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Hereditary angioedema: current and emerging treatment options
Jerrold H Levy1, Douglas J Freiberger, John Roback
1FAHA, Department of Anesthesiology, Emory University Hospital, Atlanta, GA 30322, USA. jlevy01@emory.edu
Anesthesia and Analgesia
|April 27, 2010
Summary
Hereditary angioedema (HAE) management is evolving with new treatments targeting the kinin pathway. This review covers current and investigational therapies, including perioperative care for HAE patients.
Area of Science:
- Immunology
- Pharmacology
- Genetics
Background:
- Hereditary angioedema (HAE) involves kinin pathway activation, leading to severe, potentially life-threatening swelling.
- Existing treatments like steroids and antifibrinolytics have limitations and side effects.
- C1 esterase inhibitor (C1-INH) concentrates and kinin pathway modulators offer new therapeutic avenues.
Purpose of the Study:
- To review the pathophysiology of HAE.
- To discuss current and emerging management strategies for HAE.
- To highlight perioperative considerations for HAE patients.
Main Methods:
- Literature review of HAE pathophysiology and treatment options.
- Analysis of licensed and investigational drugs for HAE.
- Focus on clinical management, including acute attack and perioperative care.
Main Results:
- Newer treatments, including C1-INH concentrates and ecallantide, are now available in the US for HAE prophylaxis and acute attack management.
- These therapies target the kinin pathway, offering improved efficacy and safety profiles.
- Investigational drugs are also under development.
Conclusions:
- HAE management has advanced significantly with targeted therapies.
- Effective prophylaxis and acute treatment options are crucial for improving patient outcomes.
- Perioperative management requires careful planning due to HAE risks.
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