ArticleThe Journal of allergy and clinical immunology2004
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.
Article in The Journal of allergy and clinical immunology, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 187 papers, 4 of them syntheses that pooled it.
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Who cites it
187 citing papers in PubMed, 4 syntheses or guidelines pooled it, 643 citations in OpenAlex.
- Prevalence and Incidence of Hereditary Angioedema: A Systematic Literature Review.International archives of allergy and immunology · 2026Pooled it
- Epidemiology, economic, and humanistic burden of hereditary angioedema: a systematic review.Orphanet journal of rare diseases · 2024Pooled it
- Brazilian Guidelines for Hereditary Angioedema Management - 2017 Update Part 1: Definition, Classification and Diagnosis.Clinics (Sao Paulo, Brazil) · 2018Guideline
- Brazilian guidelines for the diagnosis and treatment of hereditary angioedema.Clinics (Sao Paulo, Brazil) · 2011Guideline
- COVID-19 as a trigger of acute attacks in people with hereditary angioedema.Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology · 2021Trial
- A randomized trial of human C1 inhibitor prophylaxis in children with hereditary angioedema.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2019Trial
- Functional Complement Analysis Can Predict Genetic Testing Results and Long-Term Outcome in Patients With Complement Deficiencies.Frontiers in immunology · 2018Trial
- Risk of angioedema following invasive or surgical procedures in HAE type I and II--the natural history.Allergy · 2013Trial
- Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema.Journal of clinical immunology · 2010Trial
- Anti-cholesterol antibody levels in hereditary angioedema.Journal of cellular and molecular medicineTrial
- Burden of Hereditary Angioedema in Adults: Impact of Disease and Patient Characteristics.International archives of allergy and immunology · 2026Article
- Hereditary angioedema and post traumatic stress disorder: a reciprocal relationship?Orphanet journal of rare diseases · 2026Article
- The 2025 WAO Guidelines for the classification, diagnosis, and treatment of hereditary angioedema, with consideration of worldwide disparities.The World Allergy Organization journal · 2026Article
- Elevated serum zonulin is associated with high attack frequency in hereditary angioedema: providing insight into the gut-angioedema axis.Orphanet journal of rare diseases · 2026Article
- Burden of disease in adult patients with hereditary angioedema: results from a multinational survey.Orphanet journal of rare diseases · 2026Article
- The International/Canadian hereditary angioedema guideline.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2026Review
- Treatment of Hereditary Angioedema With Plasma-Derived C1 Inhibitor: A Review.Clinical and translational allergy · 2025Review
- Hereditary angioedema: A national investigation of associated comorbidities and surgical procedures.The World Allergy Organization journal · 2025Article
- Hereditary angioedema diagnosis: Reflecting on the past, envisioning the future.The World Allergy Organization journal · 2025Review
- Hereditary angioedema diagnosis evaluation score (HADES): A new clinical scoring system for predicting hereditary angioedema with C1 inhibitor deficiency.The journal of allergy and clinical immunology. Global · 2025Article
127 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
58 authors at 4 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.