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How we manage persons with hereditary angioedema
Bruce L Zuraw1,2, Sandra C Christiansen1
1University of California, San Diego, La Jolla, CA, USA.
British Journal of Haematology
|April 14, 2016
Summary
Hereditary angioedema (HAE), a rare genetic disorder causing swelling attacks, is now better managed with new therapies. An integrated care approach is recommended to improve patients' quality of life.
Area of Science:
- Genetics and Immunology
- Pharmacology and Therapeutics
Background:
- Hereditary angioedema (HAE) is a rare autosomal dominant disorder.
- Characterized by recurrent swelling (subcutaneous and mucosal), HAE can lead to severe morbidity and mortality.
- Recent advancements in therapies since 2008 have significantly altered HAE management.
Purpose of the Study:
- To review the current understanding of HAE pathophysiology.
- To outline the diagnostic evaluation for recurrent angioedema without urticaria.
- To discuss the therapeutic strategies for managing HAE.
Main Methods:
- Literature review of HAE pathophysiology, diagnosis, and treatment.
- Analysis of novel therapeutic agents introduced post-2008.
- Synthesis of current clinical guidelines and expert recommendations.
Main Results:
- Understanding of HAE pathophysiology has advanced, clarifying the role of the kallikrein-kinin system.
- Diagnostic criteria for angioedema without urticaria are established.
- A range of novel therapies, including C1-inhibitor concentrates and monoclonal antibodies, are available for HAE treatment.
Conclusions:
- Novel therapies have transformed HAE management, offering improved control over attacks.
- An integrative approach to care, combining medical treatment and patient support, is crucial.
- The goal of HAE management is to normalize the lives of affected individuals through comprehensive care.
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