(Suppl 1):S08-S13. doi: 10.2500/aap.2020.41.200050. PMID: 33109318. https://pubmed.ncbi.nlm.nih.gov/33109318/

Radojicic C, Riedl MA, Craig TJ, Best JM, Rosselli J, Hahn R, Banerji A. Patient perspectives on the treatment burden of injectable medication for hereditary angioedema. Allergy Asthma Proc. 2021 May 1;42(3):S4-S10. doi: 10.2500/aap.2021.42.210025. PMID: 33980327. https://pubmed.ncbi.nlm.nih.gov/33980327/

Magerl M, Sala-Cunill A, Weber-Chrysochoou C, Trainotti S, Mormile I, Spadaro G. Could it be hereditary angioedema?-Perspectives from different medical specialties. Clin Transl Allergy. 2023 Sep;13(9):e12297. doi: 10.1002/clt2.12297. PMID: 37746796; PMCID: PMC10509412. https://pubmed.ncbi.nlm.nih.gov/37746796/

Paula J. Busse, Sandra C. Christiansen, Marc A. Riedl, Aleena Banerji, Jonathan A. Bernstein, Anthony J. Castaldo, Timothy Craig, Mark Davis-Lorton, Michael M. Frank, H. Henry Li, William R. Lumry, Bruce L. Zuraw, US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema, The Journal of Allergy and Clinical Immunology: In Practice, Volume 9, Issue 1, 2021, Pages 132-150.e3, ISSN 2213-2198 https://www.sciencedirect.com/science/article/pii/S2213219820308783?via%3Dihub

Übersichtsarbeiten zu HAE

Maurer M, Magerl M. Differences and Similarities in the Mechanisms and Clinical Expression of Bradykinin-Mediated vs. Mast Cell-Mediated Angioedema. Clin Rev Allergy Immunol. 2021 Feb 3. doi: 10.1007/s12016-021-08841-w. Epub ahead of print. PMID: 33534062.  https://pubmed.ncbi.nlm.nih.gov/33534062/

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 Jan 9. doi: 10.1007/s12016-021-08832-x. Epub ahead of print. PMID: 33423210.  https://pubmed.ncbi.nlm.nih.gov/33423210/

Fakten kompakt Berinert – Ersetzen, was fehlt: C1-Esterase-Inhibitor-Therapie beim hereditären Angioödem, Thieme Verlag 2020  https://lp.thieme.de/emag/CP/Fakten-kompakt-Berinert-2020

Aygören-Pürsün E, Bork K. Hereditäres Angioödem [Hereditary angioedema]. Internist (Berl). 2019 Sep;60(9):987-995. German. doi: 10.1007/s00108-019-0644-1. PMID: 31363809. https://pubmed.ncbi.nlm.nih.gov/31363809/

Busse PJ, Christiansen SC. Hereditary Angioedema. N Engl J Med. 2020 Mar 19;382(12):1136-1148. doi: 10.1056/NEJMra1808012. PMID: 32187470. https://pubmed.ncbi.nlm.nih.gov/32187470/ Longhurst HJ, Bork K. Hereditary angioedema: an update on causes, manifestations and treatment. Br J Hosp Med (Lond). 2019 Jul 2;80(7):391-398. doi: 10.12968/hmed.2019.80.7.391. PMID: 31283393. https://pubmed.ncbi.nlm.nih.gov/31283393/

Bindke G, Schorling K, Wieczorek D, Kapp A, Wedi B. Genderaspekt bei Angioödemen [Gender aspects in angioedema]. Hautarzt. 2019 Feb;70(2):92-100. German. doi: 10.1007/s00105-018-4347-7. PMID: 30627747.  https://pubmed.ncbi.nlm.nih.gov/30627747/

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. Epub 2014 Mar 17. PMID: 24635050.  https://pubmed.ncbi.nlm.nih.gov/24635050/

Bork, K..: Systematic review of the efficacy and safety of a purified, pasteurized C1 inhibitor concentrate for the treatment of patients with type I or II hereditary angioedema – Allergy Asthma Clin Immunol 2011  https://pubmed.ncbi.nlm.nih.gov/24635050/

Bork K, Machnig T, Wulff K, Witzke G, Prusty S, Hardt J. Clinical features of genetically characterized types of hereditary angioedema with normal C1 inhibitor: a systematic review of qualitative evidence. Orphanet J Rare Dis. 2020 Oct 15;15(1):289. doi: 10.1186/s13023-020-01570-x. PMID: 33059692; PMCID: PMC7559394.  https://pubmed.ncbi.nlm.nih.gov/33059692/

Lokalisation von Schwellungen

Rosi-Schumacher M, Shah SJ, Craig T, Goyal N. Clinical manifestations of hereditary angioedema and a systematic review of treatment options. Laryngoscope Investig Otolaryngol. 2021 Apr 3;6(3):394-403. doi: 10.1002/lio2.555. PMID: 34195359; PMCID: PMC8223449. https://pubmed.ncbi.nlm.nih.gov/34195359/

Azmy V, Brooks JP, Hsu FI. Clinical presentation of hereditary angioedema. Allergy Asthma Proc. 2020 Nov 1;41(Suppl 1):S18-S21. doi: 10.2500/aap.2020.41.200065. PMID: 33109320. https://pubmed.ncbi.nlm.nih.gov/33109320/

Zanichelli A, Farkas H, Bouillet L, Bara N, Germenis AE, Psarros F, Varga L, Andrási N, Boccon-Gibod I, Castiglioni Roffia M, Rutkowski M, Cancian M. The Global Registry for Hereditary Angioedema due to C1-Inhibitor Deficiency. Clin Rev Allergy Immunol. 2021 Aug;61(1):77-83. doi: 10.1007/s12016-021-08855-4. Epub 2021 Mar 31. PMID: 33791951; PMCID: PMC8282542 https://pubmed.ncbi.nlm.nih.gov/33791951/

Studien zum Krankheitsbild

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 Jul;112(1):58-64. doi: 10.1016/j.tripleo.2011.02.034. Epub 2011 May 20. PMID: 21601496 https://pubmed.ncbi.nlm.nih.gov/21601496/

Bork K et al., Aszites und vermeintliches akutes Abdomen bei hereditärem Angioödem durch C1-Inhibitor-Mangel, DO - 10.1055/s-2008-1047770, Deutsche Medizinische Wochenschrift 1997, S.1347-1350 https://www.researchgate.net/publication/245675775_Aszites_und_vermeintliches_akutes_Abdomen_bei_hereditarem_Angioodem_durch_C1-Inhibitor-Mangel

Bork K et al. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006; 119(3):267–74 https://pubmed.ncbi.nlm.nih.gov/16490473/ Bork K et al. 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; 112:58–64 https://pubmed.ncbi.nlm.nih.gov/21601496/ Farkas H. Management of upper airway edema caused by hereditary angioedema. Allergy Asthma Clin Immunol. 2010; 6(1):19  https://pubmed.ncbi.nlm.nih.gov/20667122/

Bork K et al. Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. J Am Dent Assoc. 2003; 134: 1088–1094 https://pubmed.ncbi.nlm.nih.gov/12956349/ Aygören-Pürsün E et al. Risk of angioedema following invasive or surgical procedures in HAE type I and II – the natural history. Allergy 2013; 68:1034–1039 https://pubmed.ncbi.nlm.nih.gov/23968383/

Farkas H. Management of upper airway edema caused by hereditary angioedema. Allergy Asthma Clin Immunol. 2010; 6(1):19  https://pubmed.ncbi.nlm.nih.gov/20667122/

Aygören-Pürsün E et al. Risk of angioedema following invasive or surgical procedures in HAE type I and II – the natural history. Allergy 2013; 68:1034–1039  https://pubmed.ncbi.nlm.nih.gov/23968383/

HAE: Verschiedene Typen und Differentialdiagnose

Pines JM, Poarch K, Hughes S. Recognition and Differential Diagnosis of Hereditary Angioedema in the Emergency Department. J Emerg Med. 2021 Jan;60(1):35-43. doi: 10.1016/j.jemermed.2020.09.044. Epub 2020 Nov 17. PMID: 33218838. https://pubmed.ncbi.nlm.nih.gov/33218838/

Bork K, Wulff K, Witzke G, Hardt J, Meinke P. Inheritance Pattern of Hereditary Angioedema Indicates Mutation-Dependent Selective Effects During Early Embryonic Development. J Allergy Clin Immunol Pract. 2022 Apr;10(4):1029-1037. doi: 10.1016/j.jaip.2021.11.022. Epub 2021 Dec 7. PMID: 34890828. https://pubmed.ncbi.nlm.nih.gov/34890828/

Leibovich-Nassi I, Reshef A. The Enigma of Prodromes in Hereditary Angioedema (HAE). Clin Rev Allergy Immunol. 2021 Aug;61(1):15-28. doi: 10.1007/s12016-021-08839-4. Epub 2021 Feb 3. PMID: 33534063. https://pubmed.ncbi.nlm.nih.gov/33534063/

Agostoni A, Cicardi M. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore). 1992 Jul;71(4):206-15. doi: 10.1097/00005792-199207000-00003. PMID: 1518394. https://pubmed.ncbi.nlm.nih.gov/1518394/

Riedl MA. Hereditary angioedema with normal C1-INH (HAE type III). J Allergy Clin Immunol Pract. 2013 Sep-Oct;1(5):427-32. doi: 10.1016/j.jaip.2013.06.004. Epub 2013 Aug 2. PMID: 24565612. https://pubmed.ncbi.nlm.nih.gov/24565612/

Bernstein JA, Cremonesi P, Hoffmann TK, Hollingsworth J. Angioedema in the emergency department: a practical guide to differential diagnosis and management. Int J Emerg Med. 2017 Dec;10(1):15. doi: 10.1186/s12245-017-0141-z. Epub 2017 Apr 13. PMID: 28405953; PMCID: PMC5389952. https://pubmed.ncbi.nlm.nih.gov/28405953/

Bezalel S, Mahlab-Guri K, Asher I, Werner B, Sthoeger ZM. Angiotensin-converting enzyme inhibitor-induced angioedema. Am J Med. 2015 Feb;128(2):120-5. doi: 10.1016/j.amjmed.2014.07.011. Epub 2014 Jul 21. PMID: 25058867. https://pubmed.ncbi.nlm.nih.gov/25058867/

Farkas H, Varga L, Széplaki G, Visy B, Harmat G, Bowen T. Management of hereditary angioedema in pediatric patients. Pediatrics. 2007 Sep;120(3):e713-22. doi: 10.1542/peds.2006-3303. Epub 2007 Aug 27. PMID: 17724112. https://pubmed.ncbi.nlm.nih.gov/17724112/

Faktor XII und Garadacimab

Cohn DM, Renné T. Targeting factor XIIa for therapeutic interference with hereditary angioedema. J Intern Med. 2024 Oct;296(4):311-326. doi: 10.1111/joim.20008. PMID: 39331688. https://pubmed.ncbi.nlm.nih.gov/39331688/

Craig TJ, Reshef A, Li HH, Jacobs JS, Bernstein JA, Farkas H, Yang WH, Stroes ESG, Ohsawa I, Tachdjian R, Manning ME, Lumry WR, Saguer IM, Aygören-Pürsün E, Ritchie B, Sussman GL, Anderson J, Kawahata K, Suzuki Y, Staubach P, Treudler R, Feuersenger H, Glassman F, Jacobs I, Magerl M. Efficacy and safety of garadacimab, a factor XIIa inhibitor for hereditary angioedema prevention (VANGUARD): a global, multicentre, randomised, double-blind, placebo-controlled, phase 3 trial. Lancet. 2023 Apr 1;401(10382):1079-1090. doi: 10.1016/S0140-6736(23)00350-1. Epub 2023 Feb 28. Erratum in: Lancet. 2023 Apr 15;401(10384):1266. doi: 10.1016/S0140-6736(23)00739-0. PMID: 36868261. https://pubmed.ncbi.nlm.nih.gov/36868261/

Reshef A, Hsu C, Katelaris CH, Li PH, Magerl M, Yamagami K, Guilarte M, Keith PK, Bernstein JA, Lawo JP, Shetty H, Pollen M, Wieman L, Craig TJ; VANGUARD Study Group. Long-term safety and efficacy of garadacimab for preventing hereditary angioedema attacks: Phase 3 open-label extension study. Allergy. 2025 Feb;80(2):545-556. doi: 10.1111/all.16351. Epub 2024 Oct 7. PMID: 39370961; PMCID: PMC11804312. https://pubmed.ncbi.nlm.nih.gov/39370961/

