ReviewClinical reviews in allergy & immunology2019
Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema.
Review in Clinical reviews in allergy & immunology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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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.
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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.
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Who cites it
23 citing papers in PubMed, 41 citations in OpenAlex.
- C1-esterase Inhibitor Treatment is Associated with Immune and Vascular Pathway Modulation in PASC with Neurological Symptoms: Longitudinal Plasma Proteomics.Cellular and molecular neurobiology · 2026Trial
- Randomized phase I trial outcomes show safe and sustainable inhibition of classical and lectin complement pathways by empasiprubart.Nature communications · 2025Trial
- Structure and function of therapeutic antibodies approved by the US FDA in 2025.Antibody therapeutics · 2026Review
- Association of muscle instability and long-term prophylaxis in hereditary angioedema.The World Allergy Organization journal · 2026Article
- Therapeutic Advances in Hereditary Angioedema: A Focus on Present and Future Options.Advances in therapy · 2025Review
- A Case of Severe Cutaneous Anthrax: Malignant Edema Leading to Laryngeal Involvement.Infectious diseases & clinical microbiology · 2025Article
- Comorbidities in Canadian patients with hereditary angioedema: a quantitative survey study.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2025Article
- Unveiling the Complexities of Hereditary Angioedema.Biomolecules · 2024Review
- Oxidative Stress: A Culprit in the Progression of Diabetic Kidney Disease.Antioxidants (Basel, Switzerland) · 2024Review
- Hereditary angioedema (HAE) in children and adolescents: New treatment options.Allergologie select · 2024Review
- Hereditary angioedema is associated with an increased risk of venous thromboembolism.Journal of thrombosis and haemostasis : JTH · 2022Article
- Article
- The international WAO/EAACI guideline for the management of hereditary angioedema - The 2021 revision and update.The World Allergy Organization journal · 2022Article
- Clinical manifestations of hereditary angioedema and a systematic review of treatment options.Laryngoscope investigative otolaryngology · 2021Review
- Serpins in cartilage and osteoarthritis: what do we know?Biochemical Society transactions · 2021Review
- Pharmacotherapeutic Patterns and Patients' Access to Pharmacotherapy for Some Rare Diseases in Bulgaria - A Pilot Comparative Study.Frontiers in pharmacology · 2021Article
- Is the COVID-19 thrombotic catastrophe complement-connected?Journal of thrombosis and haemostasis : JTH · 2020Review
- C1 esterase inhibitor and the contact system in COVID-19.British journal of haematology · 2020Review
- Recent advances in use of fresh frozen plasma, cryoprecipitate, immunoglobulins, and clotting factors for transfusion support in patients with hematologic disease.Seminars in hematology · 2020Review
- Complement, a Therapeutic Target in Diabetic Kidney Disease.Frontiers in medicine · 2020Review
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In the vast majority of patients with hereditary angioedema (HAE), angioedema attacks are due to the quantitative or functional deficiency of C1-esterase inhibitor (C1-INH), which leads to increased vascular permeability and unregulated release of bradykinin. Exogenous administration of C1-INH is a rational way to restore the concentration and functional activity of this protein, regulate the release of bradykinin, and attenuate or prevent subcutaneous and submucosal edema associated with HAE. Recent international guidelines for the management of HAE include C1-INH as an option for acute treatment of HAE. In addition, these guidelines recommend C1-INH as first-line treatment for long-term prophylaxis and as the therapy of choice for short-term/preprocedural prophylaxis. Several C1-INH products are available, with approved indications varying across regions. For the acute treatment of HAE, both plasma-derived and recombinant C1-INH formulations have been shown to be effective and well tolerated in adolescents and adults with HAE, with onset of relief within 30 min to a few hours. Plasma-derived C1-INH is approved for use in children, and recombinant C1-INH is being evaluated in this population. Intravenous (IV) and subcutaneous (SC) formulations of C1-INH have been approved for routine prophylaxis to prevent HAE attacks in adolescents and adults. Both formulations when administered twice weekly have been shown to reduce the frequency and severity of HAE attacks. The SC formulation of C1-INH obviates the need for repeated venous access and may facilitate self-administration of HAE prophylaxis at home, as recommended in HAE treatment guidelines. As with most rare diseases, the costs of HAE treatment are high; however, the development of additional acute and prophylactic medications for HAE may result in competitive pricing and help drive down the costs of HAE treatment.
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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.