Currently Enrolling Trials
Zilbrysq (zilucoplan) is a complement inhibitor.
Zilbrysq is specifically indicated for the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AChR) antibody positive.
Zilbrysq is supplied as an injection for subcutaneous administration.
Vaccination and Prophylaxis for Meningococcal Infection
- Vaccinate patients for meningococcal infection (serogroups A, C, W, and Y [MenACWY] and serogroup B [MenB]) according to current ACIP recommendations at least 2 weeks prior to administering the first dose of Zilbrysq.
- If urgent Zilbrysq therapy is indicated in a patient who is not up to date with vaccines for both MenACWY and MenB according to ACIP recommendations, administer meningococcal vaccine(s) as soon as possible and provide the patient with antibacterial drug prophylaxis. Healthcare providers who prescribe Zilbrysq must enroll in the Zilbrysq REMS.
Recommended Testing Before Initiating Zilbrysq
- Before initiating Zilbrysq, obtain baseline lipase and amylase levels
- The recommended dosage of Zilbrysq is given once daily as a subcutaneous injection and is dependent on actual body weight:
|Body Weight||Once Daily Dosage||Plunger Rod Color of Prefilled Syringe|
|Less than 56 kg||16.6 mg||RUBINE RED|
|56 kg to less than 77 kg||23 mg||ORANGE|
|77 kg and above||32.4 mg||DARK BLUE|
Mechanism of Action
Zilbrysq (zilucoplan) binds to the complement protein C5 and inhibits its cleavage to C5a and C5b, preventing the generation of the terminal complement complex, C5b-9. The precise mechanism by which zilucoplan exerts its therapeutic effect in generalized myasthenia gravis is unknown but is presumed to involve reduction of C5b-9 deposition at the neuromuscular junction.
Adverse effects associated with the use of Zilbrysq may include, but are not limited to, the following:
- injection site reactions
- upper respiratory tract infection
The Zilbrysq drug label comes with the following Black Box Warning:
- Life-threatening and fatal meningococcal infections have occurred in patients treated with complement inhibitors; Zilbrysq is a complement inhibitor. Meningococcal infection may become rapidly life-threatening or fatal if not recognized and treated early.
- Complete or update meningococcal vaccination at least 2 weeks prior to administering the first dose of Zilbrysq, unless the risk of delaying therapy outweighs the risk of developing a meningococcal infection. Comply with the most current Advisory Committee on Immunization Practices (ACIP) recommendations for meningococcal vaccinations in patients receiving a complement inhibitor.
- Persons receiving Zilbrysq are at increased risk for invasive disease caused by N. meningitidis, even if they develop antibodies following vaccination. Monitor patients for early signs of meningococcal infections and evaluate immediately if infection is suspected.
- Zilbrysq is available only through a restricted program called Zilbrysq REMS.
Clinical Trial Results
The FDA approval of Zilbrysq was based on data from the RAISE study, a multi-center, Phase 3, randomized, double-blind, placebo-controlled study to assess the efficacy, safety profile, and tolerability of Zilbrysq in adult patients with anti-acetylcholine receptor (AChR) antibody-positive gMG. Patients were randomized in a 1:1 ratio to receive daily subcutaneous injections of 0.3 mg/kg Zilbrysq or placebo for 12 weeks.