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