Clinical Guidance for Managing Meningococcal Disease Risk in Patients Receiving Complement Inhibitor Therapy

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:

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