Managing the Risk of Meningococcal Disease among Patients Who Receive Complement Inhibitor Therapy
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 eculizumab (Soliris®) recipients. Experts believe this increased risk likely also applies to ravulizumab (Ultomiris™) recipients. 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 three rare health conditions:
- Atypical hemolytic uremic syndrome (aHUS)
- Paroxysmal nocturnal hemoglobinuria (PNH)
- Generalized myasthenia gravis (MG)
Heightened Awareness Key for Clinicians and Patients
Clinicians should have a high index of suspicion for meningococcal disease in patients taking 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
Taking Complement Inhibitors Increases the Risk of Meningococcal Disease
People taking complement inhibitors are at increased risk for meningococcal disease. The risk in eculizumab recipients is 1,000 to 2,000 times greater risk for getting meningococcal disease 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 Taking Complement Inhibitors
Despite disease occurring among vaccinated individuals, clinicians should continue immunizing 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 complement inhibitor recipients. 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.
- Food and Drug Administration. UltomirisTM (ravulizumab) product label pdf icon[20 pages]external icon. Accessed 5/2019.
- Food and Drug Administration. Soliris® (eculizumab) product label pdf icon[33 pages]external icon. Accessed 6/2017.
- McNamara LA, Topaz N, Wang X, Hariri S, Fox L, MacNeil J. High risk for invasive meningococcal disease among patients receiving eculizumab (Soliris) despite receipt of meningococcal vaccine. MMWR Morb Mortal Wkly Rep. 2017;66(27):734–7.
- For Patients: Taking Complement Inhibitors Increases Your Risk for Meningococcal Disease
- Atypical Hemolytic Uremic Syndrome (aHUS)external icon
- Paroxysmal Nocturnal Hemoglobinuria (PNH)external icon
- Child and Adolescent Indications Schedule: Vaccines That Might Be Indicated for Persons Aged 0 through 18 Years Based On Medical Indications
- Adult Immunization Schedule by Medical and Other Indications: Recommended Immunization Schedule for Adults Aged 19 Years or Older by Medical Conditions and Other Indications, United States, 2017