Staubach P, Tachdjian R, Li HH, Hakl R, Aygören-Pürsün E, Wieman L, Lawo JP, Craig TJ. Timing of Onset of Garadacimab for Preventing Hereditary Angioedema Attacks. Clin Exp Allergy. 2024 Dec;54(12):1020-1023 https://pubmed.ncbi.nlm.nih.gov/39353415/

Chong-Neto HJ. A narrative review of recent literature of the quality of life in hereditary angioedema patients. World Allergy Organ J. 2023 Mar 20;16(3) https://pubmed.ncbi.nlm.nih.gov/36994443/

Shi GH, Pisupati K, Parker JG, Corvari VJ, Payne CD, Xu W, Collins DS, De Felippis MR. Subcutaneous Injection Site Pain of Formulation Matrices. Pharm Res. 2021 May;38(5):779-793. doi: 10.1007/s11095-021-03047-3. Epub 2021 May 3. PMID: 33942212. https://pubmed.ncbi.nlm.nih.gov/33942212/

COMPACT-Studien (klinische Wirksamkeit und Verträglichkeit von Berinert^®

2000/3000

West JB, Poarch K, Lumry WR. Preventive Treatment of Hereditary Angioedema: A Review of Phase III Clinical Trial Data for Subcutaneous C1 Inhibitor and Relevance for Patient Management. Clin Ther. 2021 Dec 5:S0149-2918(21)00411-2. doi: 10.1016/j.clinthera.2021.10.008. Epub ahead of print. PMID: 34879971. https://pubmed.ncbi.nlm.nih.gov/34879971/

LevyDS, et al., Long-Term Efficacy of Subcutaneous C1 Inhibitor in Pediatric Patients with Hereditary Angioedema. Pediatr Allergy Immunol Pulmonol. 2020;33(3):136-141 https://pubmed.ncbi.nlm.nih.gov/32953229/

Levy DS, 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  https://pubmed.ncbi.nlm.nih.gov/32042283/

Lumry WR, Zuraw B, Cicardi M, Craig T, Anderson J, Banerji A, Bernstein JA, Caballero T, Farkas H, Gower RG, Keith PK, Levy DS, Li HH, Magerl M, Manning M, Riedl MA, Lawo JP, Prusty S, Machnig T, Longhurst H; on behalf of the COMPACT Investigators. Long-term health-related quality of life in patients treated with subcutaneous C1-inhibitor replacement therapy for the prevention of hereditary angioedema attacks: findings from the COMPACT open-label extension study. Orphanet J Rare Dis. 2021 Feb 15;16(1):86. doi: 10.1186/s13023-020-01658-4. PMID: 33588897; PMCID: PMC7885603. Bernstein JA, et al., Long-term safety and efficacy of subcutaneous C1-inhibitor in older patients with hereditary angioedema. Ann Allergy Asthma Immunol. 2020;125(3):334-340.e1 https://pubmed.ncbi.nlm.nih.gov/32445670/

Craig T, 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 https://pubmed.ncbi.nlm.nih.gov/30772477/

Craig T, et al., Treatment effect of switching from intravenous to subcutaneous C1-inhibitor for prevention of hereditary angioedema attacks: COMPACT subgroup findings. J Allergy Clin Immunol Pract. 2019;7(6):2035-2038  https://pubmed.ncbi.nlm.nih.gov/30660873/

Lumry WR, et al., Health-Related Quality of Life with Subcutaneous C1-Inhibitor for Prevention of Attacks of Hereditary Angioedema. J Allergy Clin Immunol Pract. 2018;6(5):1733-1741.e3 https://pubmed.ncbi.nlm.nih.gov/29391286/

Pawaskar D, et al., Population pharmacokinetics of subcutaneous C1-inhibitor for prevention of attacks in patients with hereditary angioedema. Clin Exp Allergy. 2018;48(10):1325-1332 https://pubmed.ncbi.nlm.nih.gov/29998524/

Longhurst H, et al., Prevention of Hereditary Angioedema Attacks with a Subcutaneous C1 Inhibitor. N Engl J Med. 2017;376(12):1131-1140.  https://pubmed.ncbi.nlm.nih.gov/28328347/

Klinische Wirksamkeit und Verträglichkeit von Berinert^® 500/1500

Feussner A et al., Biochemical comparison of four commercially available C1 esterase inhibitor concentrates for treatment of hereditary angioedema – Transfusion 2014 https://pubmed.ncbi.nlm.nih.gov/24805006/

Craig TJ et al., Treatment response after repeated administration of C1 esterase inhibitor for successive acute hereditary angioedema attacks. Allergy Asthma Proc. 2012 Jul-Aug;33(4):354-61. doi: 10.2500/aap.2012.33.3589. PMID: 22856636.  https://pubmed.ncbi.nlm.nih.gov/22856636/

Craig, T. J.: Prospective Study of Rapid Relief Provided by C1 Esterase Inhibitor in Emergency Treatment of Acute Laryngeal Attacks in Hereditary Angioedema – J Clin Immunol 2012 https://pubmed.ncbi.nlm.nih.gov/20635155/

Craig, T. J.: C1-esterase inhibitor concentrate in 1085 Hereditary Angioedema attacks – final results of the I.M.P.A.C.T. 2 study – Eur J Allergy Clin Immunol 2011  https://pubmed.ncbi.nlm.nih.gov/21884533/

Bernstein JA et al., Hereditary angioedema: Validation of the end point time to onset of relief by correlation with symptom intensity. Allergy Asthma Proc. 2011 Jan-Feb;32(1):36-42. doi: 10.2500/aap.2011.32.3404. PMID: 21262096  https://pubmed.ncbi.nlm.nih.gov/21262096/

Craig TJ et al., Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks. J Allergy Clin Immunol. 2009 Oct;124(4):801-8. doi: 10.1016/j.jaci.2009.07.017. Epub 2009 Sep 19. PMID: 19767078  https://pubmed.ncbi.nlm.nih.gov/19767078/

Mit Zusatzentgelt für Kliniken: Hereditäres Angioödem effektiv behandeln, BLICKPUNKT MEDIZIN zur Zeitschrift AINS, 56. Jahrgang, Heft 7, Juli 2021  https://lp.thieme.de/emag/CP/Blickpunkt-Medizin-Berinert-2021

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 Apr;50(4):567-80.e1. doi: 10.1016/j.jemermed.2015.11.008. Epub 2016 Jan 27. PMID: 26826769. https://pubmed.ncbi.nlm.nih.gov/26826769/

Berinert^® Register

Riedl MA, Bygum A, Lumry W, Magerl M, Bernstein JA, Busse P, Craig T, Frank MM, Edelman J, Williams-Herman D, Feuersenger H, Rojavin M; Berinert Registry investigators. Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data. J Allergy Clin Immunol Pract. 2016 Sep-Oct;4(5):963-71. doi: 10.1016/j.jaip.2016.04.018. Epub 2016 Jun 8. PMID: 27286778. https://pubmed.ncbi.nlm.nih.gov/27286778/

Fox J, Vegh AB, Martinez-Saguer I, Wuillemin WA, Edelman J, Williams-Herman D, Rojavin M, Rosenberg T. Safety of a C1-inhibitor concentrate in pregnant women with hereditary angioedema. Allergy Asthma Proc. 2017 May 1;38(3):216-221. doi: 10.2500/aap.2017.38.4038. PMID: 28441992.  https://pubmed.ncbi.nlm.nih.gov/28441992/

Magerl M, Frank M, Lumry W, Bernstein J, Busse P, Craig T, Martinez-Saguer I, Riedl MA, Shapiro R, Edelman J, Williams-Herman D, Wood DN, Feuersenger H, Rojavin M; Berinert Registry Investigators. Short-term prophylactic use of C1-inhibitor concentrate in hereditary angioedema: Findings from an international patient registry. Ann Allergy Asthma Immunol. 2017 Jan;118(1):110-112. doi: 10.1016/j.anai.2016.10.006. Epub 2016 Nov 16. PMID: 27865714.  https://pubmed.ncbi.nlm.nih.gov/27865714/

Busse P, Baker J, Martinez-Saguer I, Bernstein JA, Craig T, Magerl M, Riedl M, Shapiro R, Frank M, Lumry W, Rosch J, Edelman J, Williams-Herman D, Feuersenger H, Rojavin M; Berinert Registry Investigators. Safety of C1-inhibitor concentrate use for hereditary angioedema in pediatric patients. J Allergy Clin Immunol Pract. 2017 Jul-Aug;5(4):1142-1145. doi: 10.1016/j.jaip.2016.12.033. Epub 2017 Mar 9. PMID: 28286153.  https://pubmed.ncbi.nlm.nih.gov/28286153/

Bygum A, Martinez-Saguer I, Bas M, Rosch J, Edelman J, Rojavin M, Williams-Herman D; Berinert Registry Investigators. Use of a C1 Inhibitor Concentrate in Adults ≥65 Years of Age with Hereditary Angioedema: Findings from the International Berinert^® (C1-INH) Registry. Drugs Aging. 2016 Nov;33(11):819-827. doi: 10.1007/s40266-016-0403-0. PMID: 27699634; PMCID: PMC5107191. https://pubmed.ncbi.nlm.nih.gov/27699634/

Busse P, Bygum A, Edelman J, Lumry W, Machnig T, Martinez-Saguer I, Rojavin M. Safety of C1-esterase inhibitor in acute and prophylactic therapy of hereditary angioedema: findings from the ongoing international Berinert patient registry. J Allergy Clin Immunol Pract. 2015 Mar-Apr;3(2):213-9. doi: 10.1016/j.jaip.2014.08.014. Epub 2014 Oct 29. PMID: 25609333. https://pubmed.ncbi.nlm.nih.gov/25609333/

Heimselbstbehandlung

Zanichelli, A. et al., Safety, effectiveness, and impact on quality of life of self-administration with plasma-derived nanofiltered C1 inhibitor (Berinert^®) in patients with hereditary angioedema: the SABHA study. Orphanet J Rare Dis 13, 51 (2018)  https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0797-3#citeas

Riedl MA et al., Safety and Usage of C1-Inhibitor in Hereditary Angioedema: Berinert Registry Data. J Allergy Clin Immunol Pract. 2016 Sep-Oct;4(5):963-71. doi: 10.1016/j.jaip.2016.04.018. Epub 2016 Jun 8. PMID: 27286778.  https://pubmed.ncbi.nlm.nih.gov/27286778/

Craig TJ et al. Effect of time to treatment on response to C1 esterase inhibitor concentrate for hereditary angioedema attacks. Ann Allergy Asthma Immunol. 2013;111(3):211-5. https://pubmed.ncbi.nlm.nih.gov/23987198/

Kreuz W et al., Home therapy with intravenous human C1-inhibitor in children and adolescents with hereditary angioedema. Transfusion. 2012 Jan;52(1):100-7. doi: 10.1111/j.1537-2995.2011.03240.x. Epub 2011 Jul 14. PMID: 21756262  https://pubmed.ncbi.nlm.nih.gov/21756262/

Longhurst HJ et al., HAE international home therapy consensus document. Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):22. doi: 10.1186/1710-1492-6-22. PMID: 20667125; PMCID: PMC2922091 https://pubmed.ncbi.nlm.nih.gov/20667125/

Aygören-Pürsün E et al., On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience. Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):21. doi: 10.1186/1710-1492-6-21. PMID: 20667124; PMCID: PMC2920239  https://pubmed.ncbi.nlm.nih.gov/20667124/

HAE bei Kindern und Jugendlichen

Aygören-Pürsün, Andarawewa, Hereditary angioedema with C1-inhibitor defciency (HAE-C1-INH) in childhood and adolescence Allergy, Asthma and Clinical Immunology 2019 15 Supplement 4 Wahn V et al. Hereditary angioedema in children and adolescents – A consensus update on therapeutic strategies for German‐speaking countries, Pediatr Allergy Immunol. 2020;31:974–989 https://onlinelibrary.wiley.com/doi/full/10.1111/pai.13309

Wahn V., Hereditäres Angioödem - Neue Nomenklatur, neue genetische Ursachen, neue Therapieoptionen, Pädiatrische Allergologie 03 / 2020, S.45-47 https://www.gpau.de/fileadmin/user_upload/GPA/dateien_indiziert/Sonstiges/Paed_Allergologie_2020_3_Immundef_34.pdf

Levy DS, et al., Long-Term Efficacy of Subcutaneous C1 Inhibitor in Pediatric Patients with Hereditary Angioedema. Pediatr Allergy Immunol Pulmonol. 2020;33(3):136-141 https://pubmed.ncbi.nlm.nih.gov/32953229/

