Drug Action
Human alpha1-proteinase inhibitor is a normal plasma constituent; it inhibits neutrophil elastase (an enzyme released in response to inflammation) in the lungs.
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Drug reference
Human alpha1-proteinase inhibitor is a normal plasma constituent; it inhibits neutrophil elastase (an enzyme released in response to inflammation) in the lungs.
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Progression of emphysema in patients with severe alpha1- proteinase inhibitor deficiency, despite optimal treatment with other standard therapies (specialist use only) ▶BY INTRAVENOUS INFUSION ▶Adult: 60 mg/kg once weekly
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IgA deficiency with confirmed antibodies against IgA—increased risk of severe hypersensitivity reactions
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Consider vaccination against hepatitis A and hepatitis B. hypersensitivity reactions. IgA deficiency (without known antibodies to IgA)—increased risk of hypersensitivity reactions CAUTIONS, FURTHER INFORMATION ▶Hypersensitivity reactions Hypersensitivity reactions may occur, even in patients who have previously tolerated treatment. Manufacturer advises close monitoring, including vital signs, during the first infusions. If a reaction occurs, manufacturer advises to decrease the rate of infusion or discontinue treatment; if symptoms improve promptly after stopping, the infusion may be resumed at a slower infusion rate.
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Manufacturer advises to record the brand name and batch number after each administration (in case of transmission of infective agents).
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Scottish Medicines Consortium (SMC) decisions The Scottish Medicines Consortium has advised (August 2016) that human alpha-1 proteinase inhibitor (Respreeza ®) is not recommended for use within NHS Scotland for maintenance treatment, to slow the progression of emphysema in adults with documented severe alpha1-proteinase inhibitor deficiency. The manufacturer did not present a sufficiently robust clinical or economic analysis. All Wales Medicines Strategy Group (AWMSG) decisions The All Wales Medicines Strategy Group has advised (March 2017) that human alpha-1 proteinase inhibitor (Respreeza ®) is not recommended for use within NHS Wales for maintenance treatment, to slow the progression of emphysema in adults with documented severe alpha1- proteinase inhibitor deficiency. The case for clinical and cost-effectiveness has not been proven.
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▶Common or very common Dizziness. headache. nausea ▶Uncommon Asthenia. confusion. flushing. hypersensitivity. hypotension. sensation abnormal. skin reactions. syncope. tachycardia. throat oedema ▶Rare or very rare Chest pain. chills. fever. hyperhidrosis ▶Frequency not known Eye swelling. facial swelling. lip swelling. lymph node pain
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Manufacturer advises caution—no information available.
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Manufacturer advises avoid—no information available.
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There can be variation in the licensing of different medicines containing the same drug. Powder and solvent for solution for infusion ELECTROLYTES: May contain Sodium ▶Respreeza (CSL Behring UK Ltd) A Human alpha1-proteinase inhibitor 1 gram Respreeza 1000mg powder and solvent for solution for infusion vials | 1 vial P £220.00 IMMUNOSUPPRESSANTS › MONOCLONAL ANTIBODIES
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Manufacturer advises patients receiving treatment at home should receive appropriate training regarding self-administration, and be informed of the signs of hypersensitivity reactions. Driving and skilled tasks Manufacturer advises patients and carers should be counselled on the effects on driving and performance of skilled tasks—increased risk of dizziness.
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60 mg/kg once weekly
First-line fragment. Read the complete Indications and Dose section above.
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