Abstract

Women with hereditary angioedema (HAE) are more likely to be symptomatic that men. Hormonal factors (puberty, contraception, pregnancy,....) play a significant role in the precipitation or worsening of the condition in women. So, combined contraceptive pills are not indicated and progestogen pill must be preferred. During pregnancy, attack rate can increase (38-48% of women). C1Inhibitor concentrate and tranexamic acid can be used during pregnancy. Attenuated androgens for long term prophylaxis are effective but side effects appear more often in female patients. These side effects are dose dependant and can be attenuated by titrating the dose down the lowest effective level.

Highlights

  • Women with hereditary angioedema (HAE) are more likely to be symptomatic that men

  • In HAE associated with C1 Inh deficiency, Professor Bork has shown that women have more clinical episodes than men (p < 0.02) [2]

  • Reports have noted a close relationship between female hormones and angioedema: a mother and her daughter whose HAE-related symptoms appeared to be sex hormone dependent [3]

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Summary

17. Wessler S

Nogawa N, Sumino H, Ichikawa S, Kumakura H, Takayama Y, Nakamura T, Kanda T, Mizunuma H, Kurabayashi M: Effect of long term hormone replacement therapy on angiotensin converting enzyme activity and bradykinin in post menopausal women with essential hypertension and normotensive postmenopausal women. Bork K, Barnstedt SE, Koch P, Traupe H: Hereditary angioedema with normal C1-inhibitor activity in women. Dewald G, Bork K: Missense mutations in the coagulation factor XII (Hageman factor) gene in hereditary angioedema with normal C1 inhibitor. Cichon S, Martin L, Hennies HC, Müller F, Van Driessche K, Karpushova A, Stevens W, Colombo R, Renné T, Drouet C, Bork K, Nöthen MM: Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Eur J Obstet Gynecol Reprod Biol 2010.

42. Hermans C
Findings
49. Dmowski WP

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