Safety of a C1-inhibitor concentrate in pregnant women with hereditary angioedema

Fox, J A; Vegh, Arthur B.; Martinez‐Saguer, Inmaculada; Wuillemin, Walter A.; Edelman, Jonathan M.; Williams-Herman, Debora; Rojavin, Mikhail; Rosenberg, Tanja

doi:10.2500/aap.2017.38.4038

Cited by 31 publications

(31 citation statements)

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“…C1‐INH concentrate is recommended as first‐line therapy for pregnant or breastfeeding HAE‐1/2 patients as it is safe and effective . No published experience is available for ecallantide.…”

Section: Management Of Hae‐1/2 During Pregnancy and Lactationmentioning

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

447

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: Management Of Hae‐1/2 During Pregnancy and Lactationmentioning

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

447

974

View full text Add to dashboard Cite

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“…Plasma-derived C1 inhibitor is identical to the patient’s endogenous C1 inhibitor and is the acute treatment of choice during pregnancy ( 83 ). Observational studies provide evidence as to its safety, particularly for Berinert ( 84 – 88 ).…”

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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“…15,16 In these studies, all women delivered infants at full term without complications, and the treatment was generally safe and well tolerated. Further supportive information has come from treatment with plasma-derived C1-INH products during pregnancy for acute HAE attacks 9,23,24 and HAE prophylaxis 25,26 and short-term perinatal prophylaxis. 9,25 Although no controlled studies have been conducted comparing HAE treatment regimens during pregnancy, international guidelines recommend C1-INH concentrate as first-line treatment for acute HAE attacks during pregnancy on the basis of its safety profile.…”

Section: Discussionmentioning

confidence: 99%

Hereditary Angioedema Attack in Utero and Treatment of the Mother and Fetus

Grivcheva‐Panovska

Giannetti

2020

Mayo Clinic Proceedings: Innovations, Quality & Outcomes

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Hereditary angioedema (HAE), an inherited deficiency of functional C1 esterase inhibitor (C1-INH), is characterized by unpredictable recurrent episodes of painful and often disabling swelling in subcutaneous and/or submucosal tissues. We report the case of a 23-year-old woman with type I HAE who had abdominal, facial, and peripheral attacks throughout her first pregnancy. A facial HAE attack occurred at week 38 of her pregnancy, and symptoms improved after self-administration of 50 U/kg of recombinant human C1-INH (total dose, 3500 U), but soon after she had an unusual abdominal sensation. Ultrasonography detected fetal lower lip swelling (w3 times the normal size) and limb swelling. Physical examination of the mother found cervical dilatation, indicating the final stages of labor. Two hours after treatment of her HAE attack, she spontaneously delivered a healthy male infant. Photographs taken within 2 minutes of delivery revealed resolution of the infant's facial edema, and the limb edema was resolved within 30 minutes. By 10 minutes postdelivery, the mother's facial attack had almost completely resolved. Ten months after birth, genetic analysis confirmed that the infant had type I HAE. This is the first documented case of an HAE attack in utero. Treatment of the mother with recombinant human C1-INH was effective for the maternal and fetal attacks, with resolution within approximately 2 to 2.5 hours for both patients.

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Safety of a C1-inhibitor concentrate in pregnant women with hereditary angioedema

Abstract: Administration of pnfC1-INH during pregnancy was generally safe and not associated with any treatment-related AEs. In all registry pregnancies followed up to term, the birth of a healthy baby was reported.

Cited by 31 publications

References 0 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

Hereditary Angioedema Attack in Utero and Treatment of the Mother and Fetus

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