Pregnancy-Induced Exacerbation of Hereditary Angioedema in a Multiparous Caucasian Female

Sankrithi, Praveen; Shah, Kunal; Bernabe, Celina C

doi:10.7759/cureus.8006

Cited by 4 publications

(4 citation statements)

References 9 publications

(16 reference statements)

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“…Infrequently, the manifestations of HAE‐1/2 first occur during pregnancy. Attack frequency observed during previous pregnancies is only in part predictive of that in subsequent ones 317–321 . Pregnant HAE‐1/2 patients require vigilant care and meticulous monitoring by an HAE expert.…”

Section: Management Of Hae‐1/2 In Pregnant and Breastfeeding Patientsmentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

Maurer

Magerl

Betschel

et al. 2022

Allergy

207

295

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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? This article is co-published with permission in Allergy and the World Allergy Organization Journal.

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Section: Management Of Hae‐1/2 In Pregnant and Breastfeeding Patientsmentioning

confidence: 99%

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

Maurer

Magerl

Betschel

et al. 2022

Allergy

207

295

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“…HAE nC1-INH has long been thought to have an estrogen-related component in addition to genetic mutations. High estrogen states, such as contraceptives and pregnancy, have been linked with increased severity and frequency of flares in HAE [ 10 - 11 ]. Increased estrogen has also been shown to exacerbate the disease process in 91% of female patients with HAE nC1-INH with a factor XII mutation [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Hereditary Angioedema with Normal C1 Esterase Inhibitor Refractory to Long-Term Prophylaxis: A Case Report

Cobb¹,

Bernabe²

2023

Cureus

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Hereditary angioedema (HAE) was classically thought to be related to a deficiency in the C1 esterase inhibitor (C1-INH). However, HAE with a normal C1 esterase inhibitor (HAE nC1-INH) is a rare disease that has been recently characterized. In this case, we describe a woman with a 21-year history of angioedema who, in the last two years, has experienced severe exacerbations that led to the formal diagnosis of HAE nC1-INH. The patient has been treated with current HAE long-term prophylaxis (LTP) and on-demand therapies but is still experiencing severe, frequent attacks. Our case describes the timeline of this patient's presentation and provides valuable insight into the presentation and management of HAE nC1-INH.

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“…Interestingly, attacks started in the early pregnancy with a severe presentation of the disease that was not previously present, and resolved immediately after the delivery, leaving the patient symptom free for several months. There are only a few case reports and studies available, which give a detailed description of HAE worsening during the pregnancy, but to our knowledge, non showed such progression despite prophylactic therapy [5][6][7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Successful treatment with Cinryze® replacement therapy of a pregnant patient with hereditary angioedema: a case report

et al. 2021

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Background Hereditary angioedema (HAE) is a rare disease characterized with recurrent swelling of subcutaneous or mucosal tissue that resolves in approximately 3 days. It can be presented with peripheral edema, abdominal and life-threatening laryngeal angioedema. A variety of triggers are known to cause episodes of angioedema including estrogen exposure. There are different reports regarding the effect of pregnancy on HAE attacks, and in some patients, the pregnancy is a recognized triggering factor. Case presentation We present a female Caucasian patient with pre-existing HAE and disease exacerbations during pregnancy, requiring prophylactic use of plasma-derived C1 inhibitor concentrate. She was treated with Cinryze® replacement therapy throughout the pregnancy 1000 IU i.v. 48 times. She gave birth to a healthy male infant, via C-section. After the delivery, the patient was symptom-free for 6 months and required no treatment for HAE. Conclusions In the case presented, the angioedema attacks worsened as the pregnancy progressed. The treatment with Cinryze® replacement therapy was effective and safe during pregnancy, with no adverse effects on the infant.

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Pregnancy-Induced Exacerbation of Hereditary Angioedema in a Multiparous Caucasian Female

Cited by 4 publications

References 9 publications

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

Hereditary Angioedema with Normal C1 Esterase Inhibitor Refractory to Long-Term Prophylaxis: A Case Report

Successful treatment with Cinryze® replacement therapy of a pregnant patient with hereditary angioedema: a case report

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