Benefits of progestin contraception in non‐allergic angioedema

Saule, Claire; Boccon‐Gibod, Isabelle; Fain, Olivier; Kanny, G.; Plu‐Bureau, Geneviève; Martin, Ludovic; Launay, David; Bouillet, Laurence; Gompel, Anne

doi:10.1111/cea.12055

Cited by 78 publications

(62 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…We plan to analyse the PAI-2 levels in our FXII-HAE cohort. Furthermore, in a previous retrospective study we showed that progestin was an interesting background therapy for patients with all types of HAE [20] (the introduction of progestin in 10 FXII-HAE patients resulted in the disappearance of AE attacks). During an acute attack in our FXII-HAE patients, treatment with C1-INH concentrate appears to be as effective as icatibant, despite normal levels of C1-INH.…”

Section: Discussionmentioning

confidence: 94%

Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema

Deroux

Boccon‐Gibod

Fain

et al. 2016

Clinical and Experimental Immunology

View full text Add to dashboard Cite

SummaryHereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitative deficiency in C1-inhibitor (C1-INH) or normal C1-INH. HAE with normal C1-INH is associated in 20% of cases with mutations in the gene for factor XII (FXII) or FXII-HAE. A recent review described 41 families, including 14 German and 15 Spanish families. We have constructed a register of French patients and their characteristics. A national survey was launched through the French National Center of Reference for Angioedema (CREAK) to study the clinical, biological and therapeutic characteristics of patients with HAE linked to a mutation of FXII gene. Fifty-seven patients were identified from 24 different families. In most cases they were young women (mean age at diagnosis: 31 years, mean age at first symptom: 21 years, female/male ratio: 76%). Twenty-one per cent of the patients experienced angioedema attacks only during pregnancy or when on oestrogen contraception. Sixty-three per cent had attacks at all times, but they were more severe during these same periods. Male carriers of the mutation were more frequently asymptomatic than females (P 5 0Á003). C1-INH concentrate and icatibant were both effective for treating attacks. The prophylactic use of tranexamic acid led to a 64% decrease in the number of attacks. This is one of the largest series reported of HAE patients with FXII mutation. The therapeutic management appeared to be identical to that of HAE with C1-INH deficiency.

show abstract

Section: Discussionmentioning

confidence: 94%

Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema

Deroux

Boccon‐Gibod

Fain

et al. 2016

Clinical and Experimental Immunology

View full text Add to dashboard Cite

show abstract

“…Urticaria was present in 16 patients (37% of this group), with 9 RU and 7 CU cases. All patients were advised not to resume oestrogen-based contraceptives but rather to use progestin-only contraceptives as recently suggested [33]. Three patients exhibited AO recurrence after the incriminated treatment was stopped, 1 during pregnancy and the 2 others during ovarian stimulation for infertility.…”

Section: Resultsmentioning

confidence: 99%

Distinct Conditions Support a Novel Classification for Bradykinin-Mediated Angio-Oedema

Dessart

Defendi²,

Humeau³

et al. 2015

Dermatology

View full text Add to dashboard Cite

Background: Angio-oedema (AO) can be attributable to bradykinin (BK) accumulation, as is the case for prototypical hereditary AO (HAO) due to C1 inhibitor (C1-INH) deficiency. However, our clinical experience in a reference centre has shown that some patients display a clinical history suggestive of HAO, but exhibit normal C1-INH function, have no mutation in the causative genes associated with HAO (SERPING1, F12), and report no intake of drugs known to promote AO. Objective: We sought to determine the frequency and distribution of different AO subtypes suspected to be BK-mediated AO (BK-AO) and defined by clinical, history and biological criteria (enzyme activities implicated in BK formation and catabolism). Methods: The files of all patients referred to our centre for suspected BK-AO were retrospectively analysed. Results: The distribution of patients (n = 162) was 16 and 4% with a hereditary deficiency of C1-INH or a gain of factor XII function, respectively, 29% with iatrogenic BK-AO, 21% with non-iatrogenic defective kininase activity and 30% with idiopathic increased kinin formation. Conclusion: BK-AO may be caused by multiple inherited or acquired factors triggering BK accumulation. Therefore, we propose a novel typology for BK-AO based on the imbalance of production/catabolism of BK.

show abstract

“…New therapies currently under clinical investigation include subcutaneous C1INH (CSL Behring, King of Prussia, PA, USA), hyaluronidase-facilitated subcutaneous C1INH (Viropharma, Exton, PA, USA), an oral kallikrein inhibitor Bcx 4161 (Biocryst, Durham, NC, USA), cyclomimetic cyclic peptides inhibiting plasma kallikrein (Ra Pharma, Cambridge, MA, USA), monoclonal antikallikrein antibodies Dx 2930 (Dyax, Burlington, MA, USA) and even, in the future, gene therapy for C1INH deficiency. As well as these new developments, there also appears to be a protective role for women taking progestogen-only birth control pills, particularly those with anti-gonadotrophic activity such as norethisterone [25]. In summary, it is hoped that this and future audits will serve to help inform the decision-making process in planning future care for patients with bradykinin-mediated angioedema.…”

Section: Discussionmentioning

confidence: 97%

A UK national audit of hereditary and acquired angioedema

Jolles

Williams

Carne

et al. 2013

Clinical and Experimental Immunology

View full text Add to dashboard Cite

SummaryHereditary angioedema (HAE) and acquired angioedema (AAE) are rare lifethreatening conditions caused by deficiency of C1 inhibitor (C1INH). Both are characterized by recurrent unpredictable episodes of mucosal swelling involving three main areas: the skin, gastrointestinal tract and larynx. Swelling in the gastrointestinal tract results in abdominal pain and vomiting, while swelling in the larynx may be fatal. There are limited UK data on these patients to help improve practice and understand more clearly the burden of disease. An audit tool was designed, informed by the published UK consensus document and clinical practice, and sent to clinicians involved in the care of HAE patients through a number of national organizations. Data sets on 376 patients were received from 14 centres in England, Scotland and Wales. There were 55 deaths from HAE in 33 families, emphasizing the potentially lethal nature of this disease. These data also show that there is a significant diagnostic delay of on average 10 years for type I HAE, 18 years for type II HAE and 5 years for AAE. For HAE the average annual frequency of swellings per patient affecting the periphery was eight, abdomen 5 and airway 0·5, with wide individual variation. The impact on quality of life was rated as moderate or severe by 37% of adult patients. The audit has helped to define the burden of disease in the UK and has aided planning new treatments for UK patients.

show abstract

Benefits of progestin contraception in non‐allergic angioedema

Cited by 78 publications

References 39 publications

Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema

Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema

Distinct Conditions Support a Novel Classification for Bradykinin-Mediated Angio-Oedema

A UK national audit of hereditary and acquired angioedema

Contact Info

Product

Resources

About