Key points
- Receiving complement inhibitor therapy significantly increases a patient's risk for meningococcal disease.
- Antimicrobial prophylaxis could potentially reduce the risk of meningococcal disease.
- Vaccination is also recommended but offers incomplete protection.
- Healthcare providers should have a high index of suspicion in patients with meningococcal symptoms who receive complement inhibitors.
Guiding principles
Complement inhibitor therapy increases risk
The risk of meningococcal disease is up to 2,000 times greater for people receiving complement inhibitors compared to otherwise healthy individuals.
Complement inhibitors include:
- Eculizumab (Soliris®)
- Ravulizumab (Ultomiris™)
The Food and Drug Administration (FDA)-approved prescribing information for complement inhibitors includes a black box warning for increased meningococcal disease risk. The warning includes a recommendation to administer meningococcal vaccines to patients receiving complement inhibitors.
Risk remains even for vaccinated patients
CDC data suggest meningococcal vaccines likely provide incomplete protection against invasive meningococcal disease in people receiving eculizumab. Experts believe this increased risk likely also applies to people receiving ravulizumab.
Nongroupable Neisseria meningitidis caused most infections identified in patients receiving eculizumab. Nongroupable N. meningitidis typically does not cause invasive meningococcal disease in people with normal immune systems.
Antimicrobial prophylaxis might reduce risk
In addition to vaccination, healthcare providers could consider antimicrobial prophylaxis for complement inhibitor recipients to potentially reduce meningococcal disease risk.
Recommendations
Educate patients
Clinicians should ensure all patients receiving complement inhibitors:
- Know the symptoms of meningococcal disease
- Are aware of their increased risk
- Seek care immediately if they start experiencing symptoms
- Receive rapid treatment
Have heightened awareness
Healthcare providers should have a high index of suspicion in patients receiving complement inhibitors with symptoms of meningitis or meningococcemia. This is important even if the patient
- Has symptoms that initially appear mild
- Has been fully vaccinated
- Is receiving antimicrobial prophylaxis
Vaccinate patients
CDC recommends both MenACWY and MenB vaccines for people receiving a complement inhibitor. Ideally, administer meningococcal vaccines at least 2 weeks prior to administering the first dose of the complement inhibitor.
MenACWY specifics
Initial series: Depending on the brand and age, MenACWY vaccines require 2, 3, or 4 doses
Booster doses: Administer a booster dose of MenACWY vaccine every 5 years, for the duration of complement inhibitor therapy.
Targets: Serogroups A, C, W, and Y; provides no protection against nongroupable N. meningitidis
MenB specifics
Initial series: Depending on the brand, MenB vaccines require 2 or 3 doses
Booster doses: Administer a booster dose of MenB vaccine:
- 1 year after series completion
- Every 2 to 3 years thereafter, for the duration of complement inhibitor therapy
Targets: Serogroup B; cross protection against nongroupable N. meningitidis has not been assessed
Resources
Information on conditions treated by complement inhibitors
Atypical hemolytic uremic syndrome (aHUS)
Generalized myasthenia gravis (gMG)
Neuromyelitis optica spectrum disorder (NMOSD)
Paroxysmal nocturnal hemoglobinuria (PNH)
Patient information
Meningococcal disease and people receiving complement inhibitors