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Tom 23. nr 4
Grudzień 2018

Hereditary angioedema: treatment options and availability. Balance between patients’ needs and stakeholders’ plans (streszczenie)
Anna Valerieva, Francesca Perego, Grzegorz Porebski, Maria Staevska, Marco Cicardi
Hereditary angioedema is a rare and disabling disease characterized by severe, acute, self- limiting edema of the subcutaneous and mucosal tissue. The disease carries significant mortality when the upper airways are involved. It is determined by a transient dysregulation in vascular permeability with a sudden increase in fluid extravasation. The most common genetic defects recognized on the SERPING1 gene leads to complement C1-inhibitor deficiency (C1-INH-HAE). In the last few years mutation on the factor XII gene, the angiopoietin, and the plasminogen genes were recognized to cause angioedema with normal C1-INH levels (nC1-INH-HAE). However, it is not uncommon to have family history of angioedema and unknown genetic defect. The burden of the disease on the individual patients is unpredictable since the number, location and severity of the attacks spans from silent disease to an extremely high number of attacks during lifetime. Nowadays effective and safe treatment for the C1-INH-HAE forms are available and new drugs are under investigation both for the on demand therapy and for the prophylaxis of the attacks. Acute treatments has been proved to reduce severity and duration of attack, prophylaxis reduces the number of attacks thus positively affecting the quality of life and reducing the indirect costs of the disease. Unfortunately, the costs of the medications are high limiting the accessibility and availability of treatment in different countries. This paper reviews the treatment options and the differences in the availability of the medications in three European countries, with practical suggestions for the management of the disease. Keywords: hereditary angioedema, treatment, prophylaxis, C1 inhibitor, bradykinin receptor antagonist, kallikrein inhibitors
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