HAE OVERVIEW

FIRAZYR is indicated for the treatment of acute attacks of hereditary angioedema (HAE) in adults 18 years of age and older.
Share / Send / Save

HAE is a rare, debilitating and potentially life-threatening genetic disease characterized by intermittent attacks that may be painful and edema without urticaria and pruritus. Acute attacks of HAE can involve almost any anatomical site.1,2

Epidemiology

The overall prevalence of HAE is thought to be between 1 in 10,000 and 1 in 50,000 individuals, occurring in all races and affecting both genders.2-4

Most patients start to experience attacks during childhood or adolescence and have subsequent attacks throughout their lives.5

The frequency and severity of acute attacks can vary considerably among individuals and even within the same family.6 A 2007 survey found that patients experienced a median of 12 attacks and a mean of 27 attacks per year.1

Genetic basis of HAE

HAE is an autosomal dominant disorder. The pathogenesis of this condition, in most patients, is the lack of or abnormal function of C1 esterase inhibitor.7

While approximately 75% of clinical cases of HAE involve a family history, as many as 25% of new diagnoses result from de novo mutations in the C1-INH gene.7

Most people with HAE have either type I or type II HAE.

  • Type I is the most common form of HAE, comprising up to 85% of the HAE patient population and characterized by a lack of functional C1-INH.7
  • Type II, which affects the remaining 15% of patients, results in relatively normal, or even elevated, levels of circulating C1-INH, but the protein secreted is dysfunctional.7