Diagnosis

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

Diagnosing HAE

Hereditary angioedema (HAE) is so rare that many healthcare professionals have never seen a patient with HAE, which can lead to delayed diagnosis or misdiagnosis for some patients.1-3 While about 40% of patients are now diagnosed within the first 3 years of experiencing symptoms, nearly one-third of patients still experience delays of 10 years or more.1,4,5,a

aIn a survey of 149 patients.4

Clinical presentation of HAE

HAE should be considered in patients with1,6:

  • History of recurrent angioedema without urticaria
  • Positive family history
  • Onset of symptoms in childhood or adolescence
  • Recurrent abdominal pain attacks
  • Occurrence of upper airway edema
  • Failure to respond to treatment with antihistamines, glucocorticoids, or epinephrine
  • Presence of prodromes

Confirming diagnosis

To confirm an HAE diagnosis, laboratory testing is necessary. Patients should have serum levels of C4 measured, as well as antigenic and functional C1 esterase inhibitor levels.2,6

Tests to Confirm HAE Types I and II7,8


Chart of Tests to Confirm HAE Types I and II

Importance of diagnosis

HAE is frequently misdiagnosed as another condition,2 and up to 65% of patients with HAE may have been misdiagnosed with another condition prior to receiving their HAE diagnosis.9,b

bIn a survey of 313 patients.9

Missed/delayed diagnosis can result in unnecessary treatments and surgery.2,9 Misdiagnosis or delays in diagnosis also mean patients are at risk of not receiving appropriate treatment for potentially life-threatening laryngeal attacks. This risk makes early, accurate diagnosis critical.2,7