Table 2.

Required criteria for a diagnosis of HAE with normal C1-INH (HAE-FXII and HAE-U)

1. A history of recurrent angioedema in the absence of concomitant urticaria or use of a medication known to cause angioedema

2. Documented normal or near-normal C4, C1-INH protein, and C1-INH function

3. One of the following:

a. A demonstrated F12 mutation associated with the disease

b. A positive family history of angioedema and documented evidence of lack of efficacy of chronic high-dose antihistamine therapya

Notes:

^aCetirizine at 40 mg/d or the equivalent for at least 1 month and an interval expected to be associated with 3 or more attacks of angioedema. Data from Zuraw et al.25

Abbreviations: C1-INH, C1 esterase inhibitor; HAE-FXII, hereditary angioedema – caused by factor XII; HAE-U, hereditary angioedema - unknown cause.