RESUMO
IMPORTANCE: Hereditary angioedema is a rare disease of potentially life-threatening attacks of angioedema that can affect patients of all ages, including women of childbearing age. Pregnancy can affect the course of the disease and the choice of treatment used. It is important for the care providers to recognize this disease and understand its mechanism in order to provide appropriate care for the patients. OBJECTIVE: The goal of this article is to provide an overview of hereditary angioedema and guideline for management of pregnant patients with hereditary angioedema. EVIDENCE ACQUISITION: A search of the available English language literature was performed on PubMed and Ovid MEDLINE using the key words hereditary angioedema and pregnancy. Additional articles were selected from the reference lists of the reviewed articles. RESULTS: The data for hereditary angioedema in pregnant patients come from observational studies, case reports, retrospective reviews, and questionnaires. The course of hereditary angioedema can be variable between different patients and pregnancies. Plasma-derived C1 inhibitor concentrate is both safe and effective as treatment for attacks and as preventive therapy in pregnancy. With proper recognition, understanding of the disease, and appropriate medical management, most patients will undergo successful pregnancy and delivery. CONCLUSIONS AND RELEVANCE: Clinicians should maintain high suspicion for this disease when patients present with recurrent episodes of angioedema without urticaria or severe abdominal pains of unclear etiology. Treatment plans during pregnancy and delivery should be individualized, and the patient's care should be shared by a clinician experienced in the management of this disease.