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Related Experiment Video

Updated: Jun 19, 2026

Scaled-Up Preparation of an Intermediate of Upatinib, ACT051-3

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Update on therapeutic developments for hereditary angioedema.

Sandra C Christiansen¹, Bruce L Zuraw

¹Department of Allergy, Kaiser Permanente and Department of Medicine, University of California San Diego, La Jolla, California, USA. sandra.c.christiansen@kp.org

Allergy and Asthma Proceedings

|October 22, 2009

Summary

New hereditary angioedema (HAE) treatments target kinin generation, offering hope beyond traditional therapies. Emerging options include C1 inhibitor replacement and plasma kallikrein inhibitors for improved HAE management.

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Published on: September 28, 2015

Area of Science:

Immunology
Pharmacology
Genetics

Background:

Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent, potentially life-threatening swelling attacks.
Current prophylactic treatments for HAE, such as androgens and antifibrinolytics, have limitations due to side effects.
Acute HAE management has historically relied on supportive care, with limited specific interventions.

Purpose of the Study:

To review the molecular mechanisms of HAE attacks, focusing on kinin generation.
To discuss novel and emerging therapeutic strategies for both prophylaxis and acute treatment of HAE.
To highlight the transformative potential of new HAE therapies.

Main Methods:

Review of scientific literature on HAE pathogenesis and treatment.
Discussion of newly approved and investigational HAE therapies.
Analysis of the role of C1 inhibitor (C1INH) and kinin-kallikrein system in HAE.

Main Results:

Understanding HAE attacks involves recognizing the central role of kinin generation.
Approved therapies include C1 inhibitor (C1INH) replacement for HAE prophylaxis.
Investigational treatments include purified/recombinant C1INH, plasma kallikrein inhibitors, and B2-receptor antagonists for acute HAE management.

Conclusions:

Emerging therapies based on targeting the kinin pathway represent a significant advancement in HAE treatment.
Novel treatments offer improved efficacy and potentially better safety profiles compared to traditional HAE therapies.
The future of HAE management is poised for transformation with these new therapeutic options.