Hereditary Angioedema Precipitated by Estrogen Replacement Therapy in a Menopausal Woman

Abstract

We report the first documented case in the literature of hereditary angioedema presenting after commencement of estrogen replacement therapy for menopausal symptoms. The late presentation of the disease and the precipitation of attacks by physiological doses of estrogen replacement therapy make this a highly unusual case. The pathophysiology of hereditary angioedema and its hormonal links are discussed.

Hereditary Angioedema in Women

“…9 One hypothesis explaining this outcome is that, although estrogen levels decrease at the menopause, ovarian androgens also decrease, and some women could be more sensitive to their protective effect.…”

The bradykinin-degrading aminopeptidase P is increased in women taking the oral contraceptive pill

“…In addition, it was noted that androgen prophylaxis, which is used as a treatment for hereditary angioedema, increased plasma AP-P activity. 19 Hereditary angioedema is influenced by changes in female hormones 20,21 and progesterone-only pills have been used successfully by women who have had previous angioedema attacks when using the combined OCP. 22,23 These studies support our findings that progesterone increases AP-P expression and activity.…”

Contraception in hereditary angioedema

“…There is enough evidence that contraindicates the use of OCs or HT in patients with HAE because its use exacerbates and increases the crisis, and the first therapeutic prevention is the suspension of oral estrogen (1)(2)(3)(4)(5)(6). In addition, there is evidence demonstrating that the use of oral estrogen reduces the concentration of C1INH (5).…”

“…Type I corresponds to the congenital deficiency of C1INH, thus there are lower levels of this protein in the plasma than in normal conditions. In type II HAE, production and concentration of C1INH is normal or high, but a functional deficiency exists (1,2). Type III is a form of HAE that has only been described in women.…”