Pharmacological basis for the treatment of hereditary angioedema

• Autores: A Ramón Albert, Rodrigo Bonilla Peñarrubia, Carlos Devesa García, A. Soria Aledo, Pedro García Salom
• Localización: European journal of clinical pharmacy: atención farmacéutica, ISSN 2385-409X, Vol. 18, Nº. 2, 2016, págs. 113-125
• Idioma: inglés
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• Resumen

  • Hereditary angioedema (HAE) is a rare genetic disorder caused by a deficiency or dysfunction of the C1 esterase inhibitor (C1-INH) that is manifested by disabling recurrent attacks of angioedema that can be life-threatening. The C1-INH is the main control protein downregulating four interconnected pathways involving inflammation and vascular permeability and bradykinin production. There is a significant phenotypic both intra-individual and inter-individual variability. Its diagnostic criteria are serum levels of C4 and C1-INH and C1-INH activity.

    HAE management has two main goals: treating the acute episodes and limiting their manifestation in short and long term. Currently, C1-INH concentrates are used both for on-demand therapy, and for prophylactic therapy, whereas contact system modulators, ecallantide andicatibant, are used for the treatment of acute attacks only. Attenuated androgens are the choice for long-term prophylaxis, but sometimes (contraindications, side-effects, etc.), antifibrinolytics or C1-INH concentrates should be considered as other options. Short-term prophylaxis should be bared in mind before certain surgeries or diagnostic procedures. Recent HAE management guidelines emphasized home-based therapy and self-management as the preferred strategies whenever possible, aiming to improve the patients’ quality of life