Author: V. Dimov, M.D., Allergist/Immunologist and Assistant Professor at University of Chicago
Reviewer: S. Randhawa, M.D., Allergist/Immunologist and Assistant Professor at NSU
A
Acquired
Angioedema
ACEi-related
Hereditary
Angioedema
Autosomal dominant
Androgens for prophylaxis
HAE mnemonic
1
C1 inhibitor is low
C1 q level is normal
11 chromosome
11 years is the mean age of onset
11 years before the diagnosis is made, on average
Treatment of HAE
R
Recombinant human C1 inhibitor from mammary secretions of trangenic rabbit
Rabbit
Rhucin
Angioedema (AE) can be allergic or non-allergic.
There are 5 types of non-allergic angioedema (AE):
- acquired AE
- hereditary AE (HAE)
- ACE-inhibitor induced AE
- idiopathic AE, can occur with chronic urticaria
- pseudoallergic AE, e.g. reaction to NSAIDs
There are 3 types of HAE that are differentiated by C4 and C1-INH levels
- type I HAE - low C4, low C1-INH function, low C1-INH antigen level
- type II HAE - low C4, low C1-INH function, normal C1-INH antigen level
- type III HAE - all normal
Treatment of acute HAE attacks
- C1-INH, 20 units/kg, IV infusion
- Icatibant, 30 mg SC, bradykinin B2 receptor antagonist
- Ecallantide, 30 mg SC, kallikrein receptor antagonist
Prophylaxis of HAE attacks
- C1-INH, 1,000 units, IV infusion every 3-4 days
- attenuated androgen, e.g. danocrine 200 mg PO TID
Angioedema (AE) Classification (click to enlarge the image):
References
New Directions in the Treatment of Angioedema. Medscape, 2012.
International consensus on hereditary and acquired angioedema. Annals, 2012.
Published: 02/07/2008
Updated: 12/12/2012
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