Hereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent episodes of edema involving the skin, gastrointestinal tract, and/or airway. With a prevalence of approximately 1 in 50,000, HAE is rare, affecting fewer than 10,000 people in the United States.1 There is a range of severity and frequency of symptoms in patients with HAE. Some patients experience relatively mild symptoms on rare occasions, others have symptoms 1 or more times each month, and still others have recurrent debilitating attacks that may lead to tragic outcomes, including narcotic addiction and/or inappropriate surgery due to recurrent acute abdominal pain from intestinal edema. Laryngeal edema leading to death or near-death from asphyxiation may also occur, sometimes in patients who had not previously had severe symptoms.2 As a group, HAE patients are more likely to require frequent emergency department visits and hospitalizations, consistently miss work or school, and endure the negative psychosocial effect of HAE as a chronic disease.3 The morbidity and potential mortality of HAE, if untreated, are therefore profound, especially for those with moderate or severe symptoms.
展开▼
