Managing the Risk of Meningococcal Disease among Patients Who Receive Complement Inhibitor Therapy

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To learn more about the increased risk of meningococcal disease in patients receiving eculizumab and prevention strategies, read the July 7, 2017 MMWR.

Recent data suggest that meningococcal vaccines likely provide incomplete protection against invasive meningococcal disease in people receiving eculizumab (Soliris®). Experts believe this increased risk likely also applies to people receiving ravulizumab (Ultomiris™). In addition to vaccination, clinicians could consider antimicrobial prophylaxis for complement inhibitor recipients to potentially reduce the risk of meningococcal disease.

Clinicians most commonly prescribe complement inhibitors for treatment of four rare health conditions:

  • Atypical hemolytic uremic syndrome (aHUS)
  • Paroxysmal nocturnal hemoglobinuria (PNH)
  • Generalized myasthenia gravis (MG)
  • Neuromyelitis optica spectrum disorder (NMOSD)

Heightened awareness key for clinicians and patients

Clinicians should have a high index of suspicion for meningococcal disease in patients receiving complement inhibitors with symptoms of meningitis or meningococcemia. You need to have this high index of suspicion even if your patient

  • Has symptoms that initially appear mild
  • Has been fully vaccinated
  • Is receiving antimicrobial prophylaxis

Clinicians should also 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
  • Obtain rapid treatment

Receiving complement inhibitors increases the risk of meningococcal disease

People receiving complement inhibitors are at increased risk for meningococcal disease. The risk of getting meningococcal disease is 1,000 to 2,000 times greater for people receiving eculizumab compared to otherwise healthy individuals in the United States. The Food and Drug Administration (FDA)-approved prescribing information for complement inhibitors includes a black box warning for increased risk of meningococcal disease. The warning includes a recommendation to administer meningococcal vaccines to patients receiving complement inhibitors.

Risk for meningococcal disease remains even if vaccinated

Recent data show that some patients receiving eculizumab who were up to date with meningococcal vaccination still developed meningococcal disease. Nongroupable Neisseria meningitidis caused most of these infections. Nongroupable N. meningitidis typically does not cause invasive meningococcal disease in people with normal immune systems.

Meningococcal conjugate (MenACWY) vaccine targets serogroups A, C, W, and Y, and provides no protection against nongroupable N. meningitidis. FDA licensed serogroup B meningococcal (MenB) vaccines specifically for protection against serogroup B meningococcal disease. Researchers have not assessed the extent of any potential cross protection from MenB vaccines for nongroupable N. meningitidis strains.

CDC still recommends meningococcal vaccination for patients receiving complement inhibitors

Despite disease occurring among vaccinated individuals, clinicians should continue vaccinating patients who receive a complement inhibitor. Ideally, you should administer meningococcal vaccines at least 2 weeks prior to administering the first dose of the complement inhibitor. CDC recommends both MenACWY and MenB vaccines for people receiving a complement inhibitor. Depending on the brand, the full series of MenB vaccine requires 2 or 3 doses. Administer a booster dose of MenACWY vaccine every 5 years, for the duration of complement inhibitor therapy. Administer a booster dose of MenB vaccine 1 year after series completion and then every 2 to 3 years thereafter, for the duration of complement inhibitor therapy.


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