HAE update: epidemiology and burden of disease

Abstract

Angioedema (AE) is characterized by swelling of the mucosa or submucosa and/or the subcutaneous tissue of the skin. Hereditary AE (HAE) is an autosomal dominant genetic disorder caused by dysfunctional C1 esterase inhibitor, which affects an estimated 1:10,000 to 1:50,000 patients. A survey study that was conducted between 2007 and 2008 to assess the burden of illness of HAE patients revealed they had significant decreases in physical and mental health, impaired work productivity, and a significantly greater amount of depression compared with patients afflicted with other chronic diseases. The cost of treating HAE is quite significant ranging from $14,000 a year for mild disease to $96,000 for severe disease. Given the changing paradigm for evaluation and treatment with the advent of increased recognition and availability of novel prophylactic and on-demand therapeutic agents, updated survey studies of this nature to assess burden of illness, quality of life, and associated treatment costs should be conducted to provide a better understanding of the optimal management of HAE patients in the United States.