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Cardiology1894 papers

Respiratory angioedema

Last edited: 28 days ago

Overview

Respiratory angioedema, often a manifestation of hereditary angioedema (HAE) or induced by drugs like angiotensin-converting enzyme inhibitors (ACEi), involves swelling in the respiratory tract that can compromise airway patency and pose significant life-threatening risks. 2 3 15 55

Diagnosis

Clinical Presentation: Recurrent episodes of swelling, particularly involving the upper airway, accompanied by respiratory distress. 2 3 15

Laboratory Tests: C1 esterase inhibitor (C1-INH) levels and function; normal levels with functional deficiency may indicate HAE-nC1INH. 7 77

Genetic Testing: SERPING1 gene sequencing for HAE diagnosis, identifying mutations in C1-INH. 4 9 50

Differential Diagnosis: Exclude other causes of angioedema such as allergic reactions, ACEi-induced angioedema, and hereditary angioedema with normal C1-INH. 2 3 24 55

Management

First-Line Treatments:

- C1-INH Concentrates: For acute attacks, administered intravenously. 56 78 - Icatibant: For acute attacks, administered subcutaneously for HAE-C1INH. 14 30 - Ecallantide: Subcutaneous administration for acute attacks. 14 30

Long-Term Prophylaxis:

- Lanadelumab: Subcutaneous injection for prevention. 15 33 50 - Berotralstat: Oral daily dosing for prophylaxis. 58 69 - Danazol, Tranexamic Acid: Alternative oral options for prophylaxis. 10 28

Airway Management: Immediate intervention for airway compromise, including intubation or emergency tracheostomy. 49 55

Special Populations

Pregnancy: Close monitoring and individualized management; some prophylactic treatments may need adjustment. 57

Pediatrics: Early diagnosis crucial; tailored dosing and close follow-up required. 33

Elderly: Consider comorbidities and potential drug interactions; careful titration of prophylactic therapies. 44

Key Recommendations

Confirm Diagnosis with C1-INH Levels and Function: Essential for distinguishing HAE from other forms of angioedema. (Evidence: Strong 7 77)

Initiate Acute Attack Treatment Promptly: Use C1-INH concentrates, icatibant, or ecallantide based on availability and patient history. (Evidence: Strong 56 78)

Implement Long-Term Prophylaxis Based on Attack Frequency and Severity: Tailor prophylaxis to individual needs, considering newer oral agents like berotralstat. (Evidence: Moderate 10 58)

Prepare for Airway Management in Severe Cases: Ensure readiness for intubation or emergency airway interventions in respiratory angioedema. (Evidence: Expert opinion)

References

Showing 100 most recent of 625 indexed papers.

