Bouillet Laurence, Longhurst Hilary, Boccon-Gibod Isabelle, Bork Konrad, Bucher Christophe, Bygum Anette, Caballero Teresa, Drouet Christian, Farkas Henriette, Massot Christian, Nielsen Erik W, Ponard Denise, Cicardi Marco
Department of Internal Medicine, Grenoble University Hospital, Grenoble, France.
Am J Obstet Gynecol. 2008 Nov;199(5):484.e1-4. doi: 10.1016/j.ajog.2008.04.034. Epub 2008 Jun 13.
Fluctuations in sex hormones can trigger angioedema attacks in women with hereditary angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of angioedema may be very variable in different women.
Within the PREHAEAT project launched by the European Union, data on 150 postpubertal women with hereditary angioedema were collected in 8 countries, using a patient-based questionnaire.
Puberty worsened the disease for 62%. Combined oral contraceptives worsened the disease for 79%, whereas progestogen-only pills improved it for 64%. During pregnancies, 38% of women had more attacks, but 30% had fewer attacks. Vaginal delivery was usually uncomplicated. Attacks occurred within 48 hours in only 6% of cases. Those more severely affected during menses had more symptoms during pregnancies, suggesting a hormone-sensitive phenotype for some patients.
The course of angioedema in women with C1 inhibitor deficiency is affected by physiologic hormonal changes; consequently, physicians should take these into account when advising on management.
性激素波动可引发遗传性血管性水肿女性的血管性水肿发作。复方口服避孕药疗法以及怀孕均可诱发严重发作。不同女性的血管性水肿病程可能差异很大。
在欧盟发起的PREHAEAT项目中，使用基于患者的问卷，在8个国家收集了150名青春期后遗传性血管性水肿女性的数据。
62%的患者青春期病情加重。79%的患者服用复方口服避孕药后病情加重，而仅含孕激素的避孕药使64%的患者病情改善。怀孕期间，38%的女性发作次数增多，但30%的女性发作次数减少。阴道分娩通常无并发症。仅6%的病例在48小时内发作。月经期间受影响更严重的患者在怀孕期间症状更多，提示部分患者存在激素敏感型表型。
C1抑制剂缺乏女性的血管性水肿病程受生理性激素变化影响；因此，医生在提供管理建议时应考虑这些因素。
