Risk of thromboembolism in patients with hereditary angioedema treated with plasma-derived C1-inhibitor

Farkas, Henriette; Veszeli, Nóra; Zotter, Zsuzsanna; Várnai, Katalin

doi:10.2500/aap.2016.37.3933

Cited by 32 publications

(12 citation statements)

References 20 publications

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“…Thromboembolic events due to C1‐INH concentrate use in HAE are rare, and patients who experience such events often have underlying thromboembolic risk factors (eg, implanted central venous catheters . There are no known interactions with other medicinal products.…”

Section: Therapymentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

Allergy

470

974

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Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up‐to‐date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1‐inhibitor (type 1) and HAE with dysfunctional C1‐inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE‐1/2 be defined and classified?, (2) How should HAE‐1/2 be diagnosed?, (3) Should HAE‐1/2 patients receive prophylactic and/or on‐demand treatment and what treatment options should be used?, (4) Should HAE‐1/2 management be different for special HAE‐1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE‐1/2 management incorporate self‐administration of therapies and patient support measures?

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Section: Therapymentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

Allergy

470

974

View full text Add to dashboard Cite

show abstract

“…In practice, hereditary angioedema itself is not known to be associated with clinical thrombosis or bleeding tendency, despite in vitro abnormality. Exogenous plasma-derived C1 inhibitor has been associated with thrombosis in vivo at both therapeutic and supra-therapeutic doses in some but not all series ( 79 – 80 ). Use of in-dwelling catheters may act synergistically to increase risk of thrombosis and should be avoided ( 81 ).…”

Section: Resultsmentioning

confidence: 99%

Optimum Use of Acute Treatments for Hereditary Angioedema: Evidence-Based Expert Consensus

Longhurst

2018

Front. Med.

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Acute treatment of hereditary angioedema due to C1 inhibitor deficiency has become available in the last 10 years and has greatly improved patients’ quality of life. Two plasma-derived C1 inhibitors (Berinert and Cinryze), a recombinant C1 inhibitor (Ruconest/Conestat alpha), a kallikrein inhibitor (Ecallantide), and a bradykinin B2 receptor inhibitor (Icatibant) are all effective. Durably good response is maintained over repeated treatments and several years. All currently available prophylactic agents are associated with breakthrough attacks, therefore an acute treatment plan is essential for every patient. Experience has shown that higher doses of C1 inhibitor than previously recommended may be desirable, although only recombinant C1 inhibitor has been subject to full dose–response evaluation. Treatment of early symptoms of an attack, with any licensed therapy, results in milder symptoms, more rapid resolution and shorter duration of attack, compared with later treatment. All therapies have been shown to be well-tolerated, with low risk of serious adverse events. Plasma-derived C1 inhibitors have a reassuring safety record regarding lack of transmission of virus or other infection. Thrombosis has been reported in association with plasma-derived C1 inhibitor in some case series. Ruconest was associated with anaphylaxis in a single rabbit-allergic volunteer, but no further anaphylaxis has been reported in those not allergic to rabbits despite, in a few cases, prior IgE sensitization to rabbit or milk protein. Icatibant is associated with high incidence of local reactions but not with systemic effects. Ecallantide may cause anaphylactoid reactions and is given under supervision. For children and pregnant women, plasma-derived C1 inhibitor has the best evidence of safety and currently remains first-line treatment.

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“…68 Thromboembolic events due to pdC1-INH concentrate use in HAE are rare, and patients who experience such events often have underlying thromboembolic risk factors. 69…”

Section: Iii-long-term Prophylaxismentioning

confidence: 99%

Updates on hereditary angioedema in pediatrics

El‐Ghoneimy

2019

Egypt J Pediatr Allergy Immunol

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Risk of thromboembolism in patients with hereditary angioedema treated with plasma-derived C1-inhibitor

Abstract: Our cohort study did not find any evidence for an increased risk of thromboembolism during treatment with pdC1-INH, despite the presence of multiple predisposing factors.

Cited by 32 publications

References 20 publications

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Optimum Use of Acute Treatments for Hereditary Angioedema: Evidence-Based Expert Consensus

Updates on hereditary angioedema in pediatrics

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