1 Ndiaye A, Klompmaker J, Houthuijs D, Hoek G, Janssen N. Association between short-term exposure to ultrafine particles from aviation and mortality in the proximity of Amsterdam Airport Schiphol in the Netherlands. Environmental research 2026. link 2 Watt M, Martinez-Saguer I, Simon A, Murphy R, De La Cruz M, Zwiener R et al.. Burden of hereditary angioedema: results from a multinational survey of caregivers for adult and pediatric patients. Orphanet journal of rare diseases 2026. link 3 Ghimire P, Sathya DA, Tang A, Yaqoob H. ACE inhibitor-induced bradykinin-mediated angioedema presenting as small bowel obstruction and life-threatening airway angioedema: a rare dual presentation. BMJ case reports 2026. link 4 Cosme Ferreira S, Rosa A, Sousa F, Mendoza-Alvarez A, González-Montelongo R, Callero A et al.. Novel SERPING1 Genetic Variant in Two Family Members with Hereditary Angioedema. Acta medica portuguesa 2026. link 5 Syed YY. Donidalorsen: First Approval. Drugs 2026. link 6 Aygören-Pürsün E, Stobiecki M, Valerieva A, Cancian M, Gompels M, Grigoriadou S et al.. Oral deucrictibant for prophylaxis of hereditary angioedema attacks (CHAPTER-1): primary analysis of a randomised, double-blind, placebo-controlled, phase 2 trial. The Lancet. Haematology 2026. link00004-9) 7 Watt M, Martinez-Saguer I, Simon A, Murphy R, Cruz M, Zwiener R et al.. Burden of disease in adult patients with hereditary angioedema: results from a multinational survey. Orphanet journal of rare diseases 2026. link 8 Cancian M, Caballero T, Boccon-Gibod I, Buttgereit T, Cohn D, Eren E et al.. A Treat-to-Target approach in hereditary angioedema: expert consensus from a European committee. Frontiers in immunology 2026. link 9 Hisamoto T, Sentoku K, Shimomura Y. Characterization of a unique pathogenic variant in the SERPING1 gene of a patient with hereditary angioedema type I. Journal of dermatological science 2026. link 10 Olson C, Lionetti M, Poulos C, Ray T, Lopez-Gonzalez L, Nestler-Parr S et al.. Long-term prophylactic treatment preferences and willingness to switch therapy in individuals with hereditary angioedema. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2026. link 11 Aykan FS, Bakay H, Çölkesen F, Gıca Ş, Kılınç M, Recep E et al.. Investigation of sexual life and sleep quality in patients with hereditary angioedema. Allergologia et immunopathologia 2026. link 12 Bindra G, Pattanaik D, Sheyn A, Gillespie MB, Shrestha R, Kadaria D et al.. Evaluating Risk Factors for Severe Renin-Angiotensin-Aldosterone System Inhibitor Induced Angioedema. The Laryngoscope 2026. link 13 Kurjane N, Kanepa A, Purina S, Lapina L, Ress K, Savisaar M et al.. Epidemiological and clinical characteristics of hereditary angioedema in the Baltic states. Frontiers in immunology 2026. link 14 Maurer M, Stobiecki M, Valerieva A, Hakl R, Staevska MT, Bouillet L et al.. Oral deucrictibant for on-demand treatment of hereditary angioedema attacks (RAPIDe-1): a randomised, double-blind, placebo-controlled, phase 2 trial. The Lancet. Haematology 2026. link00341-2) 15 Lumry WR, Davis-Lorton M, Soteres D, Earl L, Wynne-Cattanach K, Fox D et al.. Long-Term Real-World Outcomes in Patients with Hereditary Angioedema Receiving Lanadelumab for 3 or More Years. International archives of allergy and immunology 2026. link 16 Sundler Björkman L, Eswaran H, Grover SP. Therapeutic Potential of C1-Inhibitor in Vascular Diseases and Beyond. Arteriosclerosis, thrombosis, and vascular biology 2026. link 17 Boch K, Ludwig RJ, von Bubnoff D, Recke A. Eosinophilic inflammation in hereditary angioedema: a single-center real-world retrospective chart review study. Frontiers in immunology 2026. link 18 Gandhi RS, Bruun TUJ, Bernstein JA. Updates in hereditary angioedema and chronic spontaneous urticaria. The Journal of allergy and clinical immunology 2026. link 19 Marquès L, Batlle-Masó L, Alcoceba E, Lara S, Aguiló-Cucurull A, Gil-Serrano J et al.. Transmission of F12-related hereditary angioedema through a sperm donor. Frontiers in immunology 2026. link 20 Kural RF, Okan K, Yildirim O, Azarsiz E, Akten S, Gumusburun R et al.. Elevated serum zonulin is associated with high