Conestat alfa

Conestat alfa is a recombinant human (rh) C1 inhibitor developed for the treatment of HAE-C1- INH attacks. Conestat alfa is produced by a recombinant DNA technology in the mammary glands of transgenic rabbits.

In three RCT studies and one open-label extension study, Ruconest® proved to be highly effective in hereditary angioedema . Differences in post-translational glycosylation result in a significantly shorter half-life compared to C1-INH from human plasma. In October 2010, Ruconest® was approved for the treatment of attacks of HAE-C1- INH(hereditary angioedema type I) in Europe (Zuraw B et al. 2010; Riedl MA et al. 2014; Reshef A et al. 2013; Moldovan D et al. 2012).

Dosage: Ruconest® is approved in Europe for use in adolescents and adults and since 2017 also for self-administration. It is injected intravenously. According to the manufacturer, patients up to 84 kg bw receive 50 units per kg bw, with higher bw 4,200 units (two ampoules dissolved in 14 ml solvent each).

Based on experience to date, the safety profile of Ruconest® is very favorable. The most common adverse effect of Ruconest® is headache. Because a healthy volunteer with an undisclosed rabbit allergy developed an anaphylactic reaction after administration of rh-C1- INH, the European Medicines Agency (EMA) decided that Ruconest® is contraindicated in patients with a known or suspected rabbit allergy or with positive IgE to rabbit allergens due to the risk of allergic reactions.

Ruconest®

1. Moldovan D et al (2012) Efficacy and safety of recombinant human C1-inhibitor for the treatment of attacks of hereditary angioedema: European open-label extension study. Clin Exp Allergy 42:929-935
2. Reshef A et al (2013) Recombinant human C1 inhibitor for the prophylaxis of hereditary angioedema attacks: a pilot study. Allergy 68:118-q24
3. Riedl MA et al (2014) Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial. Ann Allergy Asthma Immunol 112:163-169.
4. Zuraw B et al. (2010) Recombinant human C1-inhibitor for the treatment of acute angioedema attacks in patients with hereditary angioedema. J Allergy Clin Immunol 126:821-827.