ReviewClinical reviews in allergy & immunology2025
Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment.
Review in Clinical reviews in allergy & immunology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers, 3 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
41 citing papers in PubMed, 3 syntheses or guidelines pooled it.
- International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema.Allergy · 2026Guideline
- Self-reported perspective in rare genetic diseases: a systematic review of patient-reported outcome measures classified using the international classification of functioning, disability and health framework.Journal of patient-reported outcomes · 2026Pooled it
- Prevalence and Incidence of Hereditary Angioedema: A Systematic Literature Review.International archives of allergy and immunology · 2026Pooled it
- Long-term prophylactic treatment and related real-world outcomes of patients with hereditary angioedema in Germany: A retrospective analysis of German claims and medical chart data.The World Allergy Organization journal · 2026Article
- Beyond the Index Case: A Practical Framework for Cascade Family Screening in Hereditary Angioedema.Clinical reviews in allergy & immunology · 2026Review
- [What are angioedemas and how are they classified?]Dermatologie (Heidelberg, Germany) · 2026Review
- Beyond Swelling: Clinical Insights into the Diagnosis and Management of Hereditary Angioedema.International journal of molecular sciences · 2026Review
- Transient C1-Inhibitor Functional Abnormalities During Acute Idiopathic Pancreatitis: A Diagnostic Challenge in Hereditary Angioedema Evaluation.Digestive diseases and sciences · 2026Article
- Lanadelumab Use for Hereditary Angioedema Long-Term Prophylaxis Over the Last 7 Years: A Narrative Review of Clinical and Real-World Data.Clinical reviews in allergy & immunology · 2026Review
- Algorithms in Allergy: Hereditary Angioedema.Allergy · 2026Article
- Article
- Clinical expressions, disease course, quality of life, and resilience in subgroups of patients with angioedema.The World Allergy Organization journal · 2026Article
- Therapeutic Targeting of the Bradykinin B2 Receptor in Immunological and Vascular Diseases: Insights from Kinin Biology to Clinical Outcomes.Clinical reviews in allergy & immunology · 2026Review
- Unveiling Rare Genetic Variants in DAB2IP: New Insights Into the Pathogenesis of Recurrent Angioedema.Allergy · 2026Article
- Article
- Acquired Angioedema-A Challenge in Medical Practice: A Narrative Review.Journal of clinical medicine · 2026Review
- An International Delphi Study on Barriers to On-Demand Treatment of Hereditary Angioedema Attacks.Clinical and translational allergy · 2026Article
- Hemophilia and hereditary angioedema: parallel therapeutic advances in genetic diseases of serine protease pathways.Research and practice in thrombosis and haemostasis · 2026Review
- Developing a patient journey map to improve care and experience in Chinese patients with hereditary angioedema.The World Allergy Organization journal · 2026Article
- The power of a pill: Ekterly (Sebetralstat): first oral on-demand therapy for hereditary angioedema-a paradigm shift in treatment.Annals of medicine and surgery (2012) · 2026Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
35 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary angioedema (HAE) has been recognized for almost 150 years. The newest form of HAE, where C1 inhibitor levels are normal (HAE-nC1INH), was first described in 2000. Over the last two decades, new types of apparent non-mast cell-mediated angioedema with normal quantity and activity of C1INH have been described, in some cases with proven genetic pathogenic variants that co-segregate with angioedema expression within families. Like HAE due to C1INH deficiency, HAE-nC1INH patients are at risk of serious morbidity and mortality. Therefore, proactive management and treatment of HAE-nC1INH patients after an expert physician diagnosis is critically important. The underlying pathophysiology responsible for the angioedema has also been clarified in some of the HAE-nC1INH types. While several clinical guidelines and practice parameters including HAE-nC1INH have been published, we have made substantial progress in our understanding encompassing diagnostic criteria, pathophysiology, and treatment outcomes. HAE International (HAEi) and the US HAE Association (HAEA) convened a symposium of global HAE-nC1INH experts to synthesize our current knowledge in the area. Given the paucity of high-level evidence in HAE-nC1INH, all recommendations are based on expert opinion. This review and expert opinion on the best practice approach to diagnosing and treating HAE-nC1INH will support physicians to better manage patients with HAE-nC1INH.
Indexed as
Identifiers
What OpenQuestion holds
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.