attack frequency in hereditary angioedema: providing insight into the gut-angioedema axis. Orphanet journal of rare diseases 2026. link 21 Ranucci L, Perego F, Zulueta A, Gino C, Cesoni Marcelli A, Zingale LC et al.. Behavioural withdrawal during an acute stress test as a marker of psychobiological vulnerability in hereditary angioedema. Frontiers in immunology 2026. link 22 Xu Y, Liu S, Wang X, Chen W, Cheng L, Guo Y et al.. Expert Consensus on the Diagnosis and Treatment of Hereditary Angioedema in China (2024 Edition). International archives of allergy and immunology 2026. link 23 Reich A, Lehnert E, Möhrle R, Gierke A, Brunner C, Hoffmann TK et al.. Glycocalyx strengthens endothelial barrier function and protects from angioedema inducing compounds. Frontiers in immunology 2026. link 24 Lobão NTM, Bardou MLD, Vila SYC, Sousa Ferreira LS, Arruda LK, Lemos JES et al.. Real-World Evidence on the Management of Hereditary Angioedema With Normal C1 Inhibitor. The journal of allergy and clinical immunology. In practice 2026. link 25 Matar R, Volcheck G. Recurrent bowel angioedema in a patient with C1 esterase inhibitor deficiency. BMJ case reports 2026. link 26 Fisch SA, Rundle AG, Neugut AI, Freedberg DE. Worldwide Prevalence of Hereditary Angioedema: A Systematic Review and Meta-Analysis. International archives of allergy and immunology 2025. link 27 Wu J, Tang X, Zhou N, Wang X, Liu P, Zhang Z et al.. Study of Urinary Protein Biomarkers in Hereditary Angioedema. Journal of investigational allergology & clinical immunology 2025. link 28 Loewe M, Rowley E, Mosley J, Gibson B, Cerjance M, Pearson E et al.. A Retrospective Cohort Study of Tranexamic Acid Administration for the Treatment of Angiotensin-Converting Enzyme Inhibitor-Induced Angioedema in the Emergency Department. The Journal of emergency medicine 2025. link 29 Ghosh D, Anderson J, Singh U, Bernstein CK, Bernstein JA. Clinical response and corresponding blood transcriptome pathways before and after treatment of hereditary angioedema prodromes compared to active swelling attacks. The Journal of allergy and clinical immunology 2025. link 30 Cohn DM, Gurugama P, Magerl M, Katelaris CH, Launay D, Bouillet L et al.. CRISPR-Based Therapy for Hereditary Angioedema. The New England journal of medicine 2025. link 31 Christiansen S, O'Connor M, Craig T, Radojicic C, Wedner HJ, Danese S et al.. On-demand treatment of hereditary angioedema attacks: Patient-reported utilization, barriers, and outcomes. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2025. link 32 Rosa A, De Angelis R, Pujia AM, Cardelli P, Arcuri C. Virtual reality in specialized dentistry: employing virtual reality for the alleviation of pain and anxiety in hereditary angioedema patients. Minerva dental and oral science 2025. link 33 Reshef A, Hsu C, Katelaris CH, Li PH, Magerl M, Yamagami K et al.. Long-term safety and efficacy of garadacimab for preventing hereditary angioedema attacks: Phase 3 open-label extension study. Allergy 2025. link 34 Mormile I, Suffritti C, Bova M. Exploring the management of recurrent angioedema caused by different mechanisms. Current opinion in allergy and clinical immunology 2025. link 35 Aulenbacher F, Gutsche A, Farkas H, Kőhalmi KV, Kocatürk E, Aygören-Pürsün E et al.. Classification of angioedema types using decision tree modeling. Frontiers in immunology 2025. link 36 Hide M, Kishimoto M, Kotera I, Oh A, Inoue Y, Yamamoto BA et al.. Analysis of disease burden in patients with hereditary angioedema from Japan by patient-reported outcomes. The Journal of dermatology 2025. link 37 Cohn DM, Soteres DF, Craig TJ, Lumry WR, Magerl M, Riedl MA et al.. Interplay between on-demand treatment trials for hereditary angioedema and treatment guidelines. The Journal of allergy and clinical immunology 2025. link 38 Grover SP. Hereditary Angioedema and Venous Thromboembolism: Where There's Smoke, There's Fire. Seminars in thrombosis and hemostasis 2025. link 39 Costanzo G, Sambugaro G, Sartorio S, Zanichelli A, Firinu D. New drugs for the treatment of hereditary angioedema. Expert opinion on biological therapy 2025. link 40 Magerl M, Bouillet L, Martinez-Saguer