Patients Receiving Eculizumab (Soliris®) at High Risk for InvasiveMeningococcal Disease Despite Vaccination Summary Eculizumab (Soliris®) recipients have a 1,000 to 2,000-fold greater risk of invasive meningococcaldisease compared to the general U.S. population. The Food and Drug Administration (FDA)-approvedprescribing information for eculizumab includes a black box warning for increased risk of meningococcaldisease, and the Advisory Committee on Immunization Practices (ACIP) recommends meningococcalvaccination for all patients receiving eculizumab. Recent data show that some patients receivingeculizumab who were vaccinated with the recommended meningococcal vaccines still developedmeningococcal disease, most often from nongroupable Neisseria meningitidis, which rarely causesinvasive disease in healthy individuals. Background Eculizumab is most commonly prescribed for treatment of 2 rare blood disorders: atypical hemolyticuremic syndrome (aHUS) and paroxysmal nocturnal hemoglobinuria (PNH). Through a request for dataon meningococcal disease cases reported to state health departments, the U.S. Centers for DiseaseControl and Prevention (CDC) identified 16 cases of meningococcal disease in eculizumab recipients inthe United States from 2008 through 2016; 11 (69%) of these were caused by nongroupable N.meningitidis. Meningococcal conjugate (MenACWY) vaccine targets serogroups A, C, W, and Y, andprovides no protection against nongroupable N. meningitidis. Serogroup B meningococcal (MenB)vaccines are licensed specifically for protection against serogroup B meningococcal disease.Researchers have not assessed the extent of any potential cross protection for nongroupable N.meningitidis strains. Recommendations for Healthcare Providers Healthcare Providers: • Could consider antimicrobial prophylaxis for the duration of eculizumab therapy to potentiallyreduce the risk of meningococcal disease. • Should continue meningococcal vaccination of all patients who receive eculizumab. • Should administer meningococcal vaccines at least 2 weeks prior to administering the first doseof eculizumab, unless the risks of delaying eculizumab therapy outweigh the risks of developing ameningococcal infection, according to the product label. • Should maintain a high index of suspicion for meningococcal disease in patients takingeculizumab who present with any symptoms consistent with either meningitis ormeningococcemia, even if the patient’s symptoms initially appear mild, and irrespective of thepatient’s meningococcal vaccine or antimicrobial prophylaxis status. For More Information Managing the Risk of Meningococcal Disease among Patients Who Receive Eculizumab Therapy https://www.cdc.gov/meningococcal/clinical/eculizumab.html Signs and Symptoms of Meningococcal Disease https://www.cdc.gov/meningococcal/about/symptoms.html Food and Drug Administration. Soliris® (eculizumab) product label https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/125166s417lbl.pdf Atypical Hemolytic Uremic Syndrome (aHUS) https://rarediseases.org/rare-diseases/atypical-hemolytic-uremic-syndrome/ Paroxysmal Nocturnal Hemoglobinuria (PNH) http://www.aamds.org/diseases/pnh Child and Adolescent Indications Schedule: Vaccines That Might Be Indicated for Persons Aged 0 through 18 Years Based On Medical Indications https://www.cdc.gov/vaccines/schedules/hcp/imz/child-indications.html 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 https://www.cdc.gov/vaccines/schedules/hcp/imz/adult-conditions.html References 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 vaccination. MMWR Morb Mortal Wkly Rep. Epub ahead of print. 2017. https://www.cdc.gov/mmwr/volumes/66/wr/mm6627e1.htm?s_cid=mm6627e1_w The Centers for Disease Control and Prevention (CDC) protects people's health and safety by preventing and controlling diseases and injuries; enhances health decisions by providing credible information on critical health issues; and promotes healthy living through strong partnerships with local, national and international organizations.