Neue Therapieempfehlungen für Kinder und Jugendliche, Monatsschrift Kinderheilkunde, Band 168, Heft 12, Dezember 2020, Springer Medizin Verlag GmbH  (PDF zum Download)

Farkas H. Pediatric hereditary angioedema due to C1-inhibitor deficiency. Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):18. doi: 10.1186/1710-1492-6-18. PMID: 20667121; PMCID: PMC2920237 https://pubmed.ncbi.nlm.nih.gov/20667121/

Aygören-Pürsün E, Soteres DF, Nieto-Martinez SA, Christensen J, Jacobson KW, Moldovan D, Van Leerberghe A, Tang Y, Lu P, Vardi M, Schranz J, Martinez-Saguer I. A randomized trial of human C1 inhibitor prophylaxis in children with hereditary angioedema. Pediatr Allergy Immunol. 2019 Aug;30(5):553-561. doi: 10.1111/pai.13060. Epub 2019 May 29. PMID: 30968444; PMCID: PMC6851661. https://pubmed.ncbi.nlm.nih.gov/30968444/

Martinez-Saguer I, Farkas H. Erythema Marginatum as an Early Symptom of Hereditary Angioedema: Case Report of 2 Newborns. Pediatrics. 2016 Feb;137(2):e20152411. doi: 10.1542/peds.2015-2411. Epub 2016 Jan 12. PMID: 26759410.  https://pubmed.ncbi.nlm.nih.gov/26759410/

HAE in der Schwangerschaft und Stillzeit

Patel AS et al., Hereditary angioedema and pregnancy complications and outcomes in a population-based cohort, Presented at the American Academy of Allergy Asthma & Immunology (AAAAI) 2020 Annual Meeting, Philadelphia, Pennsylvania, USA; March 13-16, 2020 https://education.aaaai.org/sites/default/files/325%20Patel.pdf

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 Aug;203(2):131.e1-7. doi: 10.1016/j.ajog.2010.03.003. Epub 2010 May 14. PMID: 20471627.  https://pubmed.ncbi.nlm.nih.gov/20471627/

Hereditäres Angioödem – Erfolgreiches Krankheitsmanagement in der Schwangerschaft (Literaturarbeit mit Interview) , Allergo Journal 4/2021 Medizin Report aktuell Nr. 495693, Springer Medizin Verlag GmbH

Riedl MA. Hereditary Angioedema During Pregnancy: Considerations in Management. Immunol Allergy Clin North Am. 2023 Feb;43(1):145-157. doi: 10.1016/j.iac.2022.05.011. Epub 2022 Oct 28. PMID: 36411000. https://pubmed.ncbi.nlm.nih.gov/36411000/

HAE bei älteren Patienten

Bernstein JA, et al., Long-term safety and efficacy of subcutaneous C1-inhibitor in older patients with hereditary angioedema. Ann Allergy Asthma Immunol. 2020;125(3):334-340.e1 https://pubmed.ncbi.nlm.nih.gov/32445670/

Bouillet L. Hereditary angioedema in women. Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):17. doi: 10.1186/1710-1492-6-17. PMID: 20667120; PMCID: PMC2918592 https://pubmed.ncbi.nlm.nih.gov/20667120/

Lebensqualität

Prior N 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 Jul 20;10:82. doi: 10.1186/1477-7525-10-82. PMID: 22817696; PMCID: PMC3489868 https://pubmed.ncbi.nlm.nih.gov/22817696/

Magerl M, Buttgereit T, Martinez-Saguer I, Staubach-Renz P, Greve J, Schauf L, Schön K, Towards Complete Control in HAE: A Comprehensive Analysis of Quality of Life and Gaps in Achieving Normalized Living - Key Findings from a 2024 German Online Patient Survey, Poster 2024

HAE mit normalem C1-Inhibitor (HAE Typ III)

Bork K, Wulff K, Möhl BS, Steinmüller-Magin L, Witzke G, Hardt J, Meinke P. Novel hereditary angioedema linked with a heparan sulfate 3-O-sulfotransferase 6 gene mutation. J Allergy Clin Immunol. 2021 Jan 25:S0091-6749(21)00094-4. doi: 10.1016/j.jaci.2021.01.011. Epub ahead of print. PMID: 33508266. https://pubmed.ncbi.nlm.nih.gov/33508266/

Bork K, Wulff K, Witzke G, Machnig T, Hardt J. Treatment of patients with hereditary angioedema with the c.988A>G (p.Lys330Glu) variant in the plasminogen gene. Orphanet J Rare Dis. 2020 Feb 17;15(1):52. doi: 10.1186/s13023-020-1334-8. PMID: 32066472; PMCID: PMC7026952. https://pubmed.ncbi.nlm.nih.gov/32066472/

Bork K, Zibat A, Ferrari DM, Wollnik B, Schön MP, Wulff K, Lippert U. Hereditary angioedema in a single family with specific mutations in both plasminogen and SERPING1 genes. J Dtsch Dermatol Ges. 2020 Mar;18(3):215-223. doi: 10.1111/ddg.14036. Epub 2020 Feb 17. PMID: 32065705. https://pubmed.ncbi.nlm.nih.gov/32065705/

Bork K, Wulff K, Steinmüller-Magin L, Braenne I, Staubach-Renz P, Witzke G, Hardt J. Hereditary angioedema with a mutation in the plasminogen gene. Allergy. 2018 Feb;73(2):442-450. doi: 10.1111/all.13270. Epub 2017 Sep 7. Erratum in: Allergy. 2018 Dec;73(12):2412. PMID: 28795768. https://pubmed.ncbi.nlm.nih.gov/28795768/

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 Feb;72(2):320-324. doi: 10.1111/all.13076. Epub 2016 Dec 1. PMID: 27905115. https://pubmed.ncbi.nlm.nih.gov/27905115/

Ariano A, D'Apolito M, Bova M, Bellanti F, Loffredo S, D'Andrea G, Intrieri M, Petraroli A, Maffione AB, Spadaro G, Santacroce R, Margaglione M. A myoferlin gain-of-function variant associates with a new type of hereditary angioedema. Allergy. 2020 Nov;75(11):2989-2992. doi: 10.1111/all.14454. Epub 2020 Jul 1. PMID: 32542751. https://pubmed.ncbi.nlm.nih.gov/32542751/

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 Jul;124(1):129-34. doi: 10.1016/j.jaci.2009.03.038. Epub 2009 May 27. PMID: 19477491. https://pubmed.ncbi.nlm.nih.gov/19477491/

Bafunno V, Firinu D, D'Apolito M, Cordisco G, Loffredo S, Leccese A, Bova M, Barca MP, Santacroce R, Cicardi M, Del Giacco S, Margaglione M. Mutation of the angiopoietin-1 gene (ANGPT1) associates with a new type of hereditary angioedema. J Allergy Clin Immunol. 2018 Mar;141(3):1009-1017. doi: 10.1016/j.jaci.2017.05.020. Epub 2017 Jun 8. PMID: 28601681. https://pubmed.ncbi.nlm.nih.gov/28601681/ Bork K, Barnstedt SE, Koch P, Traupe H. Hereditary angioedema with normal C1-inhibitor activity in women. Lancet. 2000 Jul 15;356(9225):213-7. doi: 10.1016/S0140-6736(00)02483-1. PMID: 10963200. https://pubmed.ncbi.nlm.nih.gov/10963200/

Literatur Update

An dieser Stelle informieren Sie regelmäßog über die aktuelle Literatur zum Thema „HAE“. Damit sind Sie immer auf dem neuesten wissenschaftlichen Stand!

Dezember 2025

REVIEWS AND GUIDELINES

Delphi Consensus on Attenuated Androgen Use for Long-Term Prophylaxis in Hereditary Angioedema: AURA Project

Dias de Castro E, Cardoso LM, Jácome de Castro J, Ferreira MB. Delphi Consensus on Attenuated Androgen Use for Long-Term Prophylaxis in Hereditary Angioedema: AURA Project. Clin Transl Allergy. 2025 Nov;15(11):e70116. doi: 10.1002/clt2.70116. PMID: 41219138; PMCID: PMC12606019.

Impact of injectable HAE on-demand treatments on health-related quality of life: a patient and caregiver interview study

Yong PFK, Craig TJ, Busse PJ, Garcez T, Hall R, Lo SH, Dixon C, Audhya PK, Wang A, Banerji A, Kiani-Alikhan S.Allergy Asthma Clin Immunol. 2025 Nov 29. doi: 10.1186/s13223-025-00997-w. Online ahead of print.PMID: 41316316

Hereditary angioedema: A national investigation of associated comorbidities and surgical procedures

Voloncs-Mindszenthy L, Horváth HR, Andrási N, Szilágyi T, Farkas H.World Allergy Organ J. 2025 Nov 16;18(11):101136. doi: 10.1016/j.waojou.2025.101136. eCollection 2025 Nov.PMID: 41323126

Clinical presentation and management of 239 children and adolescents with hereditary angioedema

Arkwright PD, Dorr AD, Bernatoniene J, Hackett S, Buckland M, Patel SY, Riordan A, Jones L, O'Sullivan CP, Coutler T, González-Martinez C, El-Shanawany T, Noronha L, Warne E, Bhole M, Noorani S, Grammatikos A, Tan TH, Yong PFK.J Allergy Clin Immunol Pract. 2025 Nov;13(11):3152-3155.e1. doi: 10.1016/j.jaip.2025.07.029. Epub 2025 Jul 29.PMID: 40744376

Lanadelumab's impact on hereditary angioedema control and quality of life across disease activity subgroups: Real-world evidence

Zanichelli A, Wuillemin WA, Aygören-Pürsün E, Banerji A, Busse PJ, Betschel SD, Cancian M, Gagnon R, Goodyear MD, Kinaciyan T, Kessel A, Magerl M, Recke A, Wedner HJ, Estepan DN, Watt M, Andresen I, Juethner S, Khutoryansky N, Martinez-Saguer I.Ann Allergy Asthma Immunol. 2025 Nov;135(5):560-569.e2. doi: 10.1016/j.anai.2025.07.025. Epub 2025 Sep 6.PMID: 40769455

Clinical and genetic study in factor XII hereditary angioedema in a population from Southern Spain.

Mera TA, Baynova K, Soto JML, Lozano JRG, Cimbollek S.Ann Allergy Asthma Immunol. 2025 Nov 10:S1081-1206(25)01309-2. doi: 10.1016/j.anai.2025.11.003. Online ahead of print.

Sebetralstat for On-Demand Treatment of Mucosal Hereditary Angioedema Attacks in KONFIDENT-S

Bernstein JA, Aygören-Pürsün E, Cancian M, Cohn DM, Craig T, Grivcheva-Panovska V, Jordan A, Lumry WR, Martinez-Saguer I, Melamed I, Ohmura K, Peter J, Riedl MA, Soteres DF, Staubach P, Stobiecki M, Chuang YH, Smith MD, Yea CM, Audhya PK, Zanichelli A, Farkas H.Clin Transl Allergy. 2025 Nov;15(11):e70118. doi: 10.1002/clt2.70118.PMID: 41252457

Long-Term Safety and Effectiveness of Sebetralstat: Interim Analysis of KONFIDENT-S Open-label Extension

Farkas H, Anderson J, Bouillet L, Caballero T, Cancian M, Craig T, Fukunaga A, Grivcheva-Panovska V, Guilarte M, Honda D, Kanarek H, Kiani-Alikhan S, Kinaciyan T, Leguevaques D, Longhurst HJ, Magerl M, Manning ME, Martinez-Saguer I, Melamed I, O'Connor ME, Peter J, Savic S, Soteres DF, Staevska M, Staubach P, Stobiecki M, Tachdjian R, Valerieva A, Yong PFK, Hao J, Iverson M, Smith MD, Yea CM, Audhya PK, Aygören-Pürsün E, Bernstein JA, Cohn DM, Lumry WR, Riedl MA, Zanichelli A, Maurer M.J Allergy Clin Immunol Pract. 2025 Nov;13(11):3094-3103.e5. doi: 10.1016/j.jaip.2025.08.020. Epub 2025 Aug 29.PMID: 4088693

C1 esterase inhibitor (C1-INH) response as a supportive diagnostic criterion for patients with suspected hereditary angioedema with normal C1-IN

Smith AM, Kanarek HJ, Rumbyrt J, Hussain Y, Lim LM, Fatteh S, Memmott H, Chu T, Patel M, Rivera R, Kashkin JM, Jones DH.World Allergy Organ J. 2025 Oct 21;18(11):101125. doi: 10.1016/j.waojou.2025.101125. eCollection 2025 Nov.PMID: 41189715

