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Published on: March 1, 2017
Eculizumab Use in Neuromyelitis Optica Spectrum Disorders: Routine Clinical Care Data From a European Cohort
Marius Ringelstein1, Susanna Asseyer1, Gero Lindenblatt1
1From the Department of Neurology (M.R., K.F., S.G.M., O.A.), Medical Faculty; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich-Heine-University Düsseldorf; Experimental and Clinical Research Center (S.A., F.P., J.B.-S.), a Cooperation Between the Max Delbrück Center for Molecular Medicine in the Helmholtz Association and Charité Universitätsmedizin; Charité - Universitätsmedizin Berlin (S.A., F.P., J.B.-S.), corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Experimental and Clinical Research Center; Max Delbrück Center for Molecular Medicine in the Helmholtz Association (MDC) (S.A., F.P., J.B.-S.), Berlin; Department of Neurology (G.L.), Johanna Etienne Hospital, Neuss; Center for Translational Neuro- and Behavioral Sciences (R.P., J.S., C.K.), University Medicine Essen, University of Duisburg-Essen; Department of Neurology (L.R., L.K.), University Hospital, Münster; Department of Neurology (K.G., A.B.), School of Medicine and Health, Technical University of Munich, Klinikum Rechts der Isar; Department of Neurology and Institute of Neuroimmunology and MS (INIMS) (V.H.), University Medical Center Hamburg-Eppendorf; Department of General Pediatrics (M.K.), Neonatology and Pediatric Cardiology, University Children's Hospital, Medical Faculty, Heinrich-Heine-University Düsseldorf; Department of Neurology (K.H., R.G., I.A.), St. Josef-Hospital, Ruhr University Bochum; Department of Neurology (C.O., K.R.), Charité-Universitätsmedizin Berlin, corporate member of Freie Universität and Humboldt-Universität zu Berlin; Center of Clinical Neuroscience (T.Z.), Department of Neurology, Dresden; Department of Neurology (A.E.)Halle; Department of Neurology (V.R., F.T.N.), Erlangen; Department of Neurology (K.A., R.L.), Regensburg; Department of Neurology (S.A.L., C.W.), University Hospital, Köln; Department of Neurology (S.J., M.K.-K., B.W.), Molecular Neuroimmunology Group, University of Heidelberg; Department of Neurology (S.W.), Gießen; Department of Neurology (M.Seipelt), Marburg; Department of Neurology (Y.Y.), Frankfurt; Department of Neurology (N.R., U.K.Z.), Rostock, Germany; Department of Neurology (P.S.R.), Medical University of Vienna, Austria; Department of Neurology (M.C.K.), University Hospital, Tübingen; Section of Translational Neuroimmunology (J.W., C.G.), Department of Neurology, Jena University Hospital; Department of Neurology (M.W.H., C.T.), Hannover Medical School; and Department of Neurology (M.Senel), University Hospital, Ulm, Germany.
Eculizumab effectively prevents neuromyelitis optica spectrum disorder attacks in adults with AQP4-IgG+ NMOSD. Careful consideration of meningococcal vaccination timing and infection risk is crucial for patient safety during treatment.
Area of Science:
- Neurology
- Immunology
- Pharmacology
Background:
- Neuromyelitis optica spectrum disorders (NMOSD) management requires effective attack prevention.
- Eculizumab (ECU), a complement cascade inhibitor, showed high efficacy in a phase III trial for AQP4-IgG+ NMOSD.
- Real-world effectiveness and safety data for ECU in NMOSD are essential.
Purpose of the Study:
- To evaluate the effectiveness of eculizumab in preventing attacks in patients with NMOSD in routine clinical practice.
- To assess the safety profile of eculizumab, including adverse events and mortality.
- To analyze changes in disease activity (MRI lesions) and disability (EDSS) during eculizumab therapy.
Main Methods:
- Retrospective chart review of 52 AQP4-IgG+ NMOSD patients treated with eculizumab at 20 centers.
- Evaluation of annualized attack rate (AAR), MRI activity, and Expanded Disability Status Scale (EDSS) changes.
- Analysis of adverse events, serious infections, mortality, and post-vaccination attacks.
Main Results:
- 88% of patients remained attack-free during eculizumab therapy.
- Median AAR significantly decreased from 1.0 to 0 (p < 0.001).
- EDSS scores remained stable; MRI lesions decreased. Serious infections occurred in 13%, and 10% died, particularly older, severely disabled patients.
Conclusions:
- Eculizumab is effective in preventing NMOSD attacks in real-world clinical care.
- Increased risk of attacks post-meningococcal vaccination (before ECU) and systemic infections (during ECU) requires attention.
- Further research on optimal meningococcal vaccination timing is needed.
