International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency

Teresa Caballero, Henriette Farkas, Laurence Bouillet, Tom Bowen, Anne Gompel, Christina Fagerberg, Janne Bjökander, Konrad Bork, Anette Bygum, Marco Cicardi, Caterina De Carolis, Michael Frank, Jimmy H.C. Gooi, Hilary Longhurst, Inmaculada Martínez-Saguer, Erik Waage Nielsen, Krystina Obtulowitz, Roberto Perricone, Nieves Prior

Research output: Contribution to journalArticle

134 Citations (Scopus)

Abstract

Background: There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH). Objective: We sought to elaborate guidelines for optimizing the management of gynecologic/obstetric events in female patients with HAE-C1-INH. Methods: A roundtable discussion took place at the 6th C1 Inhibitor Deficiency Workshop (May 2009, Budapest, Hungary). A review of related literature in English was performed. Results: Contraception: Estrogens should be avoided. Barrier methods, intrauterine devices, and progestins can be used. Pregnancy: Attenuated androgens are contraindicated and should be discontinued before attempting conception. Plasma-derived human C1 inhibitor concentrate (pdhC1INH) is preferred for acute treatment, short-term prophylaxis, or long-term prophylaxis. Tranexamic acid or virally inactivated fresh frozen plasma can be used for long-term prophylaxis if human plasma-derived C1-INH is not available. No safety data are available on icatibant, ecallantide, or recombinant human C1-INH (rhC1INH). Parturition: Complications during vaginal delivery are rare. Prophylaxis before labor and delivery might not be clinically indicated, but pdhC1INH therapeutic doses (20 U/kg) should be available. Nevertheless, each case should be treated based on HAE-C1-INH symptoms during pregnancy and previous labors. pdhC1INH prophylaxis is advised before forceps or vacuum extraction or cesarean section. Regional anesthesia is preferred to endotracheal intubation. Breast cancer: Attenuated androgens should be avoided. Antiestrogens can worsen angioedema symptoms. In these cases anastrozole might be an alternative. Other issues addressed include special features of HAE-C1-INH treatment in female patients, genetic counseling, infertility, abortion, lactation, menopause treatment, and endometrial cancer. Conclusions: A consensus for the management of female patients with HAE-C1-INH is presented.

Original languageEnglish
Pages (from-to)308-320
Number of pages13
JournalJournal of Allergy and Clinical Immunology
Volume129
Issue number2
DOIs
Publication statusPublished - Feb 2012

Keywords

  • Angioedema
  • C1 inhibitor deficiency
  • breast cancer
  • contraception
  • delivery
  • fertility
  • genetic counseling
  • hereditary angioedema
  • pregnancy
  • treatment

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Fingerprint Dive into the research topics of 'International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency'. Together they form a unique fingerprint.

  • Cite this

    Caballero, T., Farkas, H., Bouillet, L., Bowen, T., Gompel, A., Fagerberg, C., Bjökander, J., Bork, K., Bygum, A., Cicardi, M., De Carolis, C., Frank, M., Gooi, J. H. C., Longhurst, H., Martínez-Saguer, I., Nielsen, E. W., Obtulowitz, K., Perricone, R., & Prior, N. (2012). International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. Journal of Allergy and Clinical Immunology, 129(2), 308-320. https://doi.org/10.1016/j.jaci.2011.11.025