Myasthenia gravis in patients who are anti-acetylcholine receptor antibody positive

Active Ingredient: Zilucoplan

Indication for Zilucoplan

Population group: only adults (18 years old or older)
Therapeutic intent: Adjunct - intent

Zilucoplan is indicated as an add-on to standard therapy for the treatment of generalised myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AChR) antibody positive.

For this indication, competent medicine agencies globally authorize below treatments:

For patients weighting <56 kg 16.6 mg once daily, for patients weighting ≥56 - <77 kg 23 mg once daily and for patients weighting ≥77 kg 32.4 kg once daily

For:

Dosage regimens

Regimen A: In case that patient weight is ≤ 56 kg, subcutaneous, 16.6 milligrams zilucoplan, once daily.

Regimen B: In case that patient weight is ≥ 6 kg and patient weight is ≤ 77 kg, subcutaneous, 23 milligrams zilucoplan, once daily.

Regimen C: In case that patient weight is ≥ 77 kg, subcutaneous, 32.4 milligrams zilucoplan, once daily.

Detailed description

Before starting therapy, patients must be vaccinated against Neisseria meningitidis. If treatment needs to start less than 2 weeks after vaccination, the patient must receive appropriate prophylactic antibiotic treatment until 2 weeks after the first vaccination dose.

Posology

The recommended dose should be given as a subcutaneous injection once daily and administered about the same time every day.

Total daily dose by body weight range:

Body weight Dose*
<56 kg 16.6 mg
≥56 to <77 kg 23 mg
≥77 kg 32.4 mg

* The recommended dose corresponds to approximately 0.3 mg/kg.

Zilucoplan has not been studied in gMG patients with a Myasthenia Gravis Foundation of America (MGFA) Class V.

Missed dose

If a dose is missed, it should be administered the same day; then, normal dosing should be continued the following day. No more than one dose should be administered per day.

Dosage considerations

Administered by subcutaneous injection.

Suitable injection sites include front of the thighs, abdomen and the back of the upper arms.

Injection sites should be rotated and injections should not be given in areas where the skin is tender, erythematous, bruised, indurated or where the skin has scars or stretch marks.

Active ingredient

Zilucoplan

Zilucoplan is a 15 amino acid, synthetic macrocyclic peptide that inhibits the effects of the complement protein C5 through a dual mechanism of action. It specifically binds to C5, thereby inhibiting its cleavage by the C5 convertase to C5a and C5b, which results in a downregulation of the assembly and cytolytic activity of the membrane attack complex (MAC). Additionally, by binding to the C5b moiety of C5, zilucoplan sterically hinders binding of C5b to C6, which prevents the subsequent assembly and activity of the MAC, should any C5b be formed.

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