New Guidelines Detail Hereditary Angioedema Management in Women
Angioedema is a BIG problem in emergency rooms across the country and Tulsa is no exception! Most treatment protocols use antihistamines (Benadryl) along with steroids. aughhh!
An international panel of gynecologists and geneticists has issued practical guidelines for managing women with hereditary angioedema caused by C1 inhibitor deficiency, or HAE-C1-INH.The condition affects between one in 10,000 and one in 50,000 individuals, and estrogen is known to worsen the course of the disease.But little information has been published on how to manage gynecologic/obstetric events in women with HAE-C1-INH, the panel writes in its report, published in the Journal of Allergy and Clinical Immunology.
Effective acute treatments include plasma-derived human C1 inhibitor concentrate (pdhC1INH), icatibant acetate, ecallantide, and recombinant human C1 inhibitor (rhC1INH), the doses of which are the same during pregnancy. PdhC1INH is recommended as first-line therapy in pregnancy.
Short-term prophylaxis (before invasive medical, surgical, or dental procedures or other events that could precipitate edematous attacks) can be provided by pdhC1INH, attenuated androgens, tranexamic acid, and fresh frozen plasma, but pdhC1INH is the treatment of choice during pregnancy.
The same drugs can be used for long-term prophylaxis, but attenuated androgens are contraindicated during pregnancy and tranexamic acid during pregnancy is medically indicated only when pdgC1INH is unavailable, Dr. Teresa Caballero from Hospital La Paz Health Research Institute in Madrid and colleagues note.
Because of the contribution of estrogen to worsening of HAE symptoms, its use for contraception and menopause should be avoided.
For similar reasons, antiestrogens like tamoxifen that may have some agonistic effects on estrogen receptors should be avoided in breast cancer patients. If possible, attenuated androgens should also be avoided as prophylaxis in these women.
The guidelines also address special features of HAE-C1-INH treatment in female patients, such as genetic counseling, infertility, abortion, lactation, and endometrial cancer.
J Allergy Clin Immunol 2011.