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The Hereditary Angioedema Triggered by Estrogen: Systematic Review

Author(s): Jullyana Egito Peixoto da Costa, João Pedro de Oliveira Lopes , Bruno Debona Souto, Denis Henrique de Oliveira, Severino Correia do Prado Neto, Neire Moura de Gouveia

Background: Hereditary angioedema (HAE) triggered by estrogen related to women and their life milestones. Objective: This study aimed to establish a relationship between hereditary angioedema and estrogen in women at puberty, pregnancy or on contraceptives use, their mean age at onset of manifestations, age at diagnosis, and signs and symptoms associated with the precipitating factors for HAE. Data source: Systematic review of articles in English and Portuguese using the Virtual Health Library, Google Scholar, PubMed, and Springer from 2009 to 2019. The descriptors were “hereditary angioedema,” “female,” and “contraceptive”, combined by the boolean operator “AND”. Methods: Case reports and clinical studies on HAE in women were selected. The articles were selected by reading the title and abstract, followed by followed by integral reading, where theses, dissertations, book chapters, grey literature, and review articles were excluded. Information on age, number of people, symptomatology, life stages, and precipitating factors for HAE crises were collated in a table. Results: The mean age of the women was 28.63 years; no postmenopausal case related to hormone replacement therapy was observed. Pregnancy was majorly affected, followed by the phase with use of oral contraceptives, puberty, and eventually menstrual periods. The most evident signs and symptoms were facial edema, abdominal pain, and subcutaneous manifestations, with cases of airway involvement, specifically during pregnancy. Conclusion: The HAE symptoms initially appear, mostly during puberty, and worsen during the menstrual periods. The worst edemigenic attacks were noticed during pregnancy, confirming that estrogen is an important precipitating and aggravating factor triggering HAE.

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