Current treatment options for hereditary angioedema


Authors: MUDr. Roman Hakl
Authors‘ workplace: Ústav klinické imunologie a alergologie LF MU a FN u sv. Anny v Brně
Published in: Vnitř Lék 2016; 62(9): 736-739
Category: Reviews

Overview

Hereditární angioedém je vzácné dominantně dědičné onemocnění způsobené deficitem inhibitoru C1-esterázy (C1-INH). Onemocnění se klinicky projevuje recidivami lokalizovaných otoků podkoží a sliznic. Nemoc je hendikepující a může být i smrtelná. Charakteristická je extrémní variabilita v četnosti a závažnosti symptomů. Článek se zabývá organizací péče o pacienty a zahrnuje aktuální léčebné možnosti onemocnění. Strategie léčby zahrnuje krátkodobou a dlouhodobou profylaxi a léčbu atak. V současné době léčebné možnosti zahrnují atenuované androgeny, antifibrinolytika, rekombinantní (rhC1-INH) a plazma derivované (pdC1-INH) C1-INH, antagonistu bradykininového receptoru a inhibitor kalikreinu. V České republice je péče od roku 2011 soustředěna do 4 diagnosticko-terapeutických center.

Klíčová slova:
bradykininový receptor – C1 inhibitor – hereditární angioedém – komplementový systém


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