US EPA

7414185 

Journal Article 

Hereditary Angioedema 

Maru, S; Craig, T; , 

2019 

Springer International Publishing 

Cham 

Allergy and Asthma 

227-244 

10.1007/978-3-030-05147-1_10 

http://link.springer.com/10.1007/978-3-030-05147-1_10
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Hereditary angioedema (HAE) is a rare disease affecting approximately 1 in 50,000 people and presents with recurrent cutaneous and mucosal membrane swelling. The result is recurrent angioedema, intermittent abdominal obstruction and pain, and airway swelling. Though death is rare in patients diagnosed and on therapy, upper airway swelling can be fatal. Disability and absenteeism secondary to the frequent attacks lasting up to 3 days can limit quality of life and education and occupational stability. Multiple therapies have been approved in the last decade and have made drastic improvements in this orphan disease. This chapter will discuss epidemiology, signs and symptoms, differential diagnosis, diagnosis, treatment, and management of the HAE patient.