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The variants of hereditary angioedema (HAE) related to Complement-1 Esterase Inhibitor (C1-INH) function are:
- Type I: affecting 85% of those with HAE and characterized by low antigenic and functional plasma levels of a normal C1-INH protein
- Type II: affecting 15% of those with HAE and characterized by the presence of normal or elevated antigenic levels of a dysfunctional mutant protein together with reduced levels of the functional protein
- Type III: affecting only female patients and characterized by normal functional levels of C1-INH yet classic signs and symptoms of HAE
Acquired Type I or Type II HAE is very rare, and is due to immune complexes that are usually linked to an underlying lymphoproliferative disorder.
For more information about HAE see:
Disease Overview
Symptoms of HAE
Etiology & Prevalence
Pathophysiology
Glossary
Document Published:
12/21/2006 3:53 PM
Last Updated:
1/29/2007 10:35 AM
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