Vnitr Lek 2016, 62(9):736-739

Current treatment options for hereditary angioedema

Roman Hakl
Ústav klinické imunologie a alergologie LF MU a FN u sv. Anny v Brně

Hereditary angioedema (HAE) caused by C1-inhibitor deficiency is a rare autosomal dominant disease. Clinically, HAE is manifested by repeated episodes of localized subcutaneous or submucosal edema. The disease is disabling and can be lethal. There is an extreme variability in frequency and severity of symptoms. This article concerns on the organization of care and current treatment options of the disease. Treatment strategy includes short-term and long-term prophylaxis and treatment of attacks. Currently, treatment options include attenuated androgens, antifibrinolytics, recombinant (rhC1-INH) and plasma-derived (pdC1-INH) C1-INH, bradykinin receptor antagonist and inhibitor of kallikrein. Since 2011, the care of HAE patients in the Czech Republic is concentrated into 4 diagnostic and therapeutic centers.

Keywords: bradykinin receptor; C1 inhibitor; complement system; hereditary angioedema

Received: May 10, 2016; Accepted: July 14, 2016; Published: September 1, 2016  Show citation

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Hakl R. Current treatment options for hereditary angioedema. Vnitr Lek. 2016;62(9):736-739.
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