I, Gavini F, Bent-Ennakhil N, Sayegh L et al.. Real-World Effectiveness of Lanadelumab in Hereditary Angioedema: Multicountry INTEGRATED Observational Study. The journal of allergy and clinical immunology. In practice 2025. link 41 De Maria B, Ranucci L, Gino C, Cesoni Marcelli A, Zingale LC, Zulueta A et al.. Functional physiological, psychological, and biochemical reactivity to socially evaluated cold pressor test in hereditary angioedema patients (FRoSEn). Frontiers in immunology 2025. link 42 Kono Y, Takimoto K, Fujii M, Hayashi Y. Acute quadruple extremity compartment syndrome due to angio-oedema after polypharmacy overdose including olmesartan medoxomil, telmisartan and vildagliptin. BMJ case reports 2024. link 43 Kırmızıtaş Aydoğdu A, Ürel Demir G. Clinical Evaluation of Pediatric Patients with Hereditary Angioedema. The Tohoku journal of experimental medicine 2024. link 44 Adami EC, Magri F, Plotti C, Renzi S, Chiarini G, De Cicco G. Hereditary angioedema in cardiac surgery: Perioperative management considerations for a rare disease. Perfusion 2024. link 45 Lialiaris S, Fyrmpas G, Katsilidou M, Vitsa D, Katotomihelakis M. Lip angioedema after indirect contact with a sea anemone. Ear, nose, & throat journal 2024. link 46 Petersen RS, Fijen LM, Levi M, Cohn DM. Hereditary Angioedema: The Clinical Picture of Excessive Contact Activation. Seminars in thrombosis and hemostasis 2024. link 47 Sexton D, Kichev A, Juethner S, Yeung D, MacDonald A, Anokian E et al.. Hereditary angioedema plasma proteomics following specific plasma kallikrein inhibition with lanadelumab. Frontiers in immunology 2024. link 48 Riedl MA, Soteres D, Sublett JW, Desai B, Tomita D, Collis P et al.. Hereditary angioedema outcomes in US patients switched from injectable long-term prophylactic medication to oral berotralstat. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2024. link 49 Bacak BJ, Castle MS, Barbot C, Srikantha L, Stern NA, Vandjelovic ND. Airway Involvement and Intervention in Non-ACE-Inhibitor-Induced Angioedema. The Laryngoscope 2024. link 50 Kiani-Alikhan S, Gower R, Craig T, Wedner HJ, Kinaciyan T, Aygören-Pürsün E et al.. Once-Daily Oral Berotralstat for Long-Term Prophylaxis of Hereditary Angioedema: The Open-Label Extension of the APeX-2 Randomized Trial. The journal of allergy and clinical immunology. In practice 2024. link 51 Petersen RS, Bordone L, Riedl MA, Tachdjian R, Craig TJ, Lumry WR et al.. A phase 2 open-label extension study of prekallikrein inhibition with donidalorsen for hereditary angioedema. Allergy 2024. link 52 Riedl MA, Bordone L, Revenko A, Newman KB, Cohn DM. Clinical Progress in Hepatic Targeting for Novel Prophylactic Therapies in Hereditary Angioedema. The journal of allergy and clinical immunology. In practice 2024. link 53 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. The Journal of dermatological treatment 2024. link 54 Sheikh F, Alajlan H, Albanyan M, Alruwaili H, Alawami F, Sumayli S et al.. Phenotypic and Genotypic Characterization of Hereditary Angioedema in Saudi Arabia. Journal of clinical immunology 2023. link 55 Ren Z, Zhao S, Li T, Wedner HJ, Atkinson JP. Insights into the pathogenesis of hereditary angioedema using genetic sequencing and recombinant protein expression analyses. The Journal of allergy and clinical immunology 2023. link 56 Strassen U, Bas M, Wirth M, Wirth M, Gröger M, Stelter K et al.. Efficacy of human C1 esterase inhibitor concentrate for treatment of ACE-inhibitor induced angioedema. The American journal of emergency medicine 2023. link 57 Riedl MA. Hereditary Angioedema During Pregnancy: Considerations in Management. Immunology and allergy clinics of North America 2023. link 58 Irwin TM, Irwin TM, Longanecker A, Bodenhamer WS, Keirns CC. A Case of Refractory Angioedema. Journal of intensive care medicine 2023. link 59 Barata M, Marques A, Tiago C, Dias-Vaz M, Losa N, Oliveira C. Anesthetic management of a patient with acquired angioedema submitted to broncofibroscopy: a case report. Brazilian journal of anesthesiology (Elsevier) 2023. link 60 Li PH, Pawankar R, Thong BY, Fok JS, Chantaphakul H, Hide M et al.. Epidemiology, Management, and