Hereditary angioedema: a bradykinin-mediated swelling disorder

Jenny Björkqvist; Anna Sala-Cunill; Thomas Renné

doi:10.1160/TH12-08-0549

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 2013; 109(03): 368-374
DOI: 10.1160/TH12-08-0549

Theme Issue Article

Schattauer GmbH

Hereditary angioedema: a bradykinin-mediated swelling disorder

Autor*innen

Jenny Björkqvist

¹Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

²Center of Molecular Medicine, Karolinska Institutet, Stockholm, Sweden
Anna Sala-Cunill

¹Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

³Allergy Section, Internal Medicine Department, Hospital Universitari Vall d´Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain

⁴Allergy Research Unit, Allergy Department, Institut de Recerca Vall d’Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain
Thomas Renné

¹Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

²Center of Molecular Medicine, Karolinska Institutet, Stockholm, Sweden

Abstract

Summary

Edema is tissue swelling and is a common symptom in a variety of diseases. Edema form due to accumulation of fluids, either through reduced drainage or increased vascular permeability. There are multiple vascular signalling pathways that regulate vessel permeability. An important mediator that increases vascular leak is the peptide hormone bradykinin, which is the principal agent in the swelling disorder hereditary angioedema. The disease is autosomal dominant inherited and presents clinically with recurrent episodes of acute swelling that can be life-threatening involving the skin, the oropharyngeal, laryngeal, and gastrointestinal mucosa. Three different types of hereditary angiodema exist in patients. The review summarises current knowledge on the pathophysiology of hereditary angiodema and focuses on recent experimental and pharmacological findings that have led to a better understanding and new treatments for the disease.

Keywords

Contact phase - inflammatory mediators - proteases - SERPINs

Volltext

Referenzen

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