Managing the female patient with hereditary angioedema

Abstract

Hereditary angioedema (HAE) is a rare disorder resulting from decreased functional levels of C1-inhibitor (C1-INH), which manifests as periodic episodes of localized edema which can be extremely painful, debilitating and even fatal if the swelling affects the larynx. HAE can complicate many aspects of obstetric/gynecologic care, and an awareness of the disease is critical for clinicians involved in the care of women because of potential HAE-related complications pertaining to pregnancy, labor and delivery, and other women's health issues. This article provides a review of published literature specific to HAE and its management in female patients, including important concerns regarding obstetric/gynecologic care. A growing body of relevant experience is presented to help guide the care of women with HAE.

Keywords: C1-inhibitor; ecallantide; hereditary angioedema; icatibant; women.

Figure 1.

Contact system pathway involved in hereditary angioedema pathophysiology and primary points of action for C1-INH, ecallantide and icatibant. Activated Factor XII converts prekallikrein to kallikrein, which in turn cleaves off (the small peptide of nine amino acids) bradykinin from HMW kininogen. Bradykinin, acting through the B2-receptor, causes endothelial cell dysfunction, fluid extravasation and angioedema. Lack of normal levels of functional C1-INH is the underlying defect in the predominant forms of hereditary angioedema (type 1 and type 2 HAE-C1-INH).

Figure 2.

Hereditary angioedema patient (A) experiencing a facial attack and (B) under normal circumstances.