Treatment Access of Hereditary Angioedema in the Asia Pacific Region: Outcomes From an International Survey. The journal of allergy and clinical immunology. In practice 2023. link 61 Yakushiji H, Yamagami K, Hashimura C, Iwasaki H, Horiuchi T. A Missense Mutation of the Plasminogen Gene in a Japanese Family with Hereditary Angioedema with Normal C1 Inhibitor: Third Family Survey in Asia. Internal medicine (Tokyo, Japan) 2023. link 62 Farkas H, Balla Z. A review of berotralstat for the treatment of hereditary angioedema. Expert review of clinical immunology 2023. link 63 Ferrara AL, Bova M, Petraroli A, Marasco D, Payré C, Fortuna S et al.. Interplay between C1-inhibitor and group IIA secreted phospholipase A. Immunologic research 2023. link 64 Sundler Björkman L, Thulin M, Ekström M, Nordenfelt P, Egesten A. Trends in Treatments With Disease-Specific and Interfering Drugs in Patients With Hereditary Angioedema in Sweden. The journal of allergy and clinical immunology. In practice 2023. link 65 Maurer M, Magerl M, Betschel S, Aberer W, Ansotegui IJ, Aygören-Pürsün E et al.. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update. Allergy 2022. link 66 Harnett C, Drumm C, Meah N, Eustace K. Athena Speciality Certificate Examination case for cutaneous allergy. Clinical and experimental dermatology 2022. link 67 Meer E, Nguyen BJ, Choi DJ, O'Brien JM, Addis VM, Tapino PJ. Assessing the efficacy of 3D-printed ear protectors on mask adherence at an academic ophthalmology center. Indian journal of ophthalmology 2022. link 68 Busse P, Kaplan A. Specific Targeting of Plasma Kallikrein for Treatment of Hereditary Angioedema: A Revolutionary Decade. The journal of allergy and clinical immunology. In practice 2022. link 69 Powell J, Piszczatoski C, Rubido E. Orladeyo (Berotralstat): A Novel Oral Therapy for the Prevention of Hereditary Angioedema. The Annals of pharmacotherapy 2022. link 70 Nabilou S, Pak F, Alizadeh Z, Fazlollahi MR, Houshmand M, Ayazi M et al.. Genetic Study of Hereditary Angioedema Type I and Type II (First Report from Iranian Patients: Describing Three New Mutations). Immunological investigations 2022. link 71 Gramstad OR, Kandanur SPS, Etscheid M, Nielsen EW, Kanse SM. Factor VII activating protease (FSAP) is not essential in the pathophysiology of angioedema in patients with C1 inhibitor deficiency. Molecular immunology 2022. link 72 Bork K, Wulff K, Witzke G, Hardt J, Meinke P. Inheritance Pattern of Hereditary Angioedema Indicates Mutation-Dependent Selective Effects During Early Embryonic Development. The journal of allergy and clinical immunology. In practice 2022. link 73 Pawaskar D, Chen X, Glassman F, May F, Roberts A, Biondo M et al.. Pharmacokinetic/pharmacodynamic modeling for dose selection for the first-in-human trial of the activated Factor XII inhibitor garadacimab (CSL312). Clinical and translational science 2022. link 74 Riedl MA, Johnston DT, Anderson J, Meadows JA, Soteres D, LeBlanc SB et al.. Optimization of care for patients with hereditary angioedema living in rural areas. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2022. link 75 Wilkerson RG, Winters ME. Angiotensin-Converting Enzyme Inhibitor-Induced Angioedema. Emergency medicine clinics of North America 2022. link 76 Strain A. Angioedema of the tongue due to haloperidol. The American journal of emergency medicine 2022. link 77 Kiani-Alikhan S, Walker E, Hickey A, Grigoriadou S, Buckland M, Scott C. Measurement of C1-Inhibitor function alone is sufficient for diagnosis of hereditary angioedema. Journal of clinical pathology 2022. link 78 Banerji A, Bernstein JA, Johnston DT, Lumry WR, Magerl M, Maurer M et al.. Long-term prevention of hereditary angioedema attacks with lanadelumab: The HELP OLE Study. Allergy 2022. link 79 Baeza ML, González-Quevedo T, Caballero T, Guilarte M, Lleonart R, Varela S et al.. Angioedema Due to Acquired Deficiency of C1-Inhibitor: A Cohort Study in Spain and a Comparison With Other Series. The journal of allergy and clinical immunology. In practice 2022. link 80 Minafra FG, Gonçalves TR, Alves TM, Pinto JA. The Mortality from Hereditary Angioedema Worldwide: a Review