Management of Pregnancy in Patients with Hereditary Angioedema

Abstract

Hereditary angioedema is a rare autosomal dominant genetic disease that constitutes a primary immunodeficiency due to deficiency of the complement system. It is characterized by recurrent edema of the skin, mucous membranes and submucosal tissues, which can affect any part of the body, respiratory tract and digestive system. The most feared complication is edema of the upper respiratory tract, which can cause death by asphyxia. Abdominal pain can simulate acute abdomen. Trauma, stress, drugs or infections can trigger seizures. The diagnosis is based on the recognition of the clinical characteristics and the laboratory alterations as the presence of decreased C4 in serum and the absence or great reduction of the level or inhibitor function of C1. This disease is more severe in women because it varies with hormones, menstruation and pregnancy; and it is important for medical professionals to know what to do when a patient with this disease wishes to use contraceptives or is pregnant. Pregnancy is a special moment when the obstetrician's knowledge of this disease, its prophylactic and therapeutic management are vital because this entity does not respond to the usual treatment of allergic edema, considering there are contraindicated drugs during pregnancy. Its treatment of choice is the infusion of complement C1 Inhibitor factor. Fresh frozen plasma is an option to consider in case of short-term prophylaxis or acute attack.