Ruconest

Ruconest, with the active ingredient conestat alfa, is a recombinant human C1 esterase inhibitor used to treat acute attacks of hereditary angioedema (HAE) in patients with C1-INH deficiency. It is produced by Pharming Group N.V. in the milk of transgenic rabbits and formulated as an intravenous preparation.

HAE arises from insufficient functional C1 esterase inhibitor, leading to unregulated activation of the complement and contact cascades and increased bradykinin, which causes swelling. Ruconest supplements the missing inhibitor and helps restore control of these pathways during an attack.

Ruconest is approved for the treatment of acute HAE attacks in eligible patients in various regulatory jurisdictions. It is not used for prophylaxis but for on-demand treatment of established attacks.

Administration is intravenous. A single dose is given at 50 units per kilogram body weight, up to a maximum of 4200 units per dose, infused over 5 to 10 minutes.

Symptom relief commonly begins within 30 to 60 minutes after administration, with improvement continuing for several hours.

Common adverse effects include nausea, headache, fever, and infusion-related reactions. Severe hypersensitivity is rare; thromboembolic events have been reported in isolated cases. The drug is used with caution in patients at risk of thrombosis. Regulatory status varies by country; Ruconest has been approved in several regions for on-demand treatment of acute HAE attacks.