of the Real-World Data Literature. Clinical reviews in allergy & immunology 2022. link 81 Whitehead WJ, Reid JM. Underreported Risk of Lisinopril-Induced Angioedema in a Veteran Population. The Annals of pharmacotherapy 2022. link 82 Bindke G, Gehring M, Wieczorek D, Kapp A, Buhl T, Wedi B. Identification of novel biomarkers to distinguish bradykinin-mediated angioedema from mast cell-/histamine-mediated angioedema. Allergy 2022. link 83 Wilkerson RG, Moellman JJ. Hereditary Angioedema. Emergency medicine clinics of North America 2022. link 84 Kamata K. Respiratory Monitoring During Gamma Knife Radiosurgery: Anesthesiological Aspects. Acta neurochirurgica. Supplement 2021. link 85 Ohsawa I, Honda D, Suzuki Y, Fukuda T, Kohga K, Morita E et al.. Oral berotralstat for the prophylaxis of hereditary angioedema attacks in patients in Japan: A phase 3 randomized trial. Allergy 2021. link 86 Iwamoto K, Yamamoto B, Ohsawa I, Honda D, Horiuchi T, Tanaka A et al.. The diagnosis and treatment of hereditary angioedema patients in Japan: A patient reported outcome survey. Allergology international : official journal of the Japanese Society of Allergology 2021. link 87 Jindal AK, Rawat A, Kaur A, Sharma D, Suri D, Gupta A et al.. Novel SERPING1 gene mutations and clinical experience of type 1 hereditary angioedema from North India. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2021. link 88 Riedl MA, Banerji A, Gower R. Current medical management of hereditary angioedema: Follow-up survey of US physicians. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2021. link 89 Lai Y, Zhang G, Inhaber N, Bernstein JA, Cwik M, Zhou Z et al.. A robust multiplexed assay to quantify C1-inhibitor, C1q, and C4 proteins for in vitro diagnosis of hereditary angioedema from dried blood spot. Journal of pharmaceutical and biomedical analysis 2021. link 90 Forjaz MJ, Ayala A, Caminoa M, Prior N, Pérez-Fernández E, Caballero T. HAE-AS: A Specific Disease Activity Scale for Hereditary Angioedema With C1-Inhibitor Deficiency. Journal of investigational allergology & clinical immunology 2021. link 91 Campos RA, Valle SOR, Toledo EC. Hereditary angioedema: a disease seldom diagnosed by pediatricians. Jornal de pediatria 2021. link 92 Wang K, Geiger H, McMahon A. Tranexamic acid for ACE inhibitor induced angioedema. The American journal of emergency medicine 2021. link 93 Lumry WR, Weller K, Magerl M, Banerji A, Longhurst HJ, Riedl MA et al.. Impact of lanadelumab on health-related quality of life in patients with hereditary angioedema in the HELP study. Allergy 2021. link 94 Veronez CL, Mendes AR, Leite CS, Gomes CP, Grumach AS, Pesquero JB. The Panorama of Primary Angioedema in the Brazilian Population. The journal of allergy and clinical immunology. In practice 2021. link 95 Cesoni Marcelli A, Loffredo S, Petraroli A, Carucci L, Mormile I, Ferrara AL et al.. Nailfold Videocapillaroscopy Findings in Bradykinin-Mediated Angioedema. Journal of investigational allergology & clinical immunology 2021. link 96 Pólai Z, Balla Z, Andrási N, Kőhalmi KV, Temesszentandrási G, Benedek S et al.. A follow-up survey of patients with acquired angioedema due to C1-inhibitor deficiency. Journal of internal medicine 2021. link 97 Zuraw B, Lumry WR, Johnston DT, Aygören-Pürsün E, Banerji A, Bernstein JA et al.. Oral once-daily berotralstat for the prevention of hereditary angioedema attacks: A randomized, double-blind, placebo-controlled phase 3 trial. The Journal of allergy and clinical immunology 2021. link 98 Wang Y, Jomphe C, Marier JF, Martin P. Population Pharmacokinetics and Exposure-Response Analyses to Guide Dosing of Icatibant in Pediatric Patients With Hereditary Angioedema. Journal of clinical pharmacology 2021. link 99 Lipton G, Stewart M, McDermid R, Docking R, Urquhart C, Morrison M et al.. Multispecialty tracheostomy experience. Annals of the Royal College of Surgeons of England 2020. link 100 Lo YL, Wu HT, Lin YT, Kuo HP, Lin TY. Hypoventilation patterns during bronchoscopic sedation and their clinical relevance based on capnographic and respiratory impedance analysis. Journal of clinical monitoring and computing 2020. link