Exposure-Response Analysis of Donidalorsen for the Treatment of Hereditary Angioedema

Singh P, Witjes H, Kleijn HJ, Diep JK, Bordone L, Newman KB, Gao X, Cohn DM.Clin Transl Sci. 2025 Nov;18(11):e70388. doi: 10.1111/cts.70388.PMID: 41208217

Insights From the First 820 Patients From the Brazilian Multicenter Registry of Hereditary Angioedema: The Key Role of Genetic Testing and Targeted Therapies

Ferriani MPL, Seneda Lemos JE, Arruda LK, Nunes FL, Dias MM, Kolarik ML, Roxo-Junior P, Ferraro MF, Zanetti MET, Serpa FS, Chong-Neto HJ, Minafra FG, Valle SOR, Campos RA, da Silva J, Mansour E, Gonçalves RF, Toledo EC, Marcelino FC, Moreira IF, Azoubel-Antunes A, Ferreira JFS, Dias GAC, Aun MV, Castro APBM, Oliva-Alonso ML, Batigalia VA, Tavares CLTSV, Capelo AV, Moyses TR, Rosário Filho NA, Jannuzzi LNP, Fragnan NTML, Figueiredo JP, Fonseca JT, Teixeira AJR, Nasser NMF, Ferraroni NR, Mendonça LO, Iwashita MT, Prado AIF, Tumelero MT, Machado JA, Kamoi TO, Ferrel LM, Barbosa AMSC, de Moraes CGFB, Kruk T, Figueiredo MR, Moraes MSY, Moreno AS, Maia LSM, Traina F, Ruy PC, Pesquero JB, Bork K, Cichon S, Aragon DC, Giavina-Bianchi P, Grumach AS.J Allergy Clin Immunol Pract. 2025 Nov;13(11):3104-3117. doi: 10.1016/j.jaip.2025.08.026. Epub 2025 Sep 3.PMID: 40912615

CASE REPORTS

Abdominal Pain Attacks and Angioedema in a Patient With a Homozygous MEFV Mutation: Concurrent FXII Hereditary Angioedema

Bulut G, Camyar A, Gokmen NM.J Investig Allergol Clin Immunol. 2025 Nov 24:0. doi: 10.18176/jiaci.1115. Online ahead of print.PMID: 41283899

Implementation of genetic diagnosis and personalized management of hereditary angioedema in a Chinese regional center: a community case study of three families

Du W, Meng Z, Yang K, Zhang Q, Lin X, Zhang W, Guo W, Wang S.Front Allergy. 2025 Nov 12;6:1696666. doi: 10.3389/falgy.2025.1696666. eCollection 2025.PMID: 41312112

Unexpected genomic architecture in a sporadic case of C1-INH Hereditary Angioedema: the hidden heritability

Marchionni E, Mannucci L, Di Tommaso S, Nardone AM, Albanese M, Novelli A, Sangiuolo FC, Triggianese P, Novelli G.Orphanet J Rare Dis. 2025 Nov 5;20(1):562. doi: 10.1186/s13023-025-04092-6.PMID: 41194199

November 2025

REVIEWS AND GUIDELINES

Quality of life and burden of disease in patients with hereditary angioedema and their caregivers

Levy DS, Nagase FI, Cheung A, Rodeberg DA, Manning ME. Quality of life and burden of disease in patients with hereditary angioedema and their caregivers. Allergy Asthma Proc. 2025 Sep 1;46(5):398-405. doi: 10.2500/aap.2025.46.250048. PMID: 40958183; PMCID: PMC12419976.

Status quo and future developments in the diagnosis and treatment of hereditary angioedema

Recke A. Status quo and future developments in the diagnosis and treatment of hereditary angioedema. J Dtsch Dermatol Ges. 2025 Sep 4. doi: 10.1111/ddg.15889. Epub ahead of print. PMID: 40905425.

C1 inhibitor: from complement system to bradykinin angioedema

Defendi F, Amen A, Clavarino G, Dumestre-Pérard C. C1 inhibitor: from complement system to bradykinin angioedema. Curr Opin Immunol. 2025 Dec;97:102653. doi: 10.1016/j.coi.2025.102653. Epub 2025 Sep 3. PMID: 40907118.

CLINICAL STUDIES

Garadacimab in hereditary angioedema due to normal C1INH with F12/PLG mutations

Cohn DM, Reshef A, Staubach P, Lumry WR, Feuersenger H, Jacobs I, Pragst I, Zuraw B, Bork K. Garadacimab in hereditary angioedema due to normal C1INH with F12/PLG mutations. J Allergy Clin Immunol. 2025 Aug 26:S0091-6749(25)00886-3. doi: 10.1016/j.jaci.2025.08.005. Epub ahead of print. PMID: 40876763.

A Phase 1 Randomized Study: Garadacimab Pharmacokinetics, Safety, and Tolerability After Administration via Autoinjector/Pre-Filled Pen Versus Pre-Filled Syringe in Healthy Participants

Glassman F, Lawo JP, Bica MA, Roberts A, Kormann D, Chialda L, Miethke S, Dziadowiec I, Caltabiano S, Puchalski T. A Phase 1 Randomized Study: Garadacimab Pharmacokinetics, Safety, and Tolerability After Administration via Autoinjector/Pre-Filled Pen Versus Pre-Filled Syringe in Healthy Participants. J Clin Pharmacol. 2025 Sep 6. doi: 10.1002/jcph.70099. Epub ahead of print. PMID: 40913454.

Acquired Angioedema Related to Transient C1- Inhibitor Deficiency Triggered by Mycoplasma pneumoniae Infection: A Case Report

Peristeri AM, Akritidou O, Nikopoulou A, Theodoridou K, Leontakianakos M, Theocharidou CC, Pilianidis G. Acquired Angioedema Related to Transient C1- Inhibitor Deficiency Triggered by Mycoplasma pneumoniae Infection: A Case Report. Reports (MDPI). 2025 Sep 1;8(3):164. doi: 10.3390/reports8030164. PMID: 40981122; PMCID: PMC12452343.

Pediatric angioedema without urticaria: A rare presentation of autoimmune thyroid disease with complete remission on levothyroxine therapy

Archana MH, Shivane V, Yamichannaiah C, Memon SS. Pediatric angioedema without urticaria: A rare presentation of autoimmune thyroid disease with complete remission on levothyroxine therapy. J Postgrad Med. 2025 Jul 1;71(3):139-142. doi: 10.4103/jpgm.jpgm_211_25. Epub 2025 Sep 18. PMID: 40965010; PMCID: PMC12534102.

September 2025 II

REVIEWS AND GUIDELINES

ANDEMBRY (garadacimab-gxii)

Beninger P. ANDEMBRY (garadacimab-gxii). Clin Ther. 2025 Sep;47(9):823-824. doi: 10.1016/j.clinthera.2025.07.020. Epub 2025 Aug 20. PMID: 40841306.

Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature

Cardenas Rosales JC, Ridwan A, Ruiz-Orasma C, Galeano-Lovera SF, Gil Lopez F, Perusina J, Squire JD, Jiang L, Moustafa MA, Harris DM, Iftikhar S, Munipalli B. Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature. J Prim Care Community Health. 2025 Jan-Dec;16:21501319251360500. doi: 10.1177/21501319251360500. Epub 2025 Jul 31. PMID: 40741790; PMCID: PMC12317231.

Evaluating large language models for WAO/EAACI guideline compliance in hereditary angioedema management.

Gerek ME, Önalan T, Çölkesen F, Arslan Ş. Evaluating large language models for WAO/EAACI guideline compliance in hereditary angioedema management. Allergol Immunopathol (Madr). 2025 Jul 1;53(4):51-59. doi: 10.15586/aei.v53i4.1353. PMID: 40682228.

CLINICAL STUDIES

Expanding the Genetic and Clinical Spectrum of Hereditary Angioedema with Normal C1 Inhibitor: Novel Variants and Treatment Insights.

Gao H, Zhao Y, Chen S, Zhang Z, Yang F, Chen Z, Wang L, Yang J, He S, Tang C, Zheng S, Guan C, Xu Y, Tang L, Zhang A, Maurer M, Lee D, Ma L, Luo X. Expanding the Genetic and Clinical Spectrum of Hereditary Angioedema with Normal C1 Inhibitor: Novel Variants and Treatment Insights. J Clin Immunol. 2025 Aug 23;45(1):124. doi: 10.1007/s10875-025-01912-z. PMID: 40848077; PMCID: PMC12374894.

Matching-adjusted indirect comparison between garadacimab and lanadelumab for the long-term prophylactic treatment of patients with hereditary angioedema.

Walsh S, Haltner A, Bartlett M, Sears J, Li Y, Kelly M, Gavata-Steiger S, Nenci C, Jacobs I, Pragst I, Ray N, Samjoo IA. Matching-adjusted indirect comparison between garadacimab and lanadelumab for the long-term prophylactic treatment of patients with hereditary angioedema. J Comp Eff Res. 2025 Aug;14(8):e240237. doi: 10.57264/cer-2024-0237. Epub 2025 Jul 10. PMID: 40637159; PMCID: PMC12308543.

Comment on: "A national survey of four decades of hereditary angioedema prophylaxis" - The emerging role of Garadacimab.

Afridi H, Afzal K. Comment on: "A national survey of four decades of hereditary angioedema prophylaxis" - The emerging role of Garadacimab. Clin Immunol. 2025 Oct;279:110547. doi: 10.1016/j.clim.2025.110547. Epub 2025 Jun 23. PMID: 40562155.

Lanadelumab's impact on hereditary angioedema control and quality of life across disease activity subgroups: real-world evidence.

Zanichelli A, Wuillemin WA, Aygören-Pürsün E, Banerji A, Busse PJ, Betschel SD, Cancian M, Gagnon R, Goodyear MD, Kinaciyan T, Kessel A, Magerl M, Recke A, Wedner HJ, Estepan DN, Watt M, Andresen I, Juethner S, Khutoryansky N, Martinez-Saguer I. Lanadelumab's impact on hereditary angioedema control and quality of life across disease activity subgroups: Real-world evidence. Ann Allergy Asthma Immunol. 2025 Sep 5:S1081-1206(25)00393-X. doi: 10.1016/j.anai.2025.07.025. Epub ahead of print. PMID: 40769455.

Disease control and disease activity in hereditary angioedema: two sides of the same coin?

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 Jul 22;16:1631448. doi: 10.3389/fimmu.2025.1631448. PMID: 40766317; PMCID: PMC12321560.

Impact of hereditary angioedema attacks on health-related quality of life and work productivity.

O'Connor M, Busse PJ, Craig TJ, Radojicic C, Wedner HJ, Danese S, Ulloa J, Desai V, Andriotti T, Audhya PK, Christiansen S. Impact of hereditary angioedema attacks on health-related quality of life and work productivity. World Allergy Organ J. 2025 Jul 28;18(8):101083. doi: 10.1016/j.waojou.2025.101083. PMID: 40761580; PMCID: PMC12320545.

Clinical presentation and management of 239 children and adolescents with hereditary angioedema.

Arkwright PD, Dorr AD, Bernatoniene J, Hackett S, Buckland M, Patel SY, Riordan A, Jones L, O'Sullivan CP, Coutler T, González-Martinez C, El-Shanawany T, Noronha L, Warne E, Bhole M, Noorani S, Grammatikos A, Tan TH, Yong PFK. Clinical presentation and management of 239 children and adolescents with hereditary angioedema. J Allergy Clin Immunol Pract. 2025 Jul 29:S2213-2198(25)00719-6. doi: 10.1016/j.jaip.2025.07.029. Epub ahead of print. PMID: 40744376.

Oral berotralstat for hereditary angioedema prophylaxis in patients aged 2 to <12 years: APeX-P interim results.

Bernatoniene J, Bourgoin-Heck M, Cancian M, Yang W, Hagin D, Pagnier A, Stobiecki M, Kinaciyan T, Phillips-Angles E, Gayet S, Bara NA, Hunter J, Mateescu E, DeSpirito M, Johnston D, Long D, Iocca H, Petroni D, Aygören-Pürsün E. Oral berotralstat for hereditary angioedema prophylaxis in patients aged 2 to <12 years: APeX-P interim results. Ann Allergy Asthma Immunol. 2025 Jul 25:S1081-1206(25)00352-7. doi: 10.1016/j.anai.2025.07.012. Epub ahead of print. PMID: 40716738.

Characteristics and Comorbidities Influencing Mortality Risk Among Hereditary Angioedema Patients.

