Active Ingredient: Pegzilarginase
Pegzilarginase is indicated for the treatment of arginase 1 deficiency (ARG1-D), also known as hyperargininemia, in adults, adolescents and children aged 2 years and older.
For this indication, competent medicine agencies globally authorize below treatments:
For:
Regimen A: Intravenous, 0.1 milligrams pegzilarginase per kilogram of body weight, once weekly.
Regimen B: Subcutaneous, 0.1 milligrams pegzilarginase per kilogram of body weight, once weekly.
Pegzilarginase is intended for chronic management of patients with ARG1-D in conjunction with individualised disease management such as dietary protein restriction, amino acid supplements and pharmacological treatment including nitrogen scavengers.
Pegzilarginase should be administered by intravenous infusion or subcutaneous injection, using the same dose. In clinical trials, treatment was initiated as intravenous administration with subsequent transition to subcutaneous administration after 8 weeks, at the earliest.
The recommended initial dose of pegzilarginase is 0.1 mg/kg per week. The dose may be increased or decreased in 0.05 mg/kg increments to achieve therapeutic goals. Doses above 0.2 mg/kg per week have not been studied in clinical trials in ARG1-D.
Prior to initiating treatment, a baseline plasma arginine concentration should be obtained. After initiating treatment, the weekly dose should be adjusted based on pre-dose plasma arginine concentrations to maintain plasma arginine within the normal range. To maximise the time within the normal range, dose adjustments should be aimed at achieving a pre-dose level of plasma arginine near the upper limit of normal (ULN). The dose adjustment should typically be based on two consecutive measurements, and first such assessment performed after 4 weeks of administration. It is recommended to monitor plasma arginine levels weekly for 2 weeks after any dose adjustment to assess impact of the dose change.
Once the individualised dose level has been established, monitoring of plasma arginine concentration is recommended to be performed in accordance with standard clinical monitoring visits, with no longer intervals than 3-6 months.
Validated methods to monitor arginine levels are to be used in patients treated with pegzilarginase, as standard methods are not adequate to control residual enzyme activity of pegzilarginase after sampling, and may lead to artificially low arginine levels, and incorrect dose adjustments.
If a dose is missed, administer pegzilarginase as soon as possible. Patients should not be administered 2 doses to make up for the missed dose and should have a minimum of 4 days between doses.
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