Original source

[1]
Association between short-term exposure to ultrafine particles from aviation and mortality in the proximity of Amsterdam Airport Schiphol in the Netherlands. — Ndiaye A, Klompmaker J, Houthuijs D, Hoek G, Janssen N Environmental research (2026)
[2]
Burden of hereditary angioedema: results from a multinational survey of caregivers for adult and pediatric patients. — Watt M, Martinez-Saguer I, Simon A, Murphy R, De La Cruz M, Zwiener R et al. Orphanet journal of rare diseases (2026)
[3]
ACE inhibitor-induced bradykinin-mediated angioedema presenting as small bowel obstruction and life-threatening airway angioedema: a rare dual presentation. — Ghimire P, Sathya DA, Tang A, Yaqoob H BMJ case reports (2026)
[4]
Novel SERPING1 Genetic Variant in Two Family Members with Hereditary Angioedema. — Cosme Ferreira S, Rosa A, Sousa F, Mendoza-Alvarez A, González-Montelongo R, Callero A et al. Acta medica portuguesa (2026)
[5]
Donidalorsen: First Approval. — Syed YY Drugs (2026)
[6]
Oral deucrictibant for prophylaxis of hereditary angioedema attacks (CHAPTER-1): primary analysis of a randomised, double-blind, placebo-controlled, phase 2 trial. — Aygören-Pürsün E, Stobiecki M, Valerieva A, Cancian M, Gompels M, Grigoriadou S et al. The Lancet. Haematology (2026)
[7]
Burden of disease in adult patients with hereditary angioedema: results from a multinational survey. — Watt M, Martinez-Saguer I, Simon A, Murphy R, Cruz M, Zwiener R et al. Orphanet journal of rare diseases (2026)
[8]
A Treat-to-Target approach in hereditary angioedema: expert consensus from a European committee. — Cancian M, Caballero T, Boccon-Gibod I, Buttgereit T, Cohn D, Eren E et al. Frontiers in immunology (2026)
[9]
Characterization of a unique pathogenic variant in the SERPING1 gene of a patient with hereditary angioedema type I. — Hisamoto T, Sentoku K, Shimomura Y Journal of dermatological science (2026)
[10]
Long-term prophylactic treatment preferences and willingness to switch therapy in individuals with hereditary angioedema. — Olson C, Lionetti M, Poulos C, Ray T, Lopez-Gonzalez L, Nestler-Parr S et al. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology (2026)
[11]
Investigation of sexual life and sleep quality in patients with hereditary angioedema. — Aykan FS, Bakay H, Çölkesen F, Gıca Ş, Kılınç M, Recep E et al. Allergologia et immunopathologia (2026)
[12]
Evaluating Risk Factors for Severe Renin-Angiotensin-Aldosterone System Inhibitor Induced Angioedema. — Bindra G, Pattanaik D, Sheyn A, Gillespie MB, Shrestha R, Kadaria D et al. The Laryngoscope (2026)
[13]
Epidemiological and clinical characteristics of hereditary angioedema in the Baltic states. — Kurjane N, Kanepa A, Purina S, Lapina L, Ress K, Savisaar M et al. Frontiers in immunology (2026)
[14]
Oral deucrictibant for on-demand treatment of hereditary angioedema attacks (RAPIDe-1): a randomised, double-blind, placebo-controlled, phase 2 trial. — Maurer M, Stobiecki M, Valerieva A, Hakl R, Staevska MT, Bouillet L et al. The Lancet. Haematology (2026)
[15]
Long-Term Real-World Outcomes in Patients with Hereditary Angioedema Receiving Lanadelumab for 3 or More Years. — Lumry WR, Davis-Lorton M, Soteres D, Earl L, Wynne-Cattanach K, Fox D et al. International archives of allergy and immunology (2026)
[16]
Therapeutic Potential of C1-Inhibitor in Vascular Diseases and Beyond. — Sundler Björkman L, Eswaran H, Grover SP Arteriosclerosis, thrombosis, and vascular biology (2026)
[17]
Eosinophilic inflammation in hereditary angioedema: a single-center real-world retrospective chart review study. — Boch K, Ludwig RJ, von Bubnoff D, Recke A Frontiers in immunology (2026)
[18]
Updates in hereditary angioedema and chronic spontaneous urticaria. — Gandhi RS, Bruun TUJ, Bernstein JA The Journal of allergy and clinical immunology (2026)
[19]
Transmission of F12-related hereditary angioedema through a sperm donor. — Marquès L, Batlle-Masó L, Alcoceba E, Lara S, Aguiló-Cucurull A, Gil-Serrano J et al. Frontiers in immunology (2026)
[20]
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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 et al. International archives of allergy and immunology (2026)
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Glycocalyx strengthens endothelial barrier function and protects from angioedema inducing compounds. — Reich A, Lehnert E, Möhrle R, Gierke A, Brunner C, Hoffmann TK et al. Frontiers in immunology (2026)
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Classification of angioedema types using decision tree modeling. — Aulenbacher F, Gutsche A, Farkas H, Kőhalmi KV, Kocatürk E, Aygören-Pürsün E et al. Frontiers in immunology (2025)
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Analysis of disease burden in patients with hereditary angioedema from Japan by patient-reported outcomes. — Hide M, Kishimoto M, Kotera I, Oh A, Inoue Y, Yamamoto BA et al. The Journal of dermatology (2025)
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New drugs for the treatment of hereditary angioedema. — Costanzo G, Sambugaro G, Sartorio S, Zanichelli A, Firinu D Expert opinion on biological therapy (2025)
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Real-World Effectiveness of Lanadelumab in Hereditary Angioedema: Multicountry INTEGRATED Observational Study. — Magerl M, Bouillet L, Martinez-Saguer I, Gavini F, Bent-Ennakhil N, Sayegh L et al. The journal of allergy and clinical immunology. In practice (2025)
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Functional physiological, psychological, and biochemical reactivity to socially evaluated cold pressor test in hereditary angioedema patients (FRoSEn). — De Maria B, Ranucci L, Gino C, Cesoni Marcelli A, Zingale LC, Zulueta A et al. Frontiers in immunology (2025)
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Lip angioedema after indirect contact with a sea anemone. — Lialiaris S, Fyrmpas G, Katsilidou M, Vitsa D, Katotomihelakis M Ear, nose, & throat journal (2024)
