Meningococcal prophylaxis in neurological diseases treated with complement inhibitors: an expert consensus for Germany, Austria, and Switzerland.

Abstract

Inhibition of terminal complement activation is an effective therapeutic strategy for acetylcholine receptor antibody-positive generalized myasthenia gravis (gMG) and aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (NMOSD), but it increases the risk of invasive meningococcal infections. Consequently, vaccination against meningococcal serogroups A, C, W, Y, and B is mandatory for all patients receiving complement inhibitors. This article provides expert consensus recommendations for managing meningococcal vaccination in patients with NMOSD and gMG receiving complement-inhibiting therapies in Germany, Austria, and Switzerland. The timing and procedures of vaccination should be adapted to the underlying diagnosis and the individual risk of disease exacerbation in this population. In NMOSD, treatment often must begin promptly, particularly after an acute attack; in such cases, vaccination should be administered at treatment initiation together with antibiotic prophylaxis. By contrast, treatment urgency in gMG is typically lower, allowing vaccination to be completed in advance and thereby avoiding antibiotic exposure, which may worsen gMG symptoms. For both diseases, ceftriaxone is recommended as first-line therapy for suspected infection, rifampicin for prophylaxis, and either rifampicin or intramuscular ceftriaxone for post-exposure chemoprophylaxis. Patients should also carry a standby dose of ciprofloxacin for emergency self-administration at the first signs of meningitis, followed by immediate clinical evaluation. These recommendations should be reviewed regularly and updated as necessary to reflect emerging evidence and new vaccine options. How to prevent serious infections during treatment for NMOSD and myasthenia gravis Blocking terminal complement activation is an effective treatment for people with acetylcholine receptor antibody–positive generalized myasthenia gravis (gMG) and aquaporin-4 antibody–positive neuromyelitis optica spectrum disorder (NMOSD). However, this treatment increases the risk of serious meningococcal infections. For this reason, all patients receiving complement inhibitors must be vaccinated against meningococcal serogroups A, C, W, Y, and B. This article presents expert consensus recommendations on how to manage meningococcal vaccination in patients with NMOSD and gMG receiving complement-inhibiting therapies in Germany, Austria, and Switzerland. The timing and approach to vaccination should be adapted to the specific disease and to each patient’s risk of worsening symptoms. In NMOSD, treatment often needs to start quickly, especially after an acute attack. In these cases, vaccination should be given at the start of complement inhibitor therapy together with preventive antibiotic treatment. In contrast, treatment for gMG is usually less urgent. This often allows vaccination to be completed before therapy begins and helps avoid antibiotic use, which may worsen gMG symptoms. For both diseases, ceftriaxone is recommended as the first treatment if infection is suspected. Rifampicin is recommended for preventive antibiotic treatment, and either rifampicin or intramuscular ceftriaxone can be used after possible exposure to infection. Patients should also carry a standby dose of ciprofloxacin to take immediately if early signs of meningitis appear, followed by urgent medical evaluation. These recommendations should be reviewed regularly and updated as new evidence and vaccines become available.