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Caballero, Teresa, MD, PhD; Farkas, Henriette, MD, PhD, DSc; Bouillet, Laurence, MD, PhD; Bowen, Tom, MD; Gompel, Anne, MD, PhD; Fagerberg, Christina, MD; Bjökander, Janne, MD; Bork, Konrad, MD; Bygum, Anette, MD; Cicardi, Marco, MD; de Carolis, Caterina, MD; Frank, Michael, MD; Gooi, Jimmy H.C., MD; Longhurst, Hilary, MD; Martínez-Saguer, Inmaculada, MD; Nielsen, Erik Waage, MD; Obtulowitz, Krystina, MD; Perricone, Roberto, MD; Prior, Nieves, MD
Journal of allergy and clinical immunology, 02/2012, Volume: 129, Issue: 2Journal Article, Conference Proceeding
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.
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