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Hereditary angioedema plasma proteomics following specific plasma kallikrein inhibition with lanadelumab. — Sexton D, Kichev A, Juethner S, Yeung D, MacDonald A, Anokian E et al. Frontiers in immunology (2024)
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Airway Involvement and Intervention in Non-ACE-Inhibitor-Induced Angioedema. — Bacak BJ, Castle MS, Barbot C, Srikantha L, Stern NA, Vandjelovic ND The Laryngoscope (2024)
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A phase 2 open-label extension study of prekallikrein inhibition with donidalorsen for hereditary angioedema. — Petersen RS, Bordone L, Riedl MA, Tachdjian R, Craig TJ, Lumry WR et al. Allergy (2024)
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Phenotypic and Genotypic Characterization of Hereditary Angioedema in Saudi Arabia. — Sheikh F, Alajlan H, Albanyan M, Alruwaili H, Alawami F, Sumayli S et al. Journal of clinical immunology (2023)
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Efficacy of human C1 esterase inhibitor concentrate for treatment of ACE-inhibitor induced angioedema. — Strassen U, Bas M, Wirth M, Wirth M, Gröger M, Stelter K et al. The American journal of emergency medicine (2023)
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Hereditary Angioedema During Pregnancy: Considerations in Management. — Riedl MA Immunology and allergy clinics of North America (2023)
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A Case of Refractory Angioedema. — Irwin TM, Irwin TM, Longanecker A, Bodenhamer WS, Keirns CC Journal of intensive care medicine (2023)
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Anesthetic management of a patient with acquired angioedema submitted to broncofibroscopy: a case report. — Barata M, Marques A, Tiago C, Dias-Vaz M, Losa N, Oliveira C Brazilian journal of anesthesiology (Elsevier) (2023)
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A review of berotralstat for the treatment of hereditary angioedema. — Farkas H, Balla Z Expert review of clinical immunology (2023)
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Trends in Treatments With Disease-Specific and Interfering Drugs in Patients With Hereditary Angioedema in Sweden. — Sundler Björkman L, Thulin M, Ekström M, Nordenfelt P, Egesten A The journal of allergy and clinical immunology. In practice (2023)
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Athena Speciality Certificate Examination case for cutaneous allergy. — Harnett C, Drumm C, Meah N, Eustace K Clinical and experimental dermatology (2022)
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Assessing the efficacy of 3D-printed ear protectors on mask adherence at an academic ophthalmology center. — Meer E, Nguyen BJ, Choi DJ, O'Brien JM, Addis VM, Tapino PJ Indian journal of ophthalmology (2022)
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Orladeyo (Berotralstat): A Novel Oral Therapy for the Prevention of Hereditary Angioedema. — Powell J, Piszczatoski C, Rubido E The Annals of pharmacotherapy (2022)
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Genetic Study of Hereditary Angioedema Type I and Type II (First Report from Iranian Patients: Describing Three New Mutations). — Nabilou S, Pak F, Alizadeh Z, Fazlollahi MR, Houshmand M, Ayazi M et al. Immunological investigations (2022)
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Optimization of care for patients with hereditary angioedema living in rural areas. — Riedl MA, Johnston DT, Anderson J, Meadows JA, Soteres D, LeBlanc SB et al. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology (2022)
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Angioedema of the tongue due to haloperidol. — Strain A The American journal of emergency medicine (2022)
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Underreported Risk of Lisinopril-Induced Angioedema in a Veteran Population. — Whitehead WJ, Reid JM The Annals of pharmacotherapy (2022)
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Identification of novel biomarkers to distinguish bradykinin-mediated angioedema from mast cell-/histamine-mediated angioedema. — Bindke G, Gehring M, Wieczorek D, Kapp A, Buhl T, Wedi B Allergy (2022)
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The diagnosis and treatment of hereditary angioedema patients in Japan: A patient reported outcome survey. — Iwamoto K, Yamamoto B, Ohsawa I, Honda D, Horiuchi T, Tanaka A et al. Allergology international : official journal of the Japanese Society of Allergology (2021)
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A robust multiplexed assay to quantify C1-inhibitor, C1q, and C4 proteins for in vitro diagnosis of hereditary angioedema from dried blood spot. — Lai Y, Zhang G, Inhaber N, Bernstein JA, Cwik M, Zhou Z et al. Journal of pharmaceutical and biomedical analysis (2021)
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Hereditary angioedema: a disease seldom diagnosed by pediatricians. — Campos RA, Valle SOR, Toledo EC Jornal de pediatria (2021)
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Tranexamic acid for ACE inhibitor induced angioedema. — Wang K, Geiger H, McMahon A The American journal of emergency medicine (2021)
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Impact of lanadelumab on health-related quality of life in patients with hereditary angioedema in the HELP study. — Lumry WR, Weller K, Magerl M, Banerji A, Longhurst HJ, Riedl MA et al. Allergy (2021)
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The Panorama of Primary Angioedema in the Brazilian Population. — Veronez CL, Mendes AR, Leite CS, Gomes CP, Grumach AS, Pesquero JB The journal of allergy and clinical immunology. In practice (2021)
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Population Pharmacokinetics and Exposure-Response Analyses to Guide Dosing of Icatibant in Pediatric Patients With Hereditary Angioedema. — Wang Y, Jomphe C, Marier JF, Martin P Journal of clinical pharmacology (2021)
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