ReviewClinical and translational allergy2022
Reviewing clinical considerations and guideline recommendations of C1 inhibitor prophylaxis for hereditary angioedema.
Review in Clinical and translational allergy, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 14 citations in OpenAlex.
- Long-term prophylaxis in hereditary angioedema: Real-world treatment patterns and healthcare resource utilization.The World Allergy Organization journal · 2026Article
- Hereditary Angioedema Prophylaxis Therapy: Berotralstat and Lanadelumab Safety Profile.Medicina (Kaunas, Lithuania) · 2025Article
- A multi-centre UK-based survey on angioedema secondary to acquired C1 inhibitor deficiency.Clinical and experimental immunology · 2025Article
- Factor XII Structure-Function Relationships.Seminars in thrombosis and hemostasis · 2024Review
- The double whammy of ER-retention and dominant-negative effects in numerous autosomal dominant diseases: significance in disease mechanisms and therapy.Journal of biomedical science · 2024Review
- Dietary and physical trigger factors in hereditary angioedema: Self-conducted investigation and literature overview.Allergologie select · 2024Article
- Pathogenic variant inBMJ case reports · 2023Article
- Hereditary Angioedema.Cureus · 2023Article
- Plasma Kallikrein Inhibitors as Potential Treatment for Diabetic Macular Edema, Retinal Vein Occlusion, Hereditary Angioedema and Other Related Diseases.ACS medicinal chemistry letters · 2023Article
- Reviewing clinical considerations and guideline recommendations of C1 inhibitor prophylaxis for hereditary angioedema.Clinical and translational allergy · 2022Review
- Safety Aspects and Rational Use of Lanadelumab Injections in the Treatment of Hereditary Angioedema (HAE): Clinical Insights.Drug, healthcare and patient safety · 2022Review
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHereditary angioedema (HAE), a rare disease that is characterized by painful and recurring non-allergic swelling episodes, is caused by the deficiency or dysfunction of C1 inhibitor (C1INH) protein. A comprehensive HAE management plan may require long-term prophylaxis (LTP) in addition to on-demand treatment to help "normalize" patients' lives so that they may fully engage in work, school, family, and leisure activities.
aimThe main objective of this narrative review is to provide an overview of updated guideline recommendations specific to LTP of HAE and discuss clinical considerations and pharmacologic management options, with a focus on C1INH. MATERIALS AND
methodsThe authors reviewed relevant HAE literature for current recommendations regarding LTP and the role of C1NH.
resultsAcute HAE attacks are treated with on-demand medication; however, there is a consensus that LTP should routinely be considered for risk reduction and prevention of future episodes. The 2017 World Allergy Organization/European Academy of Allergy and Clinical Immunology guidelines recommend that all patients with HAE be evaluated for LTP routinely and the 2020 HAE Association (HAEA) guidelines emphasize that the decision to use LTP should not be based on rigid criteria, but rather should be based on individual patient needs. Both guidelines recommend C1INH as first-line/preferred therapy for LTP in a range of patient types including adults, children/adolescents, and pregnant/lactating patients. The HAEA also recommends the kallikrein inhibitor, lanadelumab, as a first-line option for LTP. HAE pathway-specific agents for LTP have not been associated with notable safety concerns. DISCUSSION: Plasma-derived C1INH has been available for 40+ years in Europe and impacts multiple targets within the HAE pathway. C1INH has been used for on-demand treatment and LTP. A subcutaneous formulation of plasma-derived C1INH is approved for LTP and produces functional C1INH activity levels consistently above the threshold needed for protection from HAE attacks. Other pathway-specific options for LTP include the plasma kallikrein inhibitors, lanadelumab-flyo and berotralstat, approved for adults and pediatric patients aged ≥12 years. C1INH is approved for adults and pediatric patients aged ≥6 years.
conclusionAssessing the need for LTP is vital in the ongoing dialogue between clinicians and patients, as both disease-related factors and patient preferences may change over time. Among available options for LTP, plasma-derived C1INH is the broadly recommended first-line option for LTP in patients with HAE, including pregnant/lactating women and pediatric patients (≥6 years).
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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.