Affiliation:
1. Allergy and Immunology, Department of Medicine, Penn State College of Medicine, Hershey, PA 17033, USA
2. Penn State Milton S. Hershey Medical Center, Hershey, PA 17033, USA
Abstract
Hereditary angioedema (HAE) is a rare, inherited disease caused by a deficiency (HAE-1) or lack of functional (HAE-2) C1 inhibitor protein. The symptoms present with mucocutaneous swelling of various organ systems, such as the respiratory and gastrointestinal systems, which can manifest as stridor and abdominal pain, respectively. HAE can present with increased frequency and severity of attacks during the pregnancy and lactation period. This is thought to be due to hormonal changes, which may trigger HAE attacks. The management of this condition in pregnant and lactating patients can be challenging for providers due to disease rarity and the lack of data regarding the management of this specific population. This review aims to provide insights for HAE management regarding rescue therapy, short-term prophylaxis, and long-term prophylaxis via the consolidation of the current literature and various international consensus guidelines. Furthermore, this review discusses when to initiate treatment and at what frequency and dosing, as well as the possible side effects that may occur as a result of therapy.