Khalid S, Hitch AT. Characteristics and Comorbidities Influencing Mortality Risk Among Hereditary Angioedema Patients. J Health Econ Outcomes Res. 2025 Jul 17;12(2):11-20. doi: 10.36469/001c.141747. PMID: 40687186; PMCID: PMC12276015.

Activation of the Complement/Lectin Pathway, Angiopoietin/Tie-2/VEGF-System, Cytokines and Chemokines in Different Angioedema Subtypes.

Duda KM, Gehring M, Wedi B. Activation of the Complement/Lectin Pathway, Angiopoietin/Tie-2/VEGF-System, Cytokines and Chemokines in Different Angioedema Subtypes. Eur J Immunol. 2025 Jul;55(7):e70010. doi: 10.1002/eji.70010. PMID: 40685762; PMCID: PMC12277871.

A national survey of four decades of hereditary angioedema prophylaxis: Efficacy and safety of old and new drugs.

Horváth HR, Visy B, Kőhalmi KV, Balla Z, Andrási N, Czaller I, Zotter Z, Farkas H. A national survey of four decades of hereditary angioedema prophylaxis: Efficacy and safety of old and new drugs. Clin Immunol. 2025 Oct;279:110542. doi: 10.1016/j.clim.2025.110542. Epub 2025 Jun 11. PMID: 40513629.

Global frequency, diagnosis, and treatment of hereditary angioedema with normal C1 inhibitor.

Magerl M, Riedl MA, Arruda LK, Bauer A, Berardi A, Bernstein JA, Bouillet L, Buckland M, Buttgereit T, Cohn DM, Craig T, Criado RF, Du-Thanh A, Fain O, Gonçalo M, Greve J, Grumach AS, Guilarte M, Katelaris C, Kinaciyan T, Latysheva EA, Lleonart R, Llosa OC, Mansour E, Grivcheva-Panovska V, Parisi C, Rosario Filho NA, Santos AS, Staubach P, Valerieva A, Rodrigues Valle SO, Danese S, Ulloa J, Audhya PK, Maurer M. Global frequency, diagnosis, and treatment of hereditary angioedema with normal C1 inhibitor. J Allergy Clin Immunol Glob. 2025 Feb 27;4(3):100446. doi: 10.1016/j.jacig.2025.100446. PMID: 40276547; PMCID: PMC12020835.

Patient-Reported Outcomes in the Phase III OASIS-HAE Study of Donidalorsen for Hereditary Angioedema.

Riedl MA, Yarlas A, Bordone L, Treadwell S, Wang S, Newman KB, Cohn DM. Patient-Reported Outcomes in the Phase III OASIS-HAE Study of Donidalorsen for Hereditary Angioedema. Allergy. 2025 Aug;80(8):2361-2368. doi: 10.1111/all.16563. Epub 2025 Apr 19. PMID: 40251904; PMCID: PMC12368753.

CASE REPORTS

Recurrent angioedema manifestation and treatment response in two patients from different families caring the myoferlin gene mutation: case series.

Fomina DS, Lebedkina MS, Bobrikova EN, Yukhnovskaya YD, Roppelt AA, Mukhina OA, Markina UA, Alexeeva YG, Nikitina EA, Maurer M, Karaulov AV, Lysenko MA, Buttgereit T. Recurrent angioedema manifestation and treatment response in two patients from different families caring the myoferlin gene mutation: case series. Orphanet J Rare Dis. 2025 Aug 12;20(1):424. doi: 10.1186/s13023-025-03932-9. PMID: 40797221; PMCID: PMC12344933.

September 2025 I

REVIEWS AND GUIDELINES

Hereditary angioedema diagnosis: Reflecting on the past, envisioning the future.

Grumach AS, Riedl MA, Cheng L, Jain S, Nova Estepan D, Zanichelli A, Hereditary angioedema diagnosis: Reflecting on the past, envisioning the future., World Allergy Organ J, 2025 Jun, PMID: 40487881, PMCID: PMC12142509, doi: 10.1016/j.waojou.2025.101060, PubStatus:

Network Meta-Analysis of Pharmacological Therapies for Long-Term Prophylactic Treatment of Patients with Hereditary Angioedema.

Walsh S, Bartlett M, Salvo-Halloran EM, Sears J, Li Y, Kelly M, Gavata-Steiger S, Nenci C, Jacobs I, Pragst I, Ray N, Samjoo IA, Network Meta-Analysis of Pharmacological Therapies for Long-Term Prophylactic Treatment of Patients with Hereditary Angioedema., Drugs R D, 2025 May 28, PMID: 40434599, doi: 10.1007/s40268-025-00511-y, PubStatus:a head of print

The potential of factor XII inhibitors in preventing hereditary angioedema attacks.

Hooi JKY, Wong JCY, Li PH, The potential of factor XII inhibitors in preventing hereditary angioedema attacks., Expert Opin Biol Ther, 2025 May 26, PMID: 40411772, doi: 10.1080/14712598.2025.2512128, PubStatus:a head of print

Angioedema without urticaria: Diagnosis and management.

Young MC, Banerji A, Angioedema without urticaria: Diagnosis and management., Allergy Asthma Proc, 2025 May 1, PMID: 40380367, doi: 10.2500/aap.2025.46.250013, PubStatus:ppublish

From burden to breakthrough: Advances in hereditary angioedema, drug allergy, and allergic disease prevention.

Bellanti JA, Settipane RA, From burden to breakthrough: Advances in hereditary angioedema, drug allergy, and allergic disease prevention., Allergy Asthma Proc, 2025 May 1, PMID: 40380358, PMCID: PMC12118138, doi: 10.2500/aap.2025.46.250030, PubStatus:ppublish

From Anxiety to Work Productivity and Activity Impairment: The Mediating Role of Fatigue in Hereditary Angioedema.

Mak HWF, Wong JCY, Chiang V, Lam DLY, Li PH, From Anxiety to Work Productivity and Activity Impairment: The Mediating Role of Fatigue in Hereditary Angioedema., Clin Exp Allergy, 2025 Jun, PMID: 39861951, PMCID: PMC12127057, doi: 10.1111/cea.14632, PubStatus:ppublish+epublish

FDA approves first factor XIIa inhibitor, for hereditary angioedema.

Mullard A, FDA approves first factor XIIa inhibitor, for hereditary angioedema., Nat Rev Drug Discov, 2025 Aug, PMID: 40579442, doi: 10.1038/d41573-025-00114-x, PubStatus:ppublish

Incidental findings related to genes associated to HAE-nC1INH: how to proceed?

Germenis AE, Sanoudou D, Incidental findings related to genes associated to HAE-nC1INH: how to proceed?, Front Immunol, 2025, PMID: 40599776, PMCID: PMC12208851, doi: 10.3389/fimmu.2025.1605727, PubStatus:

Dominant inheritance in hereditary angioedema associated with carboxypeptidase N deficiency.

Hida T, Idogawa M, Okura M, Tokino T, Uhara H, Dominant inheritance in hereditary angioedema associated with carboxypeptidase N deficiency., Allergol Int, 2025 Jul, PMID: 40175265, doi: 10.1016/j.alit.2025.01.002, PubStatus:ppublish+epublish

Exercise-Induced Angioedema, Urticaria, and Anaphylaxis-A Narrative Review.

Aman Ur Rahman W, Mortada MM, Ślimok P, Sherri A, Poznańska-Kurowska K, Zalewska-Janowska A, Kurowski M, Exercise-Induced Angioedema, Urticaria, and Anaphylaxis-A Narrative Review., Sports (Basel), 2025 Jul 3, PMID: 40711100, PMCID: PMC12300403, doi: 10.3390/sports13070215, PubStatus:epublish

CLINICAL STUDIES

Th2 predominance and decreased NK cells in patients with hereditary angioedema.

Sundler Björkman L, Elmér E, Egesten A, Skattum L, Th2 predominance and decreased NK cells in patients with hereditary angioedema., Front Immunol, 2025, PMID: 40438097, PMCID: PMC12116338, doi: 10.3389/fimmu.2025.1536128, PubStatus:

Impact of hereditary angioedema attacks on health-related quality of life and work productivity.

O'Connor M, Busse PJ, Craig TJ, Radojicic C, Wedner HJ, Danese S, Ulloa J, Desai V, Andriotti T, Audhya PK, Christiansen S, Impact of hereditary angioedema attacks on health-related quality of life and work productivity., World Allergy Organ J, 2025 Aug, PMID: 40761580, PMCID: PMC12320545, doi: 10.1016/j.waojou.2025.101083, PubStatus:

Unmet needs in hereditary angioedema: an international survey of physicians.

Buttgereit T, Aulenbacher F, Adatia A, Ayala CV, Al-Nesf MA, Altrichter S, Abuzakouk M, Al-Ahmad M, Ali RM, Berardi A, Boccon-Gibod I, Bouillet L, Brussino L, Barešić M, Busse PJ, Betschel SD, Chong-Neto H, Llosa OC, Craig TJ, Dorr AD, Junior SDD, Fomina D, Farkas H, Fok JS, Grumach AS, Greve J, Guilarte M, Gonçalo M, Grivcheva-Panovska V, Hide M, Hakl R, Jindal A, Katelaris CH, Kamaleswaran S, Kinaciyan T, Latysheva E, Sousa JIL, Bellfill RL, Mobayed H, Metz M, Nasr I, Mitrevska NT, Nicola S, Parisi CAS, Porebski G, Peter J, Ferriani MPL, Filho NR, Şekerel BE, Serpa FS, Stobiecki M, Trainotti S, Valerieva A, Wongsa C, Wong JCY, Yucel E, Li Y, Nenci C, Maurer M, Magerl M, Li PH, Unmet needs in hereditary angioedema: an international survey of physicians., Orphanet J Rare Dis, 2025 Jul 28, PMID: 40722187, PMCID: PMC12306030, doi: 10.1186/s13023-025-03739-8, PubStatus:epublish

Matching-adjusted indirect comparison between garadacimab and lanadelumab for the long-term prophylactic treatment of patients with hereditary angioedema.

Walsh S, Haltner A, Bartlett M, Sears J, Li Y, Kelly M, Gavata-Steiger S, Nenci C, Jacobs I, Pragst I, Ray N, Samjoo IA, Matching-adjusted indirect comparison between garadacimab and lanadelumab for the long-term prophylactic treatment of patients with hereditary angioedema., J Comp Eff Res, 2025 Aug, PMID: 40637159, PMCID: PMC12308543, doi: 10.57264/cer-2024-0237, PubStatus:ppublish+epublish

Real-world evidence of the effectiveness and utilization of subcutaneous C1INH long-term prophylaxis in patients with HAE in Spain and Germany.

Maurer M, Cimbollek S, Kelly M, Rodney K, Elliott J, LoboGuerrero E, Magerl M, Real-world evidence of the effectiveness and utilization of subcutaneous C1INH long-term prophylaxis in patients with HAE in Spain and Germany., Front Immunol, 2025, PMID: 40438099, PMCID: PMC12116583, doi: 10.3389/fimmu.2025.1576235, PubStatus:

Elevated level of circulating VEGF in Chinese patients with hereditary angioedema and its correlation with disease status.

Ji R, Xu Y, Zhi Y, Elevated level of circulating VEGF in Chinese patients with hereditary angioedema and its correlation with disease status., Orphanet J Rare Dis, 2025 May 26, PMID: 40420190, PMCID: PMC12105160, doi: 10.1186/s13023-025-03776-3, PubStatus:epublish

Establishing a hereditary angioedema prevalence for the United States using a large administrative claims database.

Castaldo AJ, Wells KE, Khalid S, Rashidi E, Selva CN, Corcoran D, Christiansen SC, Riedl MA, Zuraw BL, Loyo-Berrios N, Establishing a hereditary angioedema prevalence for the United States using a large administrative claims database., Ann Allergy Asthma Immunol, 2025 May 23, PMID: 40414286, doi: 10.1016/j.anai.2025.05.018, PubStatus:a head of print

Factors contributing to non-compliance with on-demand treatment guidelines in hereditary angioedema.

Betschel SD, Peter J, Lumry W, Longhurst H, Katelaris CH, van Kooten S, Heckmann M, Malloy N, Ulloa J, Danese S, Magerl M, Factors contributing to non-compliance with on-demand treatment guidelines in hereditary angioedema., Allergy Asthma Clin Immunol, 2025 May 21, PMID: 40400016, PMCID: PMC12093744, doi: 10.1186/s13223-025-00969-0, PubStatus:epublish

A nationwide questionnaire-based surveillance on pediatric hereditary angioedema in Japan.

Yamamoto BA, Ohsawa I, Toriumi S, Matsuyama M, Saito T, Shimizu T, Inage E, A nationwide questionnaire-based surveillance on pediatric hereditary angioedema in Japan., Pediatr Int, 2025 Jan-Dec, PMID: 40390650, doi: 10.1111/ped.70077, PubStatus:ppublish

Can depression and anxiety be predicted in hereditary angioedema? A comprehensive assessment.

Akten HS, Dilek E, Orman M, Mete Gokmen EN, Can depression and anxiety be predicted in hereditary angioedema? A comprehensive assessment., Allergy Asthma Proc, 2025 May 1, PMID: 40380370, doi: 10.2500/aap.2025.46.250017, PubStatus:ppublish

Garadacimab improves long-term health-related quality of life in patients with hereditary angioedema.

Guilarte M, Lumry WR, Magerl M, Martinez Saguer I, Reshef A, Sobotkova M, Braverman J, Lawo JP, Wieman L, Nenci C, Katelaris CH, Garadacimab improves long-term health-related quality of life in patients with hereditary angioedema., Allergy Asthma Proc, 2025 May 1, PMID: 40380363, doi: 10.2500/aap.2025.46.250027, PubStatus:ppublish

Becoming attack-free further improves health-related quality of life in patients with hereditary angioedema receiving garadacimab.

Staubach P, Craig TJ, Fukuda T, Aygoren-Pursun E, Hakl R, Braverman J, Lawo JP, Pollen M, Nenci C, Li PH, Farkas H, Becoming attack-free further improves health-related quality of life in patients with hereditary angioedema receiving garadacimab., Allergy Asthma Proc, 2025 May 1, PMID: 40380356, doi: 10.2500/aap.2025.46.250026, PubStatus:ppublish

Hypertension risk in patients having hereditary angioedema with plasminogen gene mutation K330E.

Bork K, Wulff K, Meinke P, Machnig T, Hardt J, Hypertension risk in patients having hereditary angioedema with plasminogen gene mutation K330E., J Allergy Clin Immunol Pract, 2025 May 15, PMID: 40379246, doi: 10.1016/j.jaip.2025.05.017, PubStatus:a head of print

Assessment of potential drug-drug interactions in patients with hereditary angioedema from the ITACA cohort: simulations from a real-life dataset considering danazol versus berotralstat.

Zanichelli A, Cattaneo D, Gidaro A, Senter R, Arcoleo F, Accardo P, Bignardi D, Borrelli P, Colangelo C, De Pasquale T, Firinu D, Perego F, Triggiani M, Spadaro G, Cogliati C, Bizzi E, Popescu Janu V, Guarino MD, Quattrocchi P, Brussino L, Rossi O, Triggianese P, Agolini S, Giardino F, Montinaro V, Cancian M, Assessment of potential drug-drug interactions in patients with hereditary angioedema from the ITACA cohort: simulations from a real-life dataset considering danazol versus berotralstat., Front Pharmacol, 2025, PMID: 40351426, PMCID: PMC12062080, doi: 10.3389/fphar.2025.1550133, PubStatus:

The Impact of COVID-19 on Patients With Bradykinin-Mediated Angioedema in the ITACA Cohort.

Senter R, Zanichelli A, Stefano A, Arcoleo F, Bignardi D, Borrelli P, Cogliati C, Colangelo C, De Pasquale T, Giacco SD, Firinu D, Gidaro A, Pizzo ML, Parente R, Perego F, Petraroli A, Janu VP, Pucci S, Quattrocchi P, Ridolfi I, Rossi O, Ruin F, Spadaro G, Triggianese P, Zoli A, Triggiani M, Cancian M, The Impact of COVID-19 on Patients With Bradykinin-Mediated Angioedema in the ITACA Cohort., Clin Exp Allergy, 2025 May 9, PMID: 40344436, doi: 10.1111/cea.70079, PubStatus:a head of print

Hereditary Angioedema Characterized by Laryngeal Obstruction.

Liu W, Wen B, Cao Y, Li Y, Hereditary Angioedema Characterized by Laryngeal Obstruction., J Allergy Clin Immunol Pract, 2025 Aug, PMID: 40622323, doi: 10.1016/j.jaip.2025.05.045, PubStatus:ppublish+epublish

The determinants of angioedema attacks related to dental and gingival procedures in hereditary angioedema patients.

Gokmen NM, Gumusburun R, Camyar A, Ozgul S, Ozısık M, Turk T, Sin AZ, The determinants of angioedema attacks related to dental and gingival procedures in hereditary angioedema patients., BMC Oral Health, 2025 Jul 2, PMID: 40604794, PMCID: PMC12225231, doi: 10.1186/s12903-025-06359-7, PubStatus:epublish

A national survey of four decades of hereditary angioedema prophylaxis: Efficacy and safety of old and new drugs.

Horváth HR, Visy B, Kőhalmi KV, Balla Z, Andrási N, Czaller I, Zotter Z, Farkas H, A national survey of four decades of hereditary angioedema prophylaxis: Efficacy and safety of old and new drugs., Clin Immunol, 2025 Oct, PMID: 40513629, doi: 10.1016/j.clim.2025.110542, PubStatus:ppublish+epublish

CASE REPORTS

Hereditary angioedema in a paediatric patient presenting with abdominal pain and duodenal dilation.

Abbaker M, Rafe'e Y, Zamir I, Esch G, Hereditary angioedema in a paediatric patient presenting with abdominal pain and duodenal dilation., BMJ Case Rep, 2025 Jul 5, PMID: 40617599, doi: 10.1136/bcr-2024-263989, PubStatus:epublish

Four patients with hereditary angioedema with different initial symptoms and clinical features.

Chen J, Wang R, Zhang S, Wang Y, Chen Y, Zhang X, Lyu C, Four patients with hereditary angioedema with different initial symptoms and clinical features., World J Emerg Med, 2025 May 1, PMID: 40406293, PMCID: PMC12093428, doi: 10.5847/wjem.j.1920-8642.2025.060, PubStatus:ppublish

A rare case of hereditary angioedema with exclusive gastrointestinal symptoms and no cutaneous involvement.

Wu X, Yin T, Yu Y, A rare case of hereditary angioedema with exclusive gastrointestinal symptoms and no cutaneous involvement., Clin J Gastroenterol, 2025 May 16, PMID: 40377882, doi: 10.1007/s12328-025-02147-6, PubStatus:a head of print

Case Report: Identification of a novel mutation, c.1067T > A, in the SERPING1 gene in a Chinese male with type 1 hereditary angioedema.

Du W, Yang K, Zhang Q, Lin X, Zhang W, Guo W, Meng Z, Wang S, Case Report: Identification of a novel mutation, c.1067T > A, in the SERPING1 gene in a Chinese male with type 1 hereditary angioedema., Front Allergy, 2025, PMID: 40364801, PMCID: PMC12069465, doi: 10.3389/falgy.2025.1554940, PubStatus:

Ureteral Edema and Hydronephrosis Associated With Hereditary Angioedema Attack.

Galata Z, Harman M, Kirdok K, Bahadır A, Gökmen NM, Ureteral Edema and Hydronephrosis Associated With Hereditary Angioedema Attack., J Allergy Clin Immunol Pract, 2025 Jun, PMID: 40340291, doi: 10.1016/j.jaip.2025.03.020, PubStatus:ppublish+epublish

Hereditary angioedema-induced laryngeal edema: A diagnostic and therapeutic race against time.

Song H, Mu T, Wang D, Zhang Q, Song X, Hereditary angioedema-induced laryngeal edema: A diagnostic and therapeutic race against time., Am J Emerg Med, 2025 Jul, PMID: 40307161, doi: 10.1016/j.ajem.2025.04.059, PubStatus:ppublish+epublish

A Two-Decade-Delayed Diagnosis of Hereditary Angioedema: A Case Report.

Shrestha S, Yadav AK, Khadka D, Shrestha R, Paudel MS, A Two-Decade-Delayed Diagnosis of Hereditary Angioedema: A Case Report., Cureus, 2025 May, PMID: 40585652, PMCID: PMC12205570, doi: 10.7759/cureus.85019, PubStatus:

Perioperative C1-esterase inhibitor therapy to allow transplantation in a highly sensitized lung transplant candidate: Three case reports.

Devaquet J, Coiffard B, Demant X, Parquin F, Devriese M, Sage E, Pedini P, Picard C, Daviet F, D'Journo XB, Guidicelli G, Belousova N, Roux A, Perioperative C1-esterase inhibitor therapy to allow transplantation in a highly sensitized lung transplant candidate: Three case reports., Am J Transplant, 2025 Jul, PMID: 39978596, doi: 10.1016/j.ajt.2025.02.009, PubStatus:ppublish+epublish

Literatur Update: Archiv

Mai 2025

REVIEWS AND GUIDELINES

Garadacimab: First Approval.

Fung S, Garadacimab: First Approval., Drugs, 2025 Apr 22, PMID: 40261472, doi: 10.1007/s40265-025-02180-2, PubStatus:a head of print

Hereditary angioedema.

Uminski K, Betschel S, Goodyear D, Hereditary angioedema., CMAJ, 2025 Apr 21, PMID: 40262800, PMCID: PMC12017814, doi: 10.1503/cmaj.241815, PubStatus:epublish

CLINICAL STUDIES

Hereditary angioedema diagnosis evaluation score (HADES): A new clinical scoring system for predicting hereditary angioedema with C1 inhibitor deficiency.

Zwiener R, Zamora R, Galmarini CM, Brion L, Arias L, Pino A, Rozenfeld P, Hereditary angioedema diagnosis evaluation score (HADES): A new clinical scoring system for predicting hereditary angioedema with C1 inhibitor deficiency., J Allergy Clin Immunol Glob, 2025 May, PMID: 39991622, PMCID: PMC11847109, doi: 10.1016/j.jacig.2025.100414, PubStatus:

Global frequency, diagnosis, and treatment of hereditary angioedema with normal C1 inhibitor.

Magerl M, Riedl MA, Arruda LK, Bauer A, Berardi A, Bernstein JA, Bouillet L, Buckland M, Buttgereit T, Cohn DM, Craig T, Criado RF, Du-Thanh A, Fain O, Gonçalo M, Greve J, Grumach AS, Guilarte M, Katelaris C, Kinaciyan T, Latysheva EA, Lleonart R, Llosa OC, Mansour E, Grivcheva-Panovska V, Parisi C, Rosario Filho NA, Santos AS, Staubach P, Valerieva A, Rodrigues Valle SO, Danese S, Ulloa J, Audhya PK, Maurer M, Global frequency, diagnosis, and treatment of hereditary angioedema with normal C1 inhibitor., J Allergy Clin Immunol Glob, 2025 Aug, PMID: 40276547, PMCID: PMC12020835, doi: 10.1016/j.jacig.2025.100446, PubStatus:

Adherence and persistence among patients with hereditary angioedema receiving long-term prophylaxis in the United States.

Zuraw BL, Lopez-Gonzalez L, Manjelievskaia J, Winer I, Dean A, Wall S, Nelson J, Nestler-Parr S, Gillard P, Christiansen SC, Adherence and persistence among patients with hereditary angioedema receiving long-term prophylaxis in the United States., Allergy Asthma Proc, 2025 Apr 29, PMID: 40300843, doi: 10.2500/aap.2025.46.250029, PubStatus:a head of print

Clinical validity of dried blood spot assay for the measurement of functional C1 inhibitor in angioedema due to C1 inhibitor deficiency.

Bernstein JA, Cheng J, Pisani T, Sexton D, Whitaker RE, Nova Estepan D, Inhaber N, Clinical validity of dried blood spot assay for the measurement of functional C1 inhibitor in angioedema due to C1 inhibitor deficiency., J Allergy Clin Immunol Glob, 2025 May, PMID: 39944300, PMCID: PMC11814660, doi: 10.1016/j.jacig.2025.100401, PubStatus:

Cascade Screening of Hereditary Angioedema in Pakistan.

Hussain M, Riaz MO, Alam M, Hassan MA, Cascade Screening of Hereditary Angioedema in Pakistan., J Coll Physicians Surg Pak, 2025 May, PMID: 40325573, doi: 10.29271/jcpsp.2025.05.580, PubStatus:ppublish

Prediction of hereditary angioedema during attacks in patients with recurrent angioedema: Awareness at a glance with the hereditary angioedema prediction score.

Demir S, Olgaç M, Yeğit OO, Toprak İD, Çakmak ME, Hormet Mİ, Öztop N, Korkmaz P, Çelik ŞK, Karabacak DE, Kahveci N, İmren IG, Erden B, Coşkun R, Karadağ P, Ünal D, Gelincik A, Prediction of hereditary angioedema during attacks in patients with recurrent angioedema: Awareness at a glance with the hereditary angioedema prediction score., Clin Transl Allergy, 2025 Apr, PMID: 40237768, PMCID: PMC12001823, doi: 10.1002/clt2.70040, PubStatus:ppublish

Health care providers' experiences with genetic testing in patients at risk for hereditary angioedema.

Laney DA, Lee TD, Druss J, O'Keefe RL, Engel P, Riedl M, Health care providers' experiences with genetic testing in patients at risk for hereditary angioedema., J Allergy Clin Immunol Glob, 2025 May, PMID: 40201039, PMCID: PMC11978370, doi: 10.1016/j.jacig.2025.100445, PubStatus:

Urticaria unveiled in hereditary angioedema with carboxypeptidase N mutation.

Giavina-Bianchi P, Giavina-Bianchi M, Kalil J, Urticaria unveiled in hereditary angioedema with carboxypeptidase N mutation., J Allergy Clin Immunol Glob, 2025 May, PMID: 39974309, PMCID: PMC11836495, doi: 10.1016/j.jacig.2025.100405, PubStatus:

Anaesthesetic considerations in the perioperative management of patients with hereditary angioedema-FXII.

García Robledo P, García Ortiz JC, Anaesthesetic considerations in the perioperative management of patients with hereditary angioedema-FXII., Rev Esp Anestesiol Reanim (Engl Ed), 2025 May, PMID: 39961533, doi: 10.1016/j.redare.2025.501732, PubStatus:ppublish+epublish

Subjective assessment of sleep quality in adult patients with hereditary angioedema.

Karabiber E, Güngören EY, Özen A, Barış S, Karakoc-Aydiner E, Subjective assessment of sleep quality in adult patients with hereditary angioedema., Front Neurol, 2025, PMID: 40308220, PMCID: PMC12042759, doi: 10.3389/fneur.2025.1555562, PubStatus:

Analyzing social media conversations to gain insights into the experiences of patients with hereditary angioedema.

Braverman J, Ellis D, Gavata-Steigrer S, Babitha Dhas A, Muthu R, Ataher QS, Analyzing social media conversations to gain insights into the experiences of patients with hereditary angioedema., Allergy Asthma Proc, 2025 Apr 28, PMID: 40295108, doi: 10.2500/aap.2025.46.250028, PubStatus:a head of print

Machine Learning-Based Cluster Analysis in Chinese Patients With Recurrent Angioedema.

Ji R, Zhi Y, Machine Learning-Based Cluster Analysis in Chinese Patients With Recurrent Angioedema., Clin Exp Allergy, 2025 Apr 20, PMID: 40254549, doi: 10.1111/cea.70063, PubStatus:a head of print

CASE REPORTS

Hereditary angioedema-induced laryngeal edema: A diagnostic and therapeutic race against time.

Song H, Mu T, Wang D, Zhang Q, Song X, Hereditary angioedema-induced laryngeal edema: A diagnostic and therapeutic race against time., Am J Emerg Med, 2025 Apr 26, PMID: 40307161, doi: 10.1016/j.ajem.2025.04.059, PubStatus:a head of print

Identification of an elusive SERPING1 deletion in a family with hereditary angioedema type I utilizing soft clipping.

Wetherby K, Chiao J, Faulkner E, Guo Y, Hou S, Yu JJ, Chen J, Wan L, Henry Li H, Identification of an elusive SERPING1 deletion in a family with hereditary angioedema type I utilizing soft clipping., Front Allergy, 2025, PMID: 40313637, PMCID: PMC12043597, doi: 10.3389/falgy.2025.1565283, PubStatus:

Recurrent Acquired Angioedema as a Heralding Sign of Splenic Marginal Zone Lymphoma Recurrence.

Arabatzis TJ, Dadeboyina CJ, Minor HJ, Zhang XM, Ray P, Recurrent Acquired Angioedema as a Heralding Sign of Splenic Marginal Zone Lymphoma Recurrence., Cureus, 2025 Mar, PMID: 40271287, PMCID: PMC12017880, doi: 10.7759/cureus.81047, PubStatus:

Phenotypic Heterogeny of Hereditary Angioedema Within a Single Family.

Fails JM, Gierer SA, Phenotypic Heterogeny of Hereditary Angioedema Within a Single Family., Kans J Med, 2025 Mar-Apr, PMID: 40276615, PMCID: PMC12017626, doi: 10.17161/kjm.vol18.22749, PubStatus:

Transitioning from nonspecific therapy to berotralstat in hereditary angioedema type I: real world data from three patients within the same family.

Vincent D, Ghanam A, Transitioning from nonspecific therapy to berotralstat in hereditary angioedema type I: real world data from three patients within the same family., Eur Ann Allergy Clin Immunol, 2025 Apr 29, PMID: 40296758, doi: 10.23822/EurAnnACI.1764-1489.391, PubStatus:a head of print

April 2025

REVIEWS AND GUIDELINES

Expert consensus on the diagnosis and treatment of hereditary angioedema in China (2024 edition).

Xu Y, Liu S, Wang X, Chen W, Cheng L, Guo Y, Li J, Liu F, Liu R, Meng J, Sun Y, Wang S, Wei Q, Yu Y, Zhang H, Zhao Z, Zhu H, Zhu R, Zhi Y, Expert consensus on the diagnosis and treatment of hereditary angioedema in China (2024 edition)., Int Arch Allergy Immunol, 2025 Apr 10, PMID: 40209692, doi: 10.1159/000545808, PubStatus:a head of print

Recent Advances in Clinical Research on Rare Intractable Hereditary Skin Diseases in Japan.

Akiyama M, Takeichi T, Ikeda S, Ishiko A, Kurosawa M, Murota H, Shimomura Y, Suzuki T, Tamai K, Tanaka A, Terui T, Amagai M, Recent Advances in Clinical Research on Rare Intractable Hereditary Skin Diseases in Japan., Keio J Med, 2025 Mar 25, PMID: 37380461, doi: 10.2302/kjm.2023-0008-IR, PubStatus:ppublish+epublish

CLINICAL STUDIES

Pharmacokinetics, Pharmacodynamics, and Safety of Subcutaneous and Intravenous Garadacimab Following Single-Dose Administration in Healthy Japanese and White Adults.

Glassman F, Lawo JP, Bica MA, Roberts A, Pawaskar D, Akama H, Jain M, Goodson S, Pharmacokinetics, Pharmacodynamics, and Safety of Subcutaneous and Intravenous Garadacimab Following Single-Dose Administration in Healthy Japanese and White Adults., J Clin Pharmacol, 2025 Apr, PMID: 39582204, PMCID: PMC11937999, doi: 10.1002/jcph.6162, PubStatus:ppublish+epublish

Health care providers' experiences with genetic testing in patients at risk for hereditary angioedema.

Content validation of the Angioedema Quality of Life Questionnaire (AE-QoL) in a population of adult and adolescent patients with hereditary angioedema (HAE).

Broderick L, Foster A, Waldman LT, Jacobs KD, Bordone L, Yarlas A, Content validation of the Angioedema Quality of Life Questionnaire (AE-QoL) in a population of adult and adolescent patients with hereditary angioedema (HAE)., J Patient Rep Outcomes, 2025 Apr 12, PMID: 40220084, PMCID: PMC11993509, doi: 10.1186/s41687-025-00876-3, PubStatus:epublish

Comparison of real-world healthcare resource utilization and costs among patients with hereditary angioedema on lanadelumab or berotralstat long-term prophylaxis.

Princic N, Evans KA, Shah CH, Sing K, Juethner S, Schultz BG, Comparison of real-world healthcare resource utilization and costs among patients with hereditary angioedema on lanadelumab or berotralstat long-term prophylaxis., J Comp Eff Res, 2025 Apr, PMID: 39976166, PMCID: PMC11963383, doi: 10.57264/cer-2024-0205, PubStatus:ppublish+epublish

Correction: The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks.

Betschel SD, Caballero T, Jones DH, Longhurst HJ, Manning M, van Kooten S, Heckmann M, Danese S, Goga L, Burnette AF, Correction: The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks., Allergy Asthma Clin Immunol, 2025 Apr 7, PMID: 40197292, PMCID: PMC11978150, doi: 10.1186/s13223-025-00960-9, PubStatus:epublish

Attack frequency and associated factors in hereditary angioedema patients: a single-centre experience.

Durmaz MSB, Sevimli N, Attack frequency and associated factors in hereditary angioedema patients: a single-centre experience., Postepy Dermatol Alergol, 2025 Feb, PMID: 40114773, PMCID: PMC11921922, doi: 10.5114/ada.2024.144488, PubStatus:ppublish+epublish

Hereditary angioedema: Patient health care experiences within underrepresented racial and ethnic groups in the United States.

Craig T, Baptist AP, Anderson J, Zaragoza-Urdaz RH, Burnette AF, Kelbel TE, Riedl MA, Vanegas A, Boyle K, Bartsch JL, Darden C, Brown TM, Schultz BG, Blair C, Sing K, Fox D, Juethner S, Hereditary angioedema: Patient health care experiences within underrepresented racial and ethnic groups in the United States., Ann Allergy Asthma Immunol, 2025 Apr, PMID: 39842773, doi: 10.1016/j.anai.2025.01.007, PubStatus:ppublish+epublish

März 2025

REVIEWS AND GUIDELINES

Hereditary Angioedema with Normal C1 Inhibitor: A Quarter Century of Forward Progress and Persisting Obstacles.

Christiansen SC, Banerji A, Bernstein JA, Busse PJ, Craig T, Li HH, Davis-Lorton M, Lumry WR, Riedl MA, Zuraw BL, Hereditary Angioedema with Normal C1 Inhibitor: A Quarter Century of Forward Progress and Persisting Obstacles., J Allergy Clin Immunol Pract, 2025 Mar 6, PMID: 40057189, doi: 10.1016/j.jaip.2025.02.036, PubStatus:a head of print

Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment.

Zuraw BL, Bork K, Bouillet L, Christiansen SC, Farkas H, Germenis AE, Grumach AS, Kaplan A, López-Lera A, Magerl M, Riedl MA, Adatia A, Banerji A, Betschel S, Boccon-Gibod I, Bova M, Boysen HB, Caballero T, Cancian M, Castaldo AJ, Cohn DM, Corcoran D, Drouet C, Fukunaga A, Hide M, Katelaris CH, Li PH, Longhurst H, Peter J, Psarros F, Reshef A, Ritchie B, Selva CN, Zanichelli A, Maurer M, Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment., Clin Rev Allergy Immunol, 2025 Mar 7, PMID: 40053270, PMCID: PMC11889046, doi: 10.1007/s12016-025-09027-4, PubStatus:epublish

Interplay between on-demand treatment trials for hereditary angioedema and treatment guidelines.

Cohn DM, Soteres DF, Craig TJ, Lumry WR, Magerl M, Riedl MA, Audhya PK, Maurer M, Bernstein JA, Interplay between on-demand treatment trials for hereditary angioedema and treatment guidelines., J Allergy Clin Immunol, 2025 Mar, PMID: 39724968, doi: 10.1016/j.jaci.2024.12.1079, PubStatus:ppublish+epublish

CLINICAL STUDIES

Population Pharmacokinetic/Pharmacodynamic and Exposure-Response Modeling of Garadacimab in Healthy Volunteers and Patients With Hereditary Angioedema.

Garcia R, Cheng S, Glassman F, Sharma A, De Miguel-Lillo B, Wiens M, Johnston C, Lawo JP, Pragst I, French J, Polhamus D, Nandy P, Population Pharmacokinetic/Pharmacodynamic and Exposure-Response Modeling of Garadacimab in Healthy Volunteers and Patients With Hereditary Angioedema., CPT Pharmacometrics Syst Pharmacol, 2025 Mar 5, PMID: 40042097, doi: 10.1002/psp4.70009, PubStatus:a head of print

Angioedema Due to Acquired C1-Inhibitor Deficiency Without Hematological Condition: A Multicenter French Cohort Study of 34 Patients.

Stammler R, Defendi F, Aubineau M, Bibes B, Boccon-Gibod I, Bouillet L, Crabol Y, Dalmas MC, de Moreuil C, Delluc A, Dingremont C, Du-Thanh A, Hadjadj J, Jeandel PY, Kalmi G, Lacoste M, Martin L, Avoy CM, Blanchard-Delaunay C, Taquet MC, Fain O, Gobert D, Angioedema Due to Acquired C1-Inhibitor Deficiency Without Hematological Condition: A Multicenter French Cohort Study of 34 Patients., J Allergy Clin Immunol Pract, 2025 Mar, PMID: 39756514, doi: 10.1016/j.jaip.2024.12.027, PubStatus:ppublish+epublish

Quality of life in patients with hereditary angioedema correlates with angioedema control: Our experience at Chandigarh, India.

Sharma R, Basu S, Tyagi R, Siniah S, Barman P, Sil A, Machhua S, Chawla S, Pandiarajan V, Pilania RK, Sharma S, Dhaliwal M, Suri D, Rawat A, Kumaran SM, Dogra S, Singh S, Jindal AK, Quality of life in patients with hereditary angioedema correlates with angioedema control: Our experience at Chandigarh, India., Asia Pac Allergy, 2025 Mar, PMID: 40051425, PMCID: PMC11882216, doi: 10.5415/apallergy.0000000000000172, PubStatus:ppublish+epublish

Indirect treatment comparison of oral sebetralstat and intravenous recombinant human C1 esterase inhibitor for on-demand treatment of hereditary angioedema attacks.

Li HH, Aygören-Pürsün E, Magerl M, Craig TJ, Manning ME, Hummel N, Kopiec A, Fu S, Morris J, Wang A, Audhya PK, Bernstein JA, Indirect treatment comparison of oral sebetralstat and intravenous recombinant human C1 esterase inhibitor for on-demand treatment of hereditary angioedema attacks., Allergy Asthma Clin Immunol, 2025 Mar 15, PMID: 40089800, PMCID: PMC11909897, doi: 10.1186/s13223-025-00955-6, PubStatus:epublish

Impact of Dental Procedures on Hereditary Angioedema Attacks: An Exploratory Observational Study.

Nadasan V, Kiss KO, Borka-Balás R, Bara NA, Impact of Dental Procedures on Hereditary Angioedema Attacks: An Exploratory Observational Study., Oral Health Prev Dent, 2025 Mar 14, PMID: 40084797, PMCID: PMC11912900, doi: 10.3290/j.ohpd.c_1907, PubStatus:epublish

Characteristics of Patients with Hereditary Angioedema Who Reduced Lanadelumab Treatment Administration Frequency: A Retrospective Observational Study of US Claims Data.

Princic N, Evans KA, Shah CH, Sing K, Juethner S, Schultz BG, Characteristics of Patients with Hereditary Angioedema Who Reduced Lanadelumab Treatment Administration Frequency: A Retrospective Observational Study of US Claims Data., Drugs Real World Outcomes, 2025 Mar, PMID: 39875773, PMCID: PMC11829876, doi: 10.1007/s40801-024-00470-x, PubStatus:ppublish+epublish

Comorbidities in Canadian patients with hereditary angioedema: a quantitative survey study.

Keith PK, Lacuesta G, Goodyear D, Betschel SD, Yap B, Dansereau MF, Tanios N, El-Sayegh R, Machnouk M, Mahfouz H, Martin A, Waserman S, Comorbidities in Canadian patients with hereditary angioedema: a quantitative survey study., Allergy Asthma Clin Immunol, 2025 Mar 19, PMID: 40108700, PMCID: PMC11924777, doi: 10.1186/s13223-025-00953-8, PubStatus:epublish

CASE REPORTS

Recurrent Hand Oedema and Abdominal Pain.

Leite-Almeida L, Silva D, Dias M, Bordalo D, Jacob S, Recurrent Hand Oedema and Abdominal Pain., J Paediatr Child Health, 2025 Mar, PMID: 39807679, doi: 10.1111/jpc.16783, PubStatus:ppublish+epublish

Recurrent Intestinal Angioedema with Normal C1-Inhibitor: A Case Report.

Jevtic D, Taylor A, Dumic I, Sviggum E, Nordstrom CW, Antic M, Recurrent Intestinal Angioedema with Normal C1-Inhibitor: A Case Report., Medicina (Kaunas), 2025 Jan 31, PMID: 40005362, PMCID: PMC11857147, doi: 10.3390/medicina61020245, PubStatus:epublish

Januar 2025

REVIEWS AND GUIDELINES

Hereditary angioedema due to C1-inhibitor deficiency: current therapeutic approaches.

Costanzo G, Sambugaro G, Firinu D, Hereditary angioedema due to C1-inhibitor deficiency: current therapeutic approaches., Curr Opin Allergy Clin Immunol, 2024 Dec 1, PMID: 39407363, PMCID: PMC11537475, doi: 10.1097/ACI.0000000000001042, PubStatus:ppublish+epublish

Hereditary angioedema in children: Review and practical perspective for clinical management.

Pagnier A, Dermesropian A, Kevorkian-Verguet C, Bourgoin-Heck M, Hoarau C, Reumaux H, Nugues F, Audouin-Pajot C, Blanc S, Carbasse A, Jurquet AL, Voidey M, Villedieu M, Bouillet L, Boccon-Gibod I, Hereditary angioedema in children: Review and practical perspective for clinical management., Pediatr Allergy Immunol, 2024 Dec, PMID: 39655944, PMCID: PMC11629734, doi: 10.1111/pai.14268, PubStatus:ppublish

Hereditary Angioedema Attacks in Patients Receiving Long-Term Prophylaxis: A Systematic Review.

Longhurst HJ, Cancian M, Grivcheva-Panovska V, Koleilat M, Magerl M, Savic S, Stobiecki M, Tachdjian R, Healy B, Yea CM, Audhya PK, Bouillet L, Hereditary Angioedema Attacks in Patients Receiving Long-Term Prophylaxis: A Systematic Review., Clin Rev Allergy Immunol, 2024 Dec, PMID: 39508959, PMCID: PMC11638394, doi: 10.1007/s12016-024-09006-1, PubStatus:ppublish+epublish

Interplay between on-demand treatment trials for hereditary angioedema and treatment guidelines.

Cohn DM, Soteres DF, Craig TJ, Lumry WR, Magerl M, Riedl MA, Audhya PK, Maurer M, Bernstein JA, Interplay between on-demand treatment trials for hereditary angioedema and treatment guidelines., J Allergy Clin Immunol, 2024 Dec 24, PMID: 39724968, doi: 10.1016/j.jaci.2024.12.1079, PubStatus:a head of print

CLINICAL STUDIES

Timing of Onset of Garadacimab for Preventing Hereditary Angioedema Attacks.

Staubach P, Tachdjian R, Li HH, Hakl R, Aygören-Pürsün E, Wieman L, Lawo JP, Craig TJ, Timing of Onset of Garadacimab for Preventing Hereditary Angioedema Attacks., Clin Exp Allergy, 2024 Dec, PMID: 39353415, PMCID: PMC11629047, doi: 10.1111/cea.14568, PubStatus:ppublish+epublish

Pharmacokinetics, Pharmacodynamics, and Safety of Subcutaneous and Intravenous Garadacimab Following Single-Dose Administration in Healthy Japanese and White Adults.

Glassman F, Lawo JP, Bica MA, Roberts A, Pawaskar D, Akama H, Jain M, Goodson S, Pharmacokinetics, Pharmacodynamics, and Safety of Subcutaneous and Intravenous Garadacimab Following Single-Dose Administration in Healthy Japanese and White Adults., J Clin Pharmacol, 2024 Nov 24, PMID: 39582204, doi: 10.1002/jcph.6162, PubStatus:a head of print

Long-term prevention of hereditary angioedema attacks with lanadelumab in adolescents.

Craig T, Tachdjian R, Bernstein JA, Anderson J, Nurse C, Watt M, Yu M, Juethner S, Long-term prevention of hereditary angioedema attacks with lanadelumab in adolescents., Ann Allergy Asthma Immunol, 2024 Dec, PMID: 39128590, doi: 10.1016/j.anai.2024.08.001, PubStatus:ppublish+epublish

Genital Attacks in Hereditary Angioedema and Their Effects on Sexual Life.

Camyar A, Bulut G, Ozisik M, Altay S, Tuncel OK, Ozgul S, Sin AZ, Gokmen NM, Genital Attacks in Hereditary Angioedema and Their Effects on Sexual Life., Medicina (Kaunas), 2024 Oct 30, PMID: 39596962, PMCID: PMC11596147, doi: 10.3390/medicina60111777, PubStatus:epublish

A clinical evaluation of patients with known mutations (plasminogen and factor XII) with a focus on prophylactic treatment.

Lochbaum R, Trainotti S, Hoffmann TK, Greve J, Hahn J, A clinical evaluation of patients with known mutations (plasminogen and factor XII) with a focus on prophylactic treatment., J Dermatolog Treat, 2024 Dec, PMID: 38086754, doi: 10.1080/09546634.2023.2290362, PubStatus:ppublish+epublish

Efficacy and safety of Donidalorsen in Hereditary Angioedema with C1 inhibitor deficiency: a systematic review and a meta analysis.

Raja A, Shuja MH, Raja S, Qammar A, Kumar S, Khurram L, Haque MA, Efficacy and safety of Donidalorsen in Hereditary Angioedema with C1 inhibitor deficiency: a systematic review and a meta analysis., Arch Dermatol Res, 2024 Dec 12, PMID: 39666085, doi: 10.1007/s00403-024-03652-3, PubStatus:epublish

Phenotypic and molecular characterization of the largest worldwide cluster of hereditary angioedema type 1.

Arias-Flórez JS, Ramirez SX, Bayona-Gomez B, Castro-Castillo L, Correa-Martinez V, Sanchez-Gomez Y, Usaquén-Martínez W, Casas-Vargas LA, Olmos Olmos CE, Contreras Bravo N, Velandia-Piedrahita CA, Morel A, Cabrera-Perez R, Santiago-Tovar N, Gaviria-Sabogal CC, Bernal IT, Fonseca-Mendoza DJ, Restrepo CM, Phenotypic and molecular characterization of the largest worldwide cluster of hereditary angioedema type 1., PLoS One, 2024, PMID: 39724085, PMCID: PMC11671010, doi: 10.1371/journal.pone.0311316, PubStatus:

Development and validation of a US quality of life instrument for hereditary angioedema due to C1 inhibitor deficiency.

Castaldo AJ, Siersbæk N, Corcoran D, Selva CN, Jervelund C, Christiansen SC, Riedl MA, Zuraw BL, Busse PJ, Development and validation of a US quality of life instrument for hereditary angioedema due to C1 inhibitor deficiency., Ann Allergy Asthma Immunol, 2024 Dec, PMID: 39038704, doi: 10.1016/j.anai.2024.07.018, PubStatus:ppublish+epublish

On-demand treatment of hereditary angioedema attacks: patient-reported utilization, barriers, and outcomes.

Christiansen S, O'Connor M, Craig T, Radojicic C, Wedner HJ, Danese S, Ulloa J, Desai V, Utter C, Andriotti T, Audhya P, Busse P, On-demand treatment of hereditary angioedema attacks: patient-reported utilization, barriers, and outcomes., Ann Allergy Asthma Immunol, 2024 Dec 16, PMID: 39694088, doi: 10.1016/j.anai.2024.12.012, PubStatus:a head of print

Management of hereditary angioedema attacks by patients on long-term prophylaxis versus on-demand therapy only.

Lumry WR, Grumach AS, Betschel SD, Radojicic C, van Kooten S, Heckmann M, Danese S, Malloy N, Goga L, Guilarte M, Management of hereditary angioedema attacks by patients on long-term prophylaxis versus on-demand therapy only., Allergy Asthma Proc, 2025 Jan 1, PMID: 39586588, doi: 10.2500/aap.2025.46.240096, PubStatus:ppublish+epublish

Real-world Effectiveness of Lanadelumab in Hereditary Angioedema: Multi-country INTEGRATED Observational Study.

Magerl M, Bouillet L, Martinez-Saguer I, Gavini F, Bent-Ennakhil N, Sayegh L, Andresen I, Real-world Effectiveness of Lanadelumab in Hereditary Angioedema: Multi-country INTEGRATED Observational Study., J Allergy Clin Immunol Pract, 2024 Dec 17, PMID: 39701274, doi: 10.1016/j.jaip.2024.12.008, PubStatus:a head of print

CASE REPORTS

Pregnancy revealing hereditary angioedema type III with factor XII mutation managed in a resource-limited setting with no C1-inhibitor concentrate availability: a case report.

Saddouk H, Tajir M, Saadi H, Housni B, Zerrouki N, Zizi N, Dikhaye S, Pregnancy revealing hereditary angioedema type III with factor XII mutation managed in a resource-limited setting with no C1-inhibitor concentrate availability: a case report., Int J Obstet Anesth, 2024 Oct 9, PMID: 39617660, doi: 10.1016/j.ijoa.2024.104283, PubStatus:a head of print

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