Abbreviated Summary of Product Characteristics
Name of the product
RUCONEST® 2100 U powder and solvent for solution for injection. Conestat alfa is the recombinant analogue of the human C1 esterase inhibitor (rhC1-INH) produced by recombinant DNA technology in the milk of transgenic rabbits.
RUCONEST® is indicated for treatment of acute angioedema attacks in adults and adolescents with hereditary angioedema (HAE) due to C1 esterase inhibitor deficiency.
Dosage and administration
Each vial of RUCONEST® (2100 U) should be reconstituted with 14 ml water for injections to obtain a solution of 150 U/ml.
RUCONEST® should be initiated under the guidance and supervision of a physician experienced in the diagnosis and treatment of hereditary angioedema until the patient (or caregiver) is competent to administer after having been properly trained.
RUCONEST® should be administered based on patient body weight:
|Body Weight||RUCONEST® dose for intravenous, infusion||Volume (ml) of reconstituted solution, (150 U/ml) to be administered|
|<84 kg||50 U/kg||Body weight in kg divided by 3|
|≥84 kg||4200 U (2 vials)||28 ml|
The reconstituted solution should be administered at room temperature as a slow intravenous injection over approximately 5 minutes. In the majority of cases, a single dose of RUCONEST® is sufficient to treat an acute angioedema attack. In case of an insufficient clinical response, an additional dose (50 U/kg body weight up to 4200 U) can be administered. Not more than two doses should be administered within 24 hours.
Known or suspected allergy to rabbits. Known hypersensitivity to any of the components of the product.
Warning and precautions
RUCONEST® is derived from the milk of transgenic rabbits and contains traces of rabbit protein. Before initiating treatment, patients should be queried about prior exposure to rabbits and sign and symptoms suggestive of an allergic reaction. Hypersensitivity reactions cannot be excluded. Patients must be closely monitored and carefully observed for any symptoms of hypersensitivity throughout the administration period. Patients should be informed of the early signs of hypersensitivity reactions including hives, generalised urticaria, tightness of the chest, wheezing, hypotension and anaphylaxis. If these symptoms occur after administration, they should alert their physician. In case of anaphylactic reactions or shock, emergency medical treatment should be administered.
Although cross-reactivity between cow milk and rabbit milk is considered unlikely, the possibility of such a cross-reactivity in a patient who has evidence of clinical allergy to cow milk cannot be excluded and the patient should be observed for signs and symptoms of hypersensitivity following RUCONEST® administration. Patients on a controlled sodium diet should be aware that each vial of RUCONEST® contains 19.5 mg sodium.
Home-treatment and self-administration
There are limited data on the use of this medicinal product in home- or self administration. Potential risks associated with home-treatment are related to the administration itself as well as the handling of adverse reactions, particularly hypersensitivity. The decision on the use of home-treatment for an individual patient should be made by the treating physician, who should ensure that appropriate training is provided and the use reviewed at intervals.
Although no interaction studies have been performed, RUCONEST® should not be administered simultaneously with tissue-type plasminogen activator (tPA).
Pregnancy and lactation
There is no experience of using RUCONEST® in pregnant or breast-feeding women. RUCONEST® is not recommended for use during pregnancy or breast-feeding, unless the treating physician judges the benefits to outweigh the possible risks.
Ability to drive and use machines
Patients should be advised not to drive or use machines if they experience headache or vertigo following RUCONEST® treatment.
Undesirable reactions with RUCONEST® are rare – one case of hypersensitivity was observed in clinical trials. The most common adverse reaction observed after administration of RUCONEST® is headache. Uncommon reactions that have been observed include vertigo, paraesthesia, throat irritation, diarrhoea, nausea, abdominal discomfort, oral paraesthesia, urticaria and swelling. There is no clinical information on overdose available.
Legal category: POM
Basic NHS price: £750
Marketing Authorisation number: EU/1/10/641/002
Further information is available from the Marketing Authorisation Holder: Pharming Group N.V., Darwinweg 24, 2333 CR Leiden, the Netherlands.
Date text last revised: November 2018 038RUC2018P-ENG
Please refer to the full SmPC for complete information including shelf life, storage of powder and storage post reconstitution. The full SmPC can be found at: https://www.medicines.org.uk/emc/product/8579/smpc. Adverse events should be reported. Reporting forms and information can be found at www.mhra.gov